Chapter

8
Surgical Interventions for
Congenital Anomalies
Diana Farmer, Nicole Sitkin, Katrine Lofberg,
Peter Donkor, and Doruk Ozgediz

INTRODUCTION unrealized promise of pediatric surgery, and concludes

with crucial future steps to reduce the burden of disease.
Great strides have been made during the past 50 years Chapter 21 addresses economic evaluation of cleft lip
in the diagnosis and management of congenital anom- and palate repair.
alies, once referred to as birth defects. Formally fatal
conditions can now be treated with success rates
exceeding 90 percent. Yet improvements in care have MORBIDITY, MORTALITY, AND ADDITIONAL
been largely limited to high-income countries (HICs),
ADVERSE CONSEQUENCES
even though many anomalies can be cured with sim-
ple operations (Chirdan, Ngiloi, and Elhalaby 2012). Congenital anomalies are one of the leading causes of
If surgery is the neglected stepchild of global health global disease, responsible for a staggering 57.7 million
(Farmer and Kim 2008), then pediatric surgery is the disability-adjusted life years (DALYs) lost worldwide
child not yet born. (WHO 2013b). DALYs—a measure of the number of
Improving the accessibility and quality of ­pediatric healthy life years lost to premature death or disability—
­surgical care in low-income (LICs) and lower-­middle- are an established metric for the burden of disease.
income countries (LMICs) has the potential to substan- Current estimates of the surgical burden of disease
tially reduce childhood mortality and lifelong disability. are acknowledged to be a “best educated guess,” given the
Data on congenital anomalies in these countries are sparse, “near total lack of pertinent data” (Jamison and others
including on the incidence (conservatively estimated at 2006, 1246). Even less is known about pediatric surgical
3 percent to 6 percent [CDC 2012; Christianson, Howson, disease (Bickler and Rode 2002). The studies that have
and Modell 2006]), country-specific d­ ifferences in disease begun to fill this knowledge gap paint a brutal picture.
burden, and cost-­effective interventions. These areas of The burden of congenital anomalies falls most heavily on
knowledge must be developed to identify implemen- LICs and LMICs, where 94 percent of anomalies occur
tation and surveillance priorities, and to advocate for (WHO 2012). Higher fertility rates translate to higher
resources. birth rates and more children born with anomalies.
This chapter briefly summarizes the growing body of Disease incidence (or frequency of disease occurring in
knowledge on surgical congenital anomalies in LICs and the population) is also higher, a phenomenon attributed
LMICs, highlights prevalent anomalies that exemplify the to higher micronutrient and macronutrient deficiencies,

Corresponding author: Diana Farmer, MD, University of California, Davis, [email protected]

129

 exposure to teratogens, prevalence of intrauterine infec- approach to addressing this challenge (Wu, Poenaru, and
tion, and self-medication with unsupervised drugs or Poley 2013).
traditional remedies (Christianson, Howson, and Modell
2006; Penchaszadeh 2002). Though decreased fertility
may reduce incidence of anomalies, most are not oth- Mortality Rate Estimation Challenges
erwise preventable and are treated through surgical The burden of disease associated with congenital anom-
interventions. alies in LICs and LMICs is most often calculated as the
Some anomalies are “quick fixes” that can be easily mortality rate, including neither measures of morbidity
repaired; others require staged, or multioperation, sur- nor the cost of ongoing illness (table 8.1). Analysis
gical interventions, and delays in treatment may result of mortality data in these countries can be challeng-
in lifelong illness, disability, and poor quality of life. The ing; for example, autopsies were performed in only
paucity of surgical resources in LICs and LMICs means 0.8 percent of nearly 1,100 neonatal deaths in Benin.
that anomalies attributed to the former category in HICs In all examined cases, autopsies provided additional
often fall into the latter in LICs and LMICs. Anomalies information on the cause of death (Ugiagbe and Osifo
resulting in visible deformity—such as clubfoot and cleft 2012). Furthermore, a high proportion of children with
lip—cause stigma, which can trigger abandonment or surgical diseases do not reach a health facility and die at
infanticide. Invisible deformities that result in chronic home or in transit, suggesting a sizable hidden mortality
disability can lead to similar outcomes. A long-term, (Mo Suwan and others 2009; Ozgediz and others 2008).
“incurable” anomaly may also endanger families’ well-­ Nonfatal a­nomalies can result in extensive, ongoing
being because key resources are allocated to care for the morbidity. The burden of disease is grossly underesti-
afflicted child. Families may fracture, with one or both mated if this measure of impairment is not included.
parents leaving the child with other family members. Extant calculations do, however, highlight marked dis-
Improving the pediatric surgical capabilities of LICs parities in outcomes between HICs, on the one hand,
and LMICs will dramatically reduce this burden. Because and LICs and LMICs, on the other hand.
children are the future economic engine powering the
development of these countries, the value of investing in
surgical care for children extends beyond DALYs averted
to encompass the future socioeconomic well-being of CHALLENGES TO PROVIDING CARE FOR
LICs and LMICs themselves. It is critical to address
CONGENITAL ANOMALIES
the gaps in knowledge that impede the development of
effective care systems. Despite the higher incidence of congenital anomalies in
LICs and LMICs, lower population prevalence is seen
compared with HICS, most likely indicating a high
Data Collection Challenges infant and child mortality rate (Christianson, Howson,
Many LICs and LMICs lack rigorous congenital anomaly and Modell 2006). In LICs and LMICs, up to 10 percent
surveillance programs, making calculations of incidence of infants die during the neonatal period (Zupan 2005);
and prevalence (the total number of individuals in the a considerable portion of this mortality can reasonably
population with a given disease) difficult (Penchaszadeh be attributed to congenital anomalies.
2002). Estimates, which range from 4 to 12 cases per 1,000
births, likely undervalue the problem because of stigma
and exclusion (Bickler and others 2010; Goksan and Treatment Delays
others 2006; Wu and Poenaru 2013). LICs and LMICs Heightened mortality rates stem from delays in treat-
often report incidence in cases over time, as opposed to ment caused by the paucity of health profession-
using standard metrics, such as incidence per 10,000 live als trained to identify and treat anomalies and by
births. Incidence and prevalence data are also skewed by pejorative cultural beliefs surrounding anomalies.
­
the survivability of the anomaly under consideration. In LICs and LMICs, many births occur at home, either
Because children with anomalies that are not immedi- with no assistance or with traditional birth atten-
ately life threatening are more likely to reach medical dants (TBAs), and patients must often travel great
centers, the relative incidence and prevalence of imme- ­distances to reach ­medical facilities. Hypothermia—a 
diately life-threatening impairments appears compara- dangerous drop in body t­emperature—is com-
tively lower in hospital-based data (Nandi, Mungongo, mon following medically unsupervised transport
and Lakhoo 2008). Population-based ­surveys—which over long d ­istances, with severe repercussions
directly collect data from noncentralized sites—are one on patient outcomes (Agarwala and ­ others 1996;

130 Essential Surgery

Table 8.1  Prevalence and Mortality of Selected Congenital Anomalies in Selected Countries
High-income countries Selected countries
Congenital Reference Reference mortality Country of reporting Prevalence at reporting
anomalies prevalence (percent) institution institution Mortality (percent)
Anorectal 1 per 5,000 live <5a Iran, Islamic Rep.b 22 (1993–96) 27.3 (1993–96)
malformations birthsa 106 (2002–05) 13.2 (2002–05)
Nigeria c
88 over 17 years 30.2
Indiad
125 over 2 years 22.0
Nigeriae 81 over 8 years 32.0
(38 percent of neonatal
obstructions)
Nigeriaf 54 over 10 years 20.4
Ethiopia g
27 over 5 years 33.0
Indiah
948 over 14 years 15.0–20.0 (staged repair)
4.5 (primary repair)
Nigeria i
55 over 10 years 20.0
Hirschsprung’s 1 per 5,000 live Less than 5–10
j, k
Iran, Islamic Rep. b
8 (1993–96) 25.0 (1993–96)
disease birthsa 50 (2002–05) 4.0 (2002–05)
Nigeriae 30 over 8 years 20.0
(14 percent of neonatal
obstructions)
Nigeriai 24 over 10 years 20.8
(18.7 percent of neonatal
obstructions)
Bangladeshl 1,273 over 5.5 years 14.3
Burkina Fasom 52 over 7 years 16.0
Eritrea n
11 over 5 years 9.1
Nigeria o
78 over 10 years 22.6
Congenital heart 8.2 per 1,000 3–7q Guatemalar 1,215 over 8 years 10.7 (overall) 32.1 (highest-
disease live births risk patients)
(Europe)p Indias 330 over 8 years 21.4 (1999–2001)
6.9 per 1,000 4.3 (2002–06)
live births
(North America)p Nepal t
5.8 per 1,000 children 20.2
Sri Lanka u
102 over 1 year 18.6
Esophageal atresia One per 3,500 live 5–15 a
Indiav
50 over 2 years 30.0
birthsa Barbadosw 2.06 per 10,000 live births 30.8
Saudi Arabia x
48 over 20 years 25.0
Bangladesh y
21 over 2 years 47.6
Chinaz 15 over 10 years 46.7 following surgery
Malaysia aa
52 over 10 years 23.0
table continues next page

Surgical Interventions for Congenital Anomalies 131

Table 8.1  Prevalence and Mortality of Selected Congenital Anomalies in Selected Countries (continued)
High-income countries Selected countries
Congenital Reference Reference mortality Country of reporting Prevalence at reporting
anomalies prevalence (percent) institution institution Mortality (percent)
Pakistanab 80 over 1 year, excluding 58.0
isolated esophageal atresia
patients
Saudi Arabiaac 94 over 15 years 30.8
Iran, Islamic Rep.b 22 (1993–96) 75.0 (1993–96)
106 (2002–05) 58.8 (2002–05)
Ethiopia g
12 over 5 years 91.7
Gastroschisis 3.3 to 5 per 10,000 1 (30-day mortality)af Ghana; Nigeria; Over four years: 23.0 (30-day mortality)
live birthsa, ad, ae South Africaaf 2 (Ghana)
5 (Nigeria)
19 (South Africa)
Iran, Islamic Rep. b
2 (1993–96) 100 (1993–96)
7 (2002–05) 85.7 (2002–05)
Iran, Islamic Rep.ag 0.65 per 1,000 births 80.0 (2005–07)
10 per 15,321 live births
(2005–07)
Nigeriaah 14 over 10 years 71.4
Nigeriaai 12 over 11 years 33.0
Colombia aj
32 over 9 years 18.8
South Africa ak
106 over 6 years; 43.0 (total)
6 neonatal surgical 68.0 patients with
admissions (2003); staged, silo-assisted
closure
15 neonatal surgical
admissions (2007)
Thailandal 49 over 3 years 14.0
Nigeriaam
7 of 2,381 patients treated at 57.1
the pediatric surgery unit over
eight years
Omphalocele 2.18–5 per 10,000 5a Iran, Islamic Rep.b 12 (1993–96) 75.0 (1993–96)
live birthsa, ad, ae 68 (2002–05) 58.8 (2002–05)
Iran, Islamic Rep. ag
2.1 per 1,000 births 20.0 (2005–07)
42 of 15,321 live newborn
births (2005–07)
Nigeriaai 42 over 11 years 43.0
Colombia aj
23 over 9 years 43.5
Nigeriaam
49 over 8 years 32.4 (patients with
omphalocele major)
table continues next page

132 Essential Surgery

Table 8.1  Prevalence and Mortality of Selected Congenital Anomalies in Selected Countries (continued)
High-income countries Selected countries
Congenital Reference Reference mortality Country of reporting Prevalence at reporting
anomalies prevalence (percent) institution institution Mortality (percent)
Congenital 1 per 2,000 to 1 per ≤ 10an Turkeyao 10 over 4 years 50.0
diaphragmatic 5,000 live birthsa Tunisia ap
28 over 13 years 39.0
hernia
Nigeria aq
64 over 24 years 35.5 (overall)
60.0 in patients born in
hospital
28.5 in patients born outside
hospital who survive until
presentation
Nigeriaar 7 over 6 years 43.0
Malaysia as
21 over 6 years 47.6
Sources: Anorectal malformations, Ethiopia: Anorectal malformations, India: Anorectal malformations, Iran, Islamic Rep.: Anorectal malformations, Nigeria:
a. Coran and others 2012. b. Peyvasteh and others 2011. c. Adejuyigbe and others 2004. d. Chalapathi and others 2004. e. Adeyemi 1989. f. Archibong and Idika 2004. g. Tefera, Teka, and Derbew
2007. h. Gangopadhyay and others 2005. i. Ekenze, Ibeziako, and Ezomike 2007. j. Rescorla and others 1992. k. Swenson 2002. l. Banu and others 2009. m. Bandre and others 2010. n. Calisti
and others 2011. o. Chirdan and Uba 2006. p. van der Linde and others 2011.q. Bernier and others 2010. r. Larrazabal and others 2007. s. Bakshi and others 2007. t. Shah and others 2008.
u. Wickramasinghe, Lambabadusuriya, and Narenthiran 2001. v. Upadhyaya and others 2007. w. Singh and others 2012. x. Rayes 2010, y. Islam and Aziz 2011. z. Yang and others 2006.
aa. Narasimman, Nallusamy, and Hassan 2013. ab. Anwar-ul-Haq and others 2009. ac. Al-Salem and others 2006. ad. Sadler 2010. ae. Canfield and others 2006. af. Manson and others 2012.
ag. Askarpour and others 2012. ah. Ameh and Chirdan 2000b. ai. Uba and Chirdan 2003. aj. Toro, Rave, and Gomez 2010. ak. Sekabira and Hadley 2009. al. Saranrittichai 2008. am. Abdur-Rahman,
Abdulrasheed, and Adeniran 2011. an. Chiu and Hedrick 2008. ao. Ozdogan and others 2010. ap. Khemakhem and others 2012. aq. Adegboye and others 2002. ar. Abubakar and others 2011.
as. Rohana, Boo, and Thambidorai 2008.

Sekabira and Hadley 2009; Uba and Chirdan 2003). surgeons and surgery teams must be a fundamental goal
The misdiagnosis of anomalies as ­ better-known of ongoing and future programs to address the pediatric
infectious diseases, and added delays for invisible
­ surgical burden of disease.
anomalies, may further hinder the provision of timely,
appropriate services. These multifactorial delays are
a crucial h
­ urdle in treating both immediately and
­non-­immediately life-­threatening  anomalies. While ESTIMATING THE IMPACT OF PEDIATRIC
non-­immediately life-­ threatening anomalies often SURGERY ON THE GLOBAL BURDEN
require emergency interventions, the period before OF DISEASE
these conditions become emergencies can be better
used to dramatically improve outcomes. In the World Health Organization’s (WHO’s) most recent
Global Health Estimates, congenital anomalies constitute
2.1 percent of the total disease burden and rank eleventh
Scarcity of Skilled Surgeons in the causes of disease burden (WHO 2013b). Although
The scarcity of trained surgeons in LICs and LMICs also impressive, these figures are likely to be underestimates
significantly contributes to the burden: one pediatric because of the limited number of anomalies included in
general surgeon may serve millions of children (Chirdan the analysis and the difficulties in evaluating incidence,
and others 2010), and physicians performing pediatric morbidity, and mortality. Only six congenital surgical
surgery may have little or no pediatric surgery training conditions had disability weights in the 2004 estimates,
(Ekenze, Ibeziako, and Ezomike 2007; Mhando, Young, and congenital anomalies were not among the new dis-
and Lakhoo 2008). Whereas North America has an ability weights estimated in 2012 (Saloman and others
estimated one pediatric cardiac surgeon per 3 million 2012; WHO 2008).
people, Sub-Saharan Africa has one per 38 million peo- Some researchers have tried to fill the gap with
ple (Bernier and others 2010); 75 percent of the world’s evidence-based estimates of selected disability weights
population is estimated to have no access to cardiac sur- (Poenaru and others 2013). Of the conditions measured
gery (Hoffman 2013). Similarly, 33 percent of the world’s in the Global Health Estimates, cardiac anomalies rep-
population is covered by 5 percent of its neurosurgeons resent the greatest overall burden (table 8.2), and, along
(Warf 2013). Expanding the pool of specially trained with neural tube anomalies and cleft lip and palate, cause

Surgical Interventions for Congenital Anomalies 133

 32 million DALYs. Some 57 percent, or 18 million, of averted or cost-effectiveness. Yet, these foundational stud-
these DALYs are estimated to be surgically preventable if ies have provided compelling evidence that pediatric sur-
outcomes in HICs could be achieved in LICs and LMICs gery is a cost-effective intervention with the potential to
(Higashi, Barendregt, and Vos 2013). These anoma- avert more than 67 percent of the DALYs associated with
lies typify the reservoir of unmet need that congenital congenital anomalies (Corlew 2010; Higashi, Barendregt,
anomalies in LICs and LMICs create (table 8.3). and Vos 2013; Ozgediz and Poenaru 2012; Poenaru 2013;
Only a small body of literature evaluates the poten- Wu and Poenaru 2013; Wu, Poenaru, and Poley 2013).
tial of surgery to reduce this burden in terms of DALYs Favorable outcomes have been reported in HICs for such
conditions as anorectal malformations (ARMs) and con-
Table 8.2  Burden of Disease due to Congenital genital diaphragmatic hernia (Poley and others 2008).
Anomalies In LICs and LMICs, the human capital approach to cleft
lip and palate repair (see ­chapter 21) has provided very
Anomaly DALYs (thousand) YLDs favorable cost-­effectiveness analysis estimates. An exten-
Cardiac 20,760 565 sion of this methodology to treatment for c­ongenital
Neural tube 10,075 759 swelling of the brain in Uganda has also yielded favorable
results, at a cost of US$59 to US$126 per DALY averted
Down syndrome 2,939 1,225
(Warf and others 2011). Surgical repair of congenital
Cleft lip 709 254 inguinal hernias in Uganda has been estimated to have
Other chromosomal 2,941 694 an incremental cost-­effectiveness of US$12 per DALY
Other congenital 20,272 1,835 averted (Eason and others 2012). Another report from
Total 57,696 5,332
Cambodia estimates a cost-effectiveness of US$99 per
DALY averted over three months for reconstructive sur-
Source: WHO 2013b.
Note: DALYs = disability-adjusted life years; YLDs = years living with disability. gery for an array of anomalies (Rattray and others 2013).

Table 8.3  Prevalent Congenital Anomalies and Avertable Disease Burden

Congenital anomaly Brief description Treatment opportunities
Cardiac anomalies Most prevalent anomalies Backlog of 1 million to 2 million children need congenital cardiac surgery
Incidence: Approximately 8 per 1,000 in India.
births Requires relatively resource-intensive treatment compared with other
Most common: Ventricular septal defect anomalies; estimated US$2,500 per operation in some programs
(hole between the lower chambers of (Hoffman 2013).
the heart) Cost containment and capacity-building strategies have been described
(Rao 2007).
Neural tube defects Incidence: Nearly 1 per 1,000 births Preventable through folate supplementation, a major public health strategy
Most common: Spina bifida in many LICs and LMICs.
ETV is an innovative, sustainable strategy to treat associated
hydrocephalus, or swelling of the brain, with favorable results compared
with traditional treatment. Longer-term follow-up confirms the feasibility
and effectiveness of community-based strategies for ETV (Warf and others
2011; Warf 2011).
Cleft lip and palate Incidence: 1 per 700 live births; slightly Global backlog of unrepaired cleft cases is between 400,000 and 2 million
higher in some regions, like Sub- cases.
Saharan Africa (Poenaru 2013) Guidelines suggest that cleft lip should be repaired in the first six months
Approximately 25 percent of cases of life; cleft palate, in the first year to 18 months. Average age at time of
associated with other anomalies repair is nearly age 10 years in Sub-Saharan Africa (Poenaru 2013).
Cleft lip may require a single corrective operation.
Approximately 20 percent of palate repair cases may require subsequent
surgery; postoperative speech therapy is essential (Semer 2001).
Sources: Hoffman 2013; Poenaru 2013; Rao 2007; Semer 2001; Warf and others 2011; Warf 2011.
Note: ETV = endoscopic third ventriculostomy; LICs = low-income countries; LMICs = lower-middle-income countries.

134 Essential Surgery

 The benefits of improved pediatric surgical services Africa and constitute a significant clinical load (Calisti
in averted morbidity and cost extend across the lifespan. and others 2011; Moore and others 2008). HD has been
Treating congenital disease at its inception may result in associated with a number of congenital syndromes and
a significantly greater reduction in the burden of disease. anomalies, and may have various genetic causes (Amiel
The following sections present examples of anoma- and others 2008). The incidence of HD is comparable to
lies that are prime targets for intervention. Because of that of ARMs (Coran and others 2012) and is one of the
the lack of cost-effectiveness data, these anomalies are leading causes of pediatric intestinal obstruction in LICs
presented as case studies that highlight the preventable and LMICs (Adeyemi 1989; Ameh and Chirdan 2000a;
burden of disease and the potential of low-cost mea- Saha and others 2012). Hidden mortality and traditional
sures adapted to low-resource settings (i.e., low-income health practices (for example, enemas) mask prevalence
­countries [LICs] and LMICs) to substantially improve (Bandre and others 2010), suggesting that the burden of
outcomes. Further research is urgently needed to develop disease may be significantly underestimated.
and evaluate cost-effective treatment programs to take
advantage of the substantial DALY upside of treating Diagnosis and Treatment in LICs and LMICs.  ARMs
congenital anomalies. Congenital heart conditions are are usually diagnosed on physical examination. HD is
discussed in more detail in volume 5, Cardiovascular, not visually identifiable, so must be diagnosed based on
Respiratory, Renal, and Endocrine Disorders. the symptoms—feeding intolerance, vomiting, abdom-
inal distension, delayed passage of the meconium (the
first stool passed by a newborn) and severe neonatal
Case Study 1: Congenital Colorectal Disease— intestinal infection, or enterocolitis (Amiel and ­others
Anorectal Malformations and Hirschprung’s Disease 2008). Meconium passage may serve as a valuable screen-
Description.  ARMs and Hirschsprung’s disease (HD) ing tool for HD in HICs, LICs, and LMICs; 95 ­percent of
are two of the most prevalent congenital anomalies. children with HD do not pass the meconium within the
first 24 hours of life, while only 1 percent of children
• ARMs are physical anomalies that prevent the passage without HD experience a comparable delay.
of fecal matter through a distinct anus. Examples HD is definitively diagnosed via rectal biopsy1 (Amiel
include absence of an anus or fusion of the anus to and others 2008), but diagnostic ability may be limited
other openings in the body (for example, the urethra). in LICs and LMICs because of the cost of biopsy anal-
• HD is a functional obstruction of the bowel caused ysis and the scarcity of pathology services (Bandre and
by the absence of the nerve cells needed to stimulate others 2010). Many practitioners are forced to provide
normal contractile movement of the bowel. If food definitive treatment without confirmation of the HD
matter cannot move through the bowel, material diagnosis. Analytic protocols for biopsy specimens can,
collects in the preceding bowel and dilates it, causing however, be adapted to the resources of medical lab-
megacolon. The intestinal tract may perforate, caus- oratories in LICs and LMICs (Babu and others 2003;
ing widespread infection and death. Poenaru and others 2010), and inexpensive radiography
can replace costly endoscopic technologies in preopera-
HD and some ARMs are not immediately life threat- tive planning (Pratap and others 2007).
ening when partial passage of fecal material is possible. In LICs and LMICs, late presentation for nonemer-
For example, female children in LICs and LMICs with gency c­ongenital colorectal disease is the norm. More
the most common female ARM—vestibular fistula, than 60 percent of patients with HD present late, as chil-
whereby the rectum opens into the vagina—often dren, adolescents, and even adults (Ameh and Chirdan
remain undiagnosed until much later in life. Untreated, 2000a; Poenaru and others 2010; Sharma and Gupta
however, non-immediately life-threatening conditions 2012; Vincent and Jackman 2009). Delayed diagnosis
can lead to substantial morbidity and eventual mortality results from a web of interacting societal, cultural, and
due to intestinal rupture. socioeconomic factors that delay the diagnosis of other
The etiology of ARMs is unclear, but both genetic anomalies detailed in this chapter. Presentation and diag-
and environmental factors have been implicated (Davies, nosis are delayed by geographical and financial barriers
Creighton, and Wilcox 2004). The incidence of ARMs is to care, social taboo, cultural norms (for example, routine
cited as one per 3,000 to 5,000 live births (Chalapathi and traditional enemas), lack of awareness among medical
others 2004; Chowdhary and others 2004; Eltayeb 2010), personnel in first- and second-level facilities, inaccu-
but this incidence varies with ethnicity and geography rate medical advice, and failed or unwarranted proce-
(Moore and others 2008; van Heurn and others 2002). dures at other medical facilities (Al-Jazaeri and ­others
ARMs are reportedly more common in Sub-Saharan 2012; Bandre and others 2010; Ekenze, Ngaikedi, and

Surgical Interventions for Congenital Anomalies 135

 Obasi 2011; Sharma and Gupta 2012; Sinha and ­others caring for children with long-term colostomies has not
2008; Vincent and Jackman 2009). Delayed diagnosis of yet been well captured.
ARMs leads to severe morbidities and elevated mortality, Primary repair reduces the number of s­urgeries,
which are further heightened for infants in rural areas minimizing treatment costs and averting colostomy-­
­
where delayed diagnosis is coupled with subsequent related morbidity and mortality. Good outcomes in LICs
transport to distant medical facilities (Adejuyigbe and and LMICs have been reported (Adeniran and Abdur-
others 2004; Chalapathi and others 2004; Eltayeb 2010; Rahman 2005; Elhalaby 2006; Ibrahim 2007; Osifo and
Turowski, Dingemann, and Giilick 2010). Okolo 2009; Pratap and others 2007), with mortality
Delayed presentation of HD is characterized by rates rivaling those in HICs (Ibrahim 2007; Osifo and
chronic constipation, abdominal distension, bowel Okolo 2009). However, the prevalence of delayed diag-
loops visible through the abdominal wall, failure to nosis and treatment render routine primary repair
thrive, anemia, malnutrition, and fecal impaction risky. Reducing delays is key to relieving the preventable
(Al-Jazaeri and others 2012; Coran and Teitelbaum burden of congenital colorectal disease. Suggestions
2000; Ekenze, Ngaikedi, and Obasi 2011; Frykman and include the following (Adeniran and Abdur-Rahman
Short 2012; Prato and others 2011; Sharma and Gupta 2005; Al-Jazaeri and others 2012; Ameh and others 2006;
2012). Some patients present with bloody diarrhea due Ekenze, Ngaikedi, and Obasi 2011; Olivieri and others
to HD-associated enterocolitis, the leading cause of 2012; Peyvestah 2011; Poenaru and others 2010):
HD-related morbidity.
In HICs, mortality for ARMs is negligible. In LICs • Increase the number of third-level facilities (major
and LMICs, mortality reaches 20 percent to 30 percent hospitals offering a full spectrum of services)
(Adejuyigbe and others 2004; Adeyemi 1989; Archibong • Increase participation of existing third-level facilities
and Idika 2004; Chalapathi and others 2004; Ekenze, in the training of community health centers in diag-
Ibeziako, and Ezomike 2007; Peyvasteh and others 2011; nosis and preoperative management
Tefera, Teka, and Derbew 2007). The mortality rate for HD • Improve training at the level of the TBA, primary care
is less than 5 percent to 10 percent in HICs (Rescorla and provider, and community health worker
others 1992; Swenson 2002) but jumps to 20 percent to • Modify medical education curricula to encompass
43 percent in LICs and LMICs (Adeyemi 1989; Ameh and ARMs and HD
Chirdan 2000a; Bandre and others 2010; Ekenze, Ibeziako, • Target surgeons at first-level hospitals to perform
and Ezomike 2007). These disparities are linked to delayed colostomies with available resources
diagnosis and treatment, sepsis,2 and the absence of crit- • Institute low-cost modifications to standard repair
ical care when patients present with advanced complica- procedures
tions (Adeniran and Abdur-Rahman 2005; Chalapathi
and others 2004; Chowdhary and others 2004). Substantial loss to follow-up after colostomy for-
Both ARMs and HD can be treated with either pri- mation in LICs and LMICs remains a challenge, and
mary (one-step) or staged (multistep) surgical repair innovative approaches to ensuring patient return or
(Coran and others 2012). These operations generally local follow-up in home regions must be developed to
do not require intensive postoperative care, but they overcome barriers to continuity of care.
do require general anesthesia. Delays in diagnosis often
preclude primary repair because these repairs cannot be
performed when the bowel is grossly distended. In such Case Study 2: Abdominal Wall Defects—Omphalocele
cases, a colostomy3 is a life-saving first step in staged and Gastroschisis
repair. Colostomy complications, however, are common Description.  Omphalocele and gastroschisis are abdom-
in HICs, LICs, and LMICs (Chalya and others 2011; inal wall defects in which the internal organs, or viscera,
Chandramouli and others 2004; Patwardhan and others protrude through the abdominal wall. In omphalocele,
2001). The risks are compounded in LICs and LMICs by the gut and other abdominal organs, such as the liver,
the prohibitive cost of colostomy bags, cultural preju- spleen, and gonads, protrude through the abdominal wall
dice, and limited parental understanding (Adeniran and into a membranous sac. In gastroschisis, no sac is present
Abdur-Rahman 2005; Chandramouli and others 2004; and usually only the gut protrudes from the abdomen
Olivieri and others 2012). Although simple, inexpensive (Coran and others 2012). Patients with omphalocele can
treatments may ameliorate some of these challenges be fed if the sac is intact; those with gastroschisis cannot
(Chalya and others 2011), a significant burden remains. be fed and quickly perish without treatment.
Many children live for years with colostomies without The incidence of omphalocele is approximately 2.18
receiving definitive repair. The burden on families of to 5 per 10,000 live births; that of gastroschisis is 3.3 to

136 Essential Surgery

5 per 10,000 live births (Canfield and others 2006; Coran necessitating the use of intravenous nutrition, often
and others 2012; Sadler 2010; Stoll and others 2001). The not available in poorer countries. It has been suggested
incidence of gastroschisis is on the rise and varies geo- that in-house birth at centers equipped to medically
graphically (Andrew, Holland, and Badawi 2010; Arnold manage patients with abdominal wall defects is essential
2004; Benjamin and others 2010; Castilla, Mastroiacovo, to improving outcomes, as is improving training for
and Orioli 2008; Laughon and others 2003; Loane, Dolk, transport personnel, obstetricians, and primary care
and Bradbury 2007; Vu and others 2008). physicians (Sekabira and Hadley 2009). Training TBAs
Risk factors for omphalocele include chromosomal to recognize omphalocele and gastroschisis, and to place
anomalies, very young and very advanced maternal children from the shoulder down in clean polyethylene
age, lack of multivitamin and folic acid supplementa- bags to protect the bowels during transport, is another
tion during pregnancy, and maternal history of fevered viable option for reducing complications associated with
illness (Botto and others 2002; Botto, Mulinare, and delayed presentation (Ameh and Chirdan 2000b).
Erickson 2002; Frolov, Alali, and Klein 2010; Mills and Surgical procedures commonly used in HICs may
others 2012). Gastroschisis has not yet been associated increase mortality in LICs and LMICs, largely as a result
with any particular genes. Risk factors for gastroschisis of infection and sepsis (Ameh and Chirdan 2000b;
include young maternal age, low socioeconomic status, Uba and Chirdan 2003); nonsurgical or altered sur-
poor nutrition, and lack of vitamin supplementation gical procedures may be preferable. Uba and Chirdan
during pregnancy (Coran and others 2012). Mothers in (2003) report the successful treatment of unruptured
LICs and LMICs are more likely to have children at both omphalocele using daily application of scab-inducing
younger and more advanced ages, to have limited family topical ointments, leading to eventual skin growth over
planning knowledge and resources, and to suffer nutri- the defect. Bedside placement of a silo bag followed by
tional deficiencies. Accordingly, their children are likely gradual reduction of the viscera into the abdomen and
to be at greater risk for omphalocele and gastroschisis. sutureless repair is a potentially cost-effective treatment
strategy worthy of additional evaluation in LICs and
Diagnosis and Treatment in LICs and LMICs. Ultra­ LMICs. Several studies have investigated low-cost alter-
sonography, a low-cost technology once in place, can detect natives to silos, including transfusion bags and female
omphalocele and gastroschisis before birth with high suc- condoms (Bustorff-Silva and others 2008; Miranda
cess (Richmond and Atkins 2005). This technology can and others 1999). The female condom is particularly
inform decisions about pregnancy termination and mode intriguing because its use requires no sutures, surgery,
of delivery, facilitating improvements in outcomes. or anesthesia; allows for easy preoperative observation;
Several effective surgical strategies for omphalocele and allows gravity to gradually move the viscera into the
have been described. Gastroschisis necessitates greater abdomen (Bustorff-Silva and others 2008).
attention to heat loss and moisture preservation because Once infants with omphalocele or gastroschisis sur-
a larger surface area of viscera is exposed. Historically, vive the neonatal period, there is little to no associated
primary closure has been the treatment of choice in that disability or mortality. These anomalies are highly treat-
it limits damage incurred by exposure. Recent studies able and thus potentially highly DALY averting.
have shown that using a silo (a moisture-retaining bag
that holds the viscera before they are returned to the
abdomen) and postponing closure for hours to days can Case Study 3: Clubfoot
be equally effective (Coran and others 2012). Description.  Talipes equinovarus, or clubfoot, is a com-
Treatment is highly effective in HICs; multiple series plex congenital anomaly in which the entire foot is rotated
report survival rates of 70 percent to 95 percent for inward (van Bosse 2011). Clubfoot may be idiopathic—
omphalocele and 90 percent or greater for gastroschisis without an identifiable cause—or associated with other
(Coran and others 2012). In comparison, the mortality congenital anomalies. The etiology is unknown but likely
associated with omphalocele and gastroschisis in LICs involves genetic and environmental factors (Dobbs and
and LMICs is shockingly elevated, with survival falling Gurnett 2009). According to estimates from HICs, the
to less than 20 percent in some studies (Askarpour incidence is approximately 1 per 1,000 live births (Coran
and others 2012; Richmond and Atkins 2005). Delayed and others 2012). However, incidence varies widely with
presentation plays a key role because hypothermia and ethnicity, and reports from LICs and LMICs have shown
gangrenous bowel may develop in the interim (Ameh that incidence may be about 1 per 500 live births (Dobbs
and Chirdan 2000b; Sekabira and Hadley 2009; Uba and Gurnett 2009; Mkandawire and Kaunda 2002);
and Chirdan 2003). An additional challenge with gastro- 80 percent of children with untreated clubfoot are born
schisis is that bowel function can be impaired initially, in LICs and LMICs (WHO 2013a). Untreated clubfoot

Surgical Interventions for Congenital Anomalies 137

 leads to lifelong disability, social stigmatization, and performed by health and allied health professionals4
decreased economic s­ elf-sufficiency in adulthood (Alavi (Janicki and others 2009; Lavy and others 2007; Mayo
and others 2012; Lourenço and Morcuende 2007). and others, n.d.), an advantageous attribute in contexts
with few orthopedic surgeons. It requires no specialized
Diagnosis and Treatment in LICs and LMICs. The surgical facilities and produces a functional foot (Gupta
most effective treatment for idiopathic clubfoot, and others 2008).
the Ponseti method, is largely nonsurgical. We have Treatment programs using the Ponseti method have
included this anomaly for analysis because it exempli- been set up in many LICs and LMICs, with success rates
fies the importance of holistically approaching surgical that regularly approximate those seen in HICs (Goksan
disease to use both low-cost surgical and nonsurgical and others 2006; Gupta and others 2008; Jawadi 2010;
interventions to reduce the burden of disease. Makhdoom and others 2011; Mendez-Tompson and
The Ponseti method, in which a series of casts are others 2012; Panjavi and others 2012; Sarrafan and
applied between incremental manipulations of the foot others 2012). The Ponseti method is also successful in
by trained practitioners, is successful in up to 98 percent treating neglected clubfoot in LICs and LMICs, yielding
of patients (WHO 2013a). The Achilles tendon is often superior outcomes and incurring significantly lower
cut through the skin to correct lingering deformity, and costs than purely surgical interventions (Adegbehingbe
corrective braces are worn for several years to prevent and others 2010; Hegazy, Nasef, and Abdel-Ghani 2009;
relapse (Dobbs and others 2000). The Ponseti method is Spiegel and others 2009).
also successful in treating neglected idiopathic clubfoot, The success of national clubfoot programs (box 8.1)
in which the anomaly is not treated before walking age provides strong evidence for the utility of task-­shifting,
(Lourenço and Morcuende 2007). If surgery alone is that is, training paramedical practitioners to perform
used to correct this aggravated form of the deformity, select health care tasks. Nonphysician practitioners
functionality is low and may degrade into crippling pain trained in orthopedics and the Ponseti method can
and weakness in adolescence and adulthood (Gupta and achieve results comparable to those of surgeons in
others 2008). HICs (Tindall and others 2005). Collaborations among
The Ponseti procedure is well suited to use in LICs diverse partners, including nongovernmental organiza-
and LMICs; it is a low-cost intervention that can be tions, ministries of health, and academic institutions,

Box 8.1

The Uganda Sustainable Clubfoot Care Project

Clubfoot, the most common cause of locomotor in serial casting in rural hospitals and medical
disability in low-income countries leads to pro- officers in tenotomy
found impairments in activities of daily life, social • Development and distribution of locally made
exclusion, and abandonment. After several years of orthoses, or mechanical devices (for example,
pilot intervention, the Uganda Sustainable Clubfoot braces) to provide support and correct alignment
Care Project was implemented in 2000. Essential of the clubfoot.
elements include the following:

• Development of a national strategic plan address- In 2006 and 2007, 872 children were treated, nearly
ing all levels of the health system 800 providers were trained, and services were made
• Endorsement by the Ministry of Health and available in 21 hospitals. Using this experience in
incorporation into the National Health Policy Uganda and a similar program in Malawi as a basis,
• Community awareness campaigns interventions have been implemented in 10 other
• Sensitization of maternity units via education countries; early two-year follow-up data suggest
• Training: Ponseti method trainers at the national similarly successful results.
hospital to train nonphysician orthopedic officers Source: Pirani and others 2009; Tindall and others 2005.

138 Essential Surgery

are instrumental for increasing the number of trained The strengths and weaknesses of these strategies are
paramedical practitioners, and thereby the availability ­summarized in table 8.4. It is critical that local exper-
of treatment (Owen and others 2012). To achieve these tise and buy-in be integrated into all efforts to increase
goals, it is critical to implement a coordinated, standard- the accessibility of pediatric surgery so as to create
ized program to decentralize care, and to integrate edu- ­sustainable systems that increase long-term capacity and
cation, awareness, and service delivery into the public take advantage of the substantial potential intellectual,
health sector. ­creative, and personnel resources in LICs and LMICs.

Strategy 1. Cultivating Treatment Centers in LICs and

IMPLEMENTATION AND SURVEILLANCE LMICs
PRIORITIES Recent humanitarian efforts have favored establishing
sustainable surgical centers in LICs and LMICs, staffed
Addressing the Burden of Disease of Congenital by either local or foreign personnel. This approach max-
Anomalies imizes the number of children treated and enables local
Strategies to increase the accessibility of surgical care for trainees to learn to practice in their future professional
children with congenital anomalies include development environments, preventing later struggles to adapt pro-
of treatment centers for specific conditions (niche hospi- tocols learned abroad to local resources (Larrazabal and
tals), short-term surgical missions, partnerships to train others 2007). The entire surgical team can be concur-
local providers, and the transporting of patients to other rently trained, prepping both the surgeon and the center
countries for care. Interventions at the policy level may to operate independently (Larrazabal and others 2007;
also play a role, as may novel tools such as telemedicine. Loisance 2012).

Table 8.4  Increasing the Accessibility of Pediatric Surgery

Strategy Advantages Disadvantages
Treatment centers in Creates infrastructure and expertise to broadly affect Streamlined centers often not integrated with local training
LICs and LMICs the burden of disease caused by congenital anomalies programs and public health care systems
Streamlines care if center focuses on one pathology or
organ system
Provides treatment in home countries
Surgical missions Minimizes costs and culture shock for patients Episodic
Streamlines care if missions focus on one condition Limited time window restricts number of patients treated
Offers opportunity to train local personnel Pressure to deliver care in limited period may restrict training
Are well suited for nonemergency conditions with efforts
backlogs Follow-up limited
Integration with existing services may be limited
Not designed to treat emergency life-threatening conditions
Academic partnerships Provides coordinated interface for students and Not as focused on delivery of care
professionals from HICs, LICs, and LMICs to learn from Research may not be truly collaborative
and collaborate with each other
Potential for medical tourism by HIC practitioners, especially
Opportunity for training and resource sharing if training not provided to LIC and LMIC partners
Facilitates scholarly approach to intervention and Financial sustainability may be limited by availability of
evaluation institutional funding
Human resources Task-shifting increases health care access Necessitates rigorous regulation and standardization
and policy changes
Telemedicine
table continues next page

Surgical Interventions for Congenital Anomalies 139

 Table 8.4  Increasing the Accessibility of Pediatric Surgery (continued)
Strategy Advantages Disadvantages
Potentially reduces transport risks and costs No opportunity for hands-on training
Builds local capacity through consultation
Improves communication between different points of
health care access
Health care delivery Increases level of care compared with local systems Costly
outside of LICs and Benefits only a few patients
LMICs
Does not contribute to developing capacity in LICs and LMICs
Note: HICs = high-income countries; LICs = low-income countries; LMICs = lower-middle-income countries.

Successful centers have developed from inter- Strategy 2. Surgical Missions

national partnerships, foreign humanitarian initia- The short-term surgical mission, also commonly referred
tives, and home-grown efforts in LICs and LMICs to as humanitarian assistance or a volunteer trip, is an
(Bode-Thomas 2012), with evidence that continuous established health care delivery model that is becoming
support through development programs can build increasingly popular. Individual providers or organized
pediatric surgery capacity. Networks of faith-based teams travel from HICs to deliver surgical care in LICs
hospitals also ­ provide specialized surgical services, and LMICs (Martiniuk and others 2012). Smile Train
such as the CURE network, which focuses primarily and Operation Smile, the two largest global cleft lip and
on neurosurgery and orthopedics; CBM International, palate charities, exemplify two different models of suc-
which supports an array of surgical services; and the cessful, ­long-­running surgical missions. Operation Smile
Pan-African Academy of Christian Surgeons network, primarily funds teams from HICs to provide short-term
which has developed training programs in association health care delivery and training in LICs and LMICs;
with BethanyKids, a faith-based pediatric surgical Smile Train5 funds local providers to offer outreach and
organization. Surgical mission–­ oriented organiza- training (Poenaru 2013). In some regions, these teams
tions may also develop treatment centers, as has been provide the only surgical services for children (Walker
done by Operation Smile, a mission program special- and others 2010). In recent years, Operation Smile has
izing in cleft lip and palate repairs (Patel and o ­ thers also developed treatment centers as a more sustainable
2013). approach (Magee and others 2012).
Treatment at these centers can be tailored to available Sustainability, follow-up for postsurgical compli-
resources. Less-expensive, but effective, diagnostic and cations, and integration with existing health systems
therapeutic modalities can be used. Simple, palliative are significant challenges for the inherently episodic
surgeries may predominate in the neonatal period if surgical mission model. Perhaps most important, sur-
early definitive repair is too risky. Cheaper surgical gical missions are better suited for “prevalent” rather
materials are produced by countries like Brazil and than “incident” conditions. Prevalent conditions incur
China; medical equipment companies may be persuaded increasing morbidity, whereas incident conditions are
to make donations; and some disposable surgical materi- surgical emergencies. A greater proportion of the burden
als can be sterilized and reused (Rao 2007; Senga and of disease may be averted by targeting emergency con-
others 2013). ditions, but emergency treatment cannot be improved
The few cost-effectiveness analyses that have been without improving capacity at the systems level (Bickler
published support the feasibility of developing local and others 2010).
treatment centers. For example, at the CURE Children’s
Hospital in Mbale, Uganda, children with hydrocephalus
are treated at an estimated cost of US$59 to US$126 per Strategy 3. Partnerships between Academic
DALY averted, with US$3 million to US$5 million saved Organizations and Development Programs
to Uganda, and US$5 million to US$188 million saved Academic organizations have increasingly focused
based on statistical calculations of the value of a life on augmenting surgical capacity in LICs and LMICs
(Warf and others 2011). through partnerships (Calland and others 2013; Qureshi

140 Essential Surgery

and others 2011). Some organizations subsidize aca- pediatric surgical conditions has not been specifically
demic surgeons, who then deliver care, train providers, analyzed.
and conduct research in LICs and LMICs. Partnerships
also provide mechanisms by which practitioners from
LICs can obtain foreign training in middle-income Strategy 5. Telemedicine
countries (MICs) (for example, a Sub-Saharan African Telemedicine6 may increase the accessibility of limited
practitioner training in India) or HICs. Training outside surgical specialists to large populations requiring care.
of the home environment has the disadvantage that the Chaotic roads and a lack of medical transport compli-
trainee may not return for practice. Funding the training cate the great distances that patients in LICs and LMICs
of local practitioners by underwriting the presence of must travel to reach care. Children seeking treatment
visiting surgeons in partnered development programs may present in critical and sometimes unsalvageable
may be an effective alternative. For example, during condition following such physiologically stressful transit
the course of a four-year project funded by the Italian (Rao 2007). With telemedicine, physicians at local or
Cooperation in Eritrea, local Eritrean surgeons achieved regional medical centers can interact with experts at
independence and favorable outcomes in the treatment centralized third-level facilities to guide patient treat-
of a wide range of congenital anomalies (Calisti and ment, potentially circumventing the need for patients to
others 2011). Overall, the scope of activities undertaken undergo life-threatening journeys. The cost burden of
by academic organizations in these partnerships varies travel and treatment for families is decreased, and unspe-
widely, and outcomes of many permutations of partner- cialized providers can receive training from specialized
ship are not yet well characterized. peers (Sekar and Vilvanathan 2007). Telemedicine can
also link medical centers for educational or research
purposes (Hadley and Mars 2008). This developing
Strategy 4. Human Resources and Policy Changes technology has the potential to beneficially decentralize
Policy changes, such as the provision of free health care specialized care and education in LICs and LMICs.
to children, may increase access to pediatric surgery
(Groen and others 2013). The expansion of training pro-
grams is key to ensuring that children with anomalies are Strategy 6. Health Services Delivery Outside of
diagnosed in a timely manner and can access appropri- Local Systems
ate surgical services. Regional professional societies offer Families may seek care in foreign treatment centers,
a limited number of training spots and scholarships, and funded out of pocket, through community fund-­
training programs need support (Elhalaby and others raising, or by humanitarian organizations. Increased
2012). More training positions are required to meet the access to advanced treatment methods saves lives
need for trained pediatric surgeons and the interest level that would otherwise be lost. However, transport-
of general surgery trainees. To date, only limited analy- ing c­hildren to foreign centers limits the number of
ses of the cost-effectiveness of such programs have been children treated. Because the cost of surgery abroad
conducted. often surpasses a family’s annual income in LICs and
Specially trained paramedical practitioners may be a LMICs, even the ­highest-earning families must rely on
viable solution to the scarcity of providers, as demon- limited governmental and nongovernmental agency
strated by the success of clubfoot programs; however, funds (Sadoh, Nwaneri, and Owobu 2011). If patients
the scope of treatment may be limited to select condi- require multiple surgeries, financial sponsors must
tions (Mayo and others, n.d.; Pirani and others 2009; decide whether to perform the surgery at all, abstain
Tindall and others 2005). The scope of practice for a from follow-up s­urgeries, or fly the patient back for
pediatric general surgeon includes congenital anoma- additional ­surgeries. If patients die abroad, the fami-
lies, acquired surgical diseases, and a high proportion lies must ­wrestle with substantial financial and emo-
of emergencies. This breadth has made it challenging to tional challenges in coordinating burial arrangements
design and implement intervention packages in hospitals (­Bode-Thomas 2012). Furthermore, this approach does
and health care systems. Other surgical providers may do not build local surgical capacity. The social and eco-
their best to meet the need, but the shortfall is tremen- nomic costs for patients and families who seek care
dous. Pediatric anesthesia providers are similarly few, as abroad are likely significant but have not been esti-
are trained pediatric neurosurgeons, cardiac surgeons, mated; an estimate may provide incentives for govern-
orthopedic surgeons, and other surgical subspecialists. ments and funders to invest in local care.
Although task-shifting has been promoted to meet the These strategies are not mutually exclusive; they may
need for essential surgery in adults, its applicability to coexist or evolve into different models.

Surgical Interventions for Congenital Anomalies 141

 Although the development of health care capacity (table 8.5). Robust data from LICs and LMICs are sparse;
within LICs and LMICs is crucial to the long-term therefore, the following recommendations—based on
reduction of the burden of disease associated with con- the available literature—should be understood as the best
genital anomalies, other intervention strategies provide available at this time. Future research will undoubtedly
additional and immediate opportunities to improve lead to more precise, substantiated recommendations.
pediatric surgical care and outcomes.
• The village health center should have health pro-
viders trained to identify anomalies. In Tanzania,
ACTION PLAN educating TBAs and using a birth card to register
and record birth data are being evaluated as tools
Clinical Intervention at Every Level for improving identification of treatable anomalies
Given that platforms for delivery vary by setting, we (Norgrove Penny, personal communication) and may
propose only general guidelines for congenital anomalies be useful in other practice settings. Public education

Table 8.5  Pediatric Surgery Capacity in an Ideal System

Village health First-level Second-level Third-level
Capacity center hospital hospital hospital
Airway management, fluid replacement, bleeding control, Xa X X X
antibiotic therapy
Blood transfusion Xa X X
Tracheal tube X a
X X
Local anesthesia X X X X
Spinal and general anesthesia X X X
Pediatric hernia X X X
Pediatric hernia (infant) Xa X
Umbilical hernia X X X
Pyloric stenosis Xa
X
Colostomy Xa X X
Neonatal bowel obstruction (atresia, stenosis, malrotation) X
Tracheoesophageal fistula repair Xa
Clubfoot X X X
Cleft lip X X X
Anorectal malformations or Hirschsprung’s disease (first stage; X X X
often colostomy)
Anorectal malformations or Hirschsprung’s disease (definitive X X
treatment)
Abdominal wall defects Xa X
Hydrocephalus Xa X
Congenital cardiac anomalies Xa
X
Spina bifida X
Bladder extrophy X
Undescended testicles X X
Hypospadias X
Note: “Capacity” is defined as trained staff available 24 hours, seven days a week, with adequate equipment and supplies.
a. Capacity should already be there but usually is not.

142 Essential Surgery

 is important for raising awareness that congenital the mechanisms to strengthen systems for pediatric
anomalies are treatable and not a death sentence; this surgery.
vital information prompts families to seek care.
• The first-level hospital should have the ability to Metric Optimization. The quantitative metrics of
stabilize pediatric patients with surgical emergencies ­disease burden should be optimized. Although DALYs
and to definitively treat conditions for which the are an accepted metric, they are difficult to apply
capacity exists. In-depth guidelines have been sug- practically. Surgical backlogs can be calculated for
gested for first-level hospitals but have not yet been congenital anomalies and can be a useful advocacy tool
tested or validated (Bickler and Ameh 2011). for estimating the resources needed to treat prevalent,
• The second-level hospital should provide life-saving nonfatal anomalies. Improved measurements of the
surgical treatments, especially those that are part of burden imposed by delayed access to care have not yet
staged repair procedures, and should house at least been developed. In middle- and high-income countries,
one specialist surgeon with training in pediatric gen- many prevalent congenital anomalies are treated in the
eral surgery. first year of life; in LICs, they are never treated or are
• The third-level hospital must be able to provide treat- treated years later, after children have suffered unnec-
ment for a broader range of neonatal emergencies and essary complications. Many diseases with 100 percent
more complex urgent conditions. The availability of survivability in HICs result in death or permanent
specialist pediatric surgeons and anesthesia providers disability in LICs and LMICs. Improved measurement
with expertise in infants and children is critical. of disparities must be highlighted as an advocacy tool
for health equity.

Research Priorities Integration Model Evaluation.  Models for the integra-

Improved data collection and identification of dispari- tion of pediatric surgical services within existing child
ties will fuel advocacy and inform targeted intervention health initiatives should be evaluated. Large-scale child
programs for congenital anomalies. Research priorities health initiatives exist (such as the WHO’s Integrated
include the following: Management of Childhood Illness but have not his-
torically included surgical care. Providers of children’s
Data Expansion for Further Evaluation.  Epidemiology, surgical services share a general concern that if the par-
prevalence, and incidence of disease in various health ticular needs of children are not specifically addressed,
contexts need further evaluation. Epidemiology may then they are often neglected. Although congenital
vary locally, but additional data are needed (Bickler anomalies are sometimes considered noncommunicable
2000). Registries for selected anomalies may assist in diseases (as in the Global Burden of Disease study), the
improving surveillance (for example, via participation agenda of the noncommunicable disease movement has
in the International Clearinghouse for Birth Defects not addressed them. Furthermore, greater planning is
Surveillance and Research). The evaluation of hidden needed between networks of specialty organizations and
mortality and morbidity will better approximate the true providers treating a broad range of congenital anomalies
burden of disease. to collaborate where possible.

Capacity Assessment and Guideline Development.  Cost-Effectiveness Data Generation.  Cost-effectiveness

The capacity for pediatric surgical care at various levels data should be generated to evaluate and select models
of the health system must be assessed, and guidelines to improve access to care. Although cost-effectiveness
for minimum human resources and infrastructure for has been estimated for adult general surgery wards in
countries at different stages of development should selected hospitals (Gosselin, Thind, and Bellardinelli
be created. The WHO Tool for Situational Analysis to 2006), only one attempt has been made for pediatric
Assess Emergency and Essential Surgical Care includes surgical wards (Rattray and others 2013). Low-cost
only two items pertaining to pediatric surgical care, technological innovations and modification of surgical
and an alternative capacity tool has been proposed procedures hold great promise to improve periopera-
(Nacul and others 2013). This tool could be refined tive care (Hadley 2008). Cost-effectiveness analysis of
and further evaluated as it is piloted in different coun- training programs could also aid advocacy for greater
tries. Although surgical outreach programs can tackle resources for training.
the backlog of nonemergency conditions, emergency
conditions require development of the whole health Marketing and Advocacy Alignment.  Marketing and
system. More work is needed to define and develop advocacy should become more aligned. Congenital

Surgical Interventions for Congenital Anomalies 143

 anomalies vary greatly in scope and severity. Some REFERENCES
treatable visible anomalies have received greater empha-
Abdur-Rahman, L. O., N. A. Abdulrasheed, and J. O. Adeniran.
sis than those invisible anomalies for which it has been
2011. “Challenges and Outcomes of Management of
more difficult to engage donor programs for targeted Anterior Abdominal Wall Defects in a Nigerian Tertiary
support. Improved multidimensional measurements of Hospital.” African Journal of Paediatric Surgery 8 (2):
the burden may help to make children and families suf- 159–63.
fering from all treatable anomalies more visible to the Abubakar, A. M., M. A. Bello, D. Y. Chinda, K. Danladi, and
public health community. Development of innovative I. M. Umar. 2011. “Challenges in the Management of Early
strategies for this process is needed. versus Late Presenting Congenital Diaphragmatic Hernia
in a Poor Resource Setting.” African Journal of Paediatric
Surgery 8 (1): 29–33.
Adegbehingbe, O. O., L. M. Oginni, O. J. Ogundele,
A. L. Ariyibi, P. O. Abiola, and O. D. Ojo. 2010. “Ponseti
CONCLUSIONS Clubfoot Management: Changing Surgical Trends in
Nigeria.” Iowa Orthopaedic Journal 30: 7–14.
This chapter highlights the considerable burden of
Adegboye, V., S. Omokhodion, O. Ogunkunle, M. Obajimi,
disease associated with congenital anomalies and
A. Brimmo, and O. Adebo. 2002. “Experience with the
outlines key strategies for intervention. The conse- Management of Congenital Diaphragmatic Hernia at the
quences of nonintervention are readily apparent. It University College Hospital, Ibadan.” Nigerian Journal of
is both an economic and a moral imperative that Paediatrics 29 (2): 40–46.
global partners invest in pediatric surgery as a vital Adejuyigbe, O., A. M. Abubakar, O. A. Sowande,
component of reducing the burden of disease and O. S. Olayinka, and A. F. Uba. 2004. “Experience with
improving the public health and economic fortunes Anorectal Malformations in Ile-Ife, Nigeria.” Pediatric
of LICs and LMICs. Surgery International 20 (11–12): 855–58.
Adeniran, J. O., and L. Abdur-Rahman. 2005. “Late-Stage
Correction of Intermediate Imperforate Anus in Males.”
Pediatric Surgery International 21 (2): 88–90.
NOTES Adeyemi, D. 1989. “Neonatal Intestinal Obstruction in a
Developing Tropical Country: Patterns, Problems, and
The World Bank classifies countries according to four income Prognosis.” Journal of Tropical Pediatrics 35 (2): 66–70.
groupings. Income is measured using gross national income Agarwala, S., V. Bhatnagar, M. Bajpai, D. Gupta, and D. Mitra.
(GNI) per capita, in U.S. dollars, converted from local currency 1996. “Factors Contributing to Poor Results of Treatment
using the World Bank Atlas method. Classifications as of July of Esophageal Atresia in Developing Countries.” Pediatric
2014 are as follows: Surgery International 11 (5–6): 312–15.
Alavi, Y., V. Jumbe, S. Hartley, S. Smith, D. Lamping, and others.
• Low-income countries (LICs) = US$1,045 or less in 2013 2012. “Indignity, Exclusion, Pain and Hunger: The Impact
• Middle-income countries (MICs) are subdivided: of Musculoskeletal Impairments in the Lives of Children
• Lower-middle-income (LMICs) = US$1,046 to US$4,125 in Malawi.” Disability and Rehabilitation 34 (20): 1736–46.
• Upper-middle-income (UMICs) = US$4,126 to US$12,745 Al-Jazaeri, A., S. Al-Shanafey, M. Zamakhshary, W. Al-Jarbou,
• High-income countries (HICs) = US$12,746 or more E. Hajr, and others. 2012. “The Impact of Variation in
Access to Care on the Management of Hirschsprung
1. Rectal biopsy: A surgeon removes small samples of tissue Disease.” Journal of Pediatric Surgery 47 (5): 952–55.
from the patient’s rectum. These samples are subjected to Al-Salem, A. H., M. Tayeb, S. Khogair, A. Roy, N. Al-Jishi,
laboratory tests that lead to a diagnosis. and others. 2006. “Esophageal Atresia with or without
2. Sepsis: Severe widespread infection within the body that Tracheoesophageal Fistula: Success and Failure in 94 Cases.”
can lead to death. Annals of Saudi Medicine 26 (2): 116–19.
3. Colostomy: Temporary creation of an opening in the Ameh, E. A., and L. B. Chirdan. 2000a. “Neonatal Intestinal
abdomen that is connected to the intestine; fecal material Obstruction in Zaria, Nigeria.” East African Medical Journal
exits the opening into a colostomy bag. 77 (9): 510–13.
4. Allied health or paramedical practitioners: Nonphysician ———. 2000b. “Ruptured Exomphalos and Gastroschisis:
health professionals who provide supplementary or emer- A Retrospective Analysis of Morbidity and Mortality
gency health services. in Nigerian Children.” Pediatric Surgery International
5. Smile Train. “Smile Train Report Card.” http://www​ 16 (1–2): 23–25.
.­smiletrain.org/our-model/. Ameh, E. A., P. M. Mshelbwala, L. Sabiu, and L. B. Chirdan. 2006.
6. Telemedicine: Using technology to exchange informa- “Colostomy in Children—An Evaluation of Acceptance
tion between different locations to improve training or among Mothers and Caregivers in a Developing Country.”
clinical care. South African Journal of Surgery 44 (4): 138–39.

144 Essential Surgery

Amiel, J., E. Sproat-Emison, M. Garcia-Barcelo, F. Lantieri, Bickler, S. W., and H. Rode. 2002. “Surgical Services for
G. Burzynski, and others. 2008. “Hirschsprung Disease, Children in Developing Countries.” Bulletin of the World
Associated Syndromes, and Genetics: A Review.” Journal of Health Organization 80 (10): 829–35.
Medical Genetics 45 (1): 1–14. Bode-Thomas, F. 2012. “Challenges in the Management
Andrew, J. A., K. W. Holland, and N. Badawi. 2011. of Congenital Heart Disease in Developing Countries.”
“Gastroschisis: An Update.” Pediatric Surgery International In Congenital Heart Disease: Selected Aspects, edited by
26 (9): 871–78. P. Syamasundar Rao, 263–72. Rijeka, Croatia: Intech. http://
Anwar-ul-Haq, N. Akhter, Ubaidullah, Javeria, Samiullah, www.intechopen.com/books/congenital-heart-disease​
and others. 2009. “Factors Affecting Survival in Patients -selectedaspects/challenges-in-the-management-of​
with Oesophageal Atresia and Tracheo-Oesophageal Fistula.” -congenital-heart-disease-in-developing-countries.
Journal of Ayub Medical College Abbottabad 21 (4): 129–33. Botto, L. D., J. D. Erickson, J. Mulinare, M. C. Lynberg,
Archibong, A. E., and I. M. Idika. 2004. “Results of Treatment and Y. Liu. 2002. “Maternal Fever, Multivitamin Use,
in Children with Anorectal Malformations in Calabar, and Selected Birth Defects: Evidence of Interaction?”
Nigeria.” South African Journal of Surgery 42 (3): 88–90. Epidemiology 13 (4): 485–88.
Arnold, M. 2004. “Is the Incidence of Gastroschisis Rising in Botto, L. D., J. Mulinare, and J. D. Erickson. 2002. “Occurrence
South Africa in Accordance with International Trends? A of Omphalocele in Relation to Maternal Multivitamin
Retrospective Analysis at Pretoria Academic and Kalafong Use: A Population-Based Study.” Pediatrics 109 (5):
Hospitals, 1981–2001.” South African Journal of Surgery 904–08.
42 (3): 86–88. Bustorff-Silva, J. M., A. F. S. Schmidt, A. Goncalves, S. Marba,
Askarpour, S., N. Ostadian, H. Javaherizadeh, and S. Chabi. and L. Sbragia. 2008. “The Female Condom as a Temporary
2012. “Omphalocele, Gastroschisis: Epidemiology, Survival, Silo: A Simple and Inexpensive Tool in the Initial
and Mortality in Imam Khomeini Hospital, Ahvaz-Iran.” Management of the Newborn with Gastroschisis.” Journal
Polski Przeglad Chirurgiczny 84 (2): 82–85. of Maternal-Fetal and Neonatal Medicine 21 (9): 648–51.
Babu, M. K., U. Kini, K. Das, A. Alladi, and A. D’Cruz. 2003. Calisti, A., K. Belay, G. Mazzoni, G. Fiocca, G. Retrosi, and
“A Modified Technique for the Diagnosis of Hirschsprung others. 2011. “Promoting Major Pediatric Surgical Care in
Disease from Rectal Biopsies.” National Medical Journal of a Low-Income Country: A 4-Year Experience in Eritrea.”
India 16 (5): 245–48. World Journal of Surgery 35 (4): 760–66.
Bakshi, K. D., B. Vaidyanathan, K. R. Sundaram, S. J. Roth, Calland, J. F., R. T. Petroze, J. Abelson, and E. Kraus. 2013.
K. Shivaprakasha, and others. 2007. “Determinants of Early “Engaging Academic Surgery in Global Health: Challenges
Outcome after Neontal Cardiac Surgery in a Developing and Opportunities in the Development of an Academic
Country.” Journal of Thoracic and Cardiovascular Surgery Track in Global Surgery.” Surgery 153 (3): 316–20.
134 (3): 765–71. Canfield, M. A., M. A. Honein, N. Yuskiv, J. Xing, C. T. Mai, and
Bandre, E., R. A. Kabore, I. Ouedraogo, O. Sore, T. Tapsoba, others. 2006. “National Estimates and Race/Ethnic-Specific
and others. 2010. “Hirschsprung’s Disease: Management Variation of Selected Birth Defects in the United States,
Problem in a Developing Country.” African Journal of 1999–2001.” Birth Defects Research Part A: Clinical and
Paediatric Surgery 7 (3): 166–68. Molecular Teratology 76 (11): 747–56.
Banu, T., M. Hoque, K. Laila, H. Ashraf Ul, and A. Hanif. 2009. Castilla, E. E., P. Mastroiacovo, and I. M. Orioli. 2008.
“Management of Male H-Type Anorectal Malformations.” “Gastroschisis: International Epidemiology and Public
Pediatric Surgery International 25 (10): 857–61. Health Perspectives.” American Journal of Medical Genetics
Benjamin, B. G., M. K. Ethen, C. L. Van Hook, C. A. Myers, and Part C: Seminars in Medical Genetics 148C (3): 162–79.
M. A. Canfield. 2010. “Gastroschisis Prevalence in Texas CDC (Centers for Disease Control). 2012. Birth defects.
1999–2003.” Birth Defects Research Part A: Clinical and Chalapathi, G., S. K. Chowdhary, K. L. Rao, R. Samujh,
Molecular Teratology 88 (3): 178–85. K. L. Narasimhan, and others. 2004. “Risk Factors in
Bernier, P. L., A. Stefanescu, G. Samoukovic, and the Primary Management of Anorectal Malformations in
C. Tchervenkov. 2010. “The Challenge of Congenital Heart Northern India.” Pediatric Surgery International 20 (6):
Disease Worldwide: Epidemiologic and Demographic 408–11.
Facts.” Pediatric Cardiac Surgery Annual of the Seminars in Chalya, P. L., J. B. Mabula, E. S. Kanumba, G. Giit, A. B.
Thoracic and Cardiovascular Surgery 13 (1): 26–34. Chandika, and others. 2011. “Experiences with Childhood
Bickler, S. W. 2000. “Non-Communicable Diseases: Is Their Colostomy at a Tertiary Hospital in Mwanza, Tanzania.”
Emergence in Industrialized Societies Related to Changes Tanzania Journal of Health Research 13 (3): 1–12.
in Neuroendocrine Function?” Medical Hypotheses 54 (5): Chandramouli, B., K. Srinivasan, J. Jagdish, and
825–28. N. Ananthakrishnan. 2004. “Morbidity and Mortality of
Bickler, S. W., and E. Ameh. 2011. Surgical Care for Children: A Colostomy and Its Closure in Children.” Journal of Pediatric
Guide for Primary Referral Hospitals. London: Macmillan. Surgery 39 (4): 596–99.
Bickler, S., D. Ozgediz, R. Gosselin, T. Weiser, D. Spiegel, and Chirdan, L. B., E. A. Ameh, F. A. Abantanga, D. Sidler, and
others. 2010. “Key Concepts for Estimating the Burden E. A. Elhalaby. 2010. “Challenges of Training and Delivery
of Surgical Conditions and the Unmet Need for Surgical of Pediatric Surgical Services in Africa.” Journal of Pediatric
Care.” World Journal of Surgery 34 (3): 374–80. Surgery 45 (3): 610–18.

Surgical Interventions for Congenital Anomalies 145

 Chirdan, L. B., P. J. Ngiloi, and E. A. Elhalaby. 2012. “Neonatal Eltayeb, A. A. 2010. “Delayed Presentation of Anorectal
Surgery in Africa.” Seminars in Pediatric Surgery 21 (2): Malformations: The Possible Associated Morbidity and
151–59. Mortality.” Pediatric Surgery International 26 (8): 801–06.
Chirdan, L. B., and A. F. Uba. 2006. “Hirschsprung’s Disease Farmer, P., and J. Kim. 2008. “Surgery and Global Health:
Presenting in the Neonatal Period in Jos, Nigeria.” Nigerian A View from beyond the OR.” World Journal of Surgery
Journal of Surgical Research 8 (1–2): 62–64. 32 (4): 533–36.
Chiu, P., and H. L. Hedrick. 2008. “Postnatal Management Frolov, P., J. Alali, and M. D. Klein. 2010. “Clinical Risk Factors for
and Long-Term Outcome for Survivors with Congenital Gastroschisis and Omphalocele in Humans: A Review of the
Diaphragmatic Hernia.” Prenatal Diagnosis 28 (7): Literature.” Pediatric Surgery International 26 (12): 1135–48.
592–603. Frykman, P. K., and S. S. Short. 2012. “Hirschsprung-Associated
Chowdhary, S. K., G. Chalapathi, K. L. Narasimhan, Enterocolitis: Prevention and Therapy.” Seminars in
R. Samujh, J. K. Mahajan, and others. 2004. “An Audit of Pediatric Surgery 21 (4): 328–35.
Neonatal Colostomy for High Anorectal Malformation: Gangopadhyay, A. N., S. Shilpa, T. V. Mohan, and S. C. Gopal.
The Developing World Perspective.” Pediatric Surgery 2005. “Single-Stage Management of All Pouch Colon
International 20 (2): 111–13. (Anorectal Malformation) in Newborns.” Journal of
Christianson, A. L., C. P. Howson, and B. Modell. 2006. Global Pediatric Surgery 40 (7): 1151–55.
Report on Birth Defects: The Hidden Toll of Dying and Goksan, S. B., A. Bursali, F. Bilgili, S. Sivacioglu, and S. Ayanoglu.
Disabled Children. White Plains, NY: March of Dimes Birth 2006. “Ponseti Technique for the Correction of Idiopathic
Defects Foundation. Clubfeet Presenting up to 1 Year of Age: A Preliminary
Coran, A. G., A. Caldamone, N. S. Adzick, T. M. Krummel, J.-M. Study in Children with Untreated or Complex Deformities.”
Laberge, and others. 2012. Pediatric Surgery: Expert Consult Archives of Orthopedic Trauma Surgery 126 (1): 15–21.
(2 volumes). 7th ed. London: Elsevier Health Sciences. Gosselin, R. A., A. Thind, and A. Bellardinelli. 2006. “Cost/
Coran, A. G., and D. H. Teitelbaum. 2000. “Recent Advances DALY Averted in a Small Hospital in Sierra Leone: What
in the Management of Hirschsprung’s Disease.” American Is the Relative Contribution of Different Services?” World
Journal of Surgery 180 (5): 382–87. Journal of Surgery 30 (4): 505–11.
Corlew, D. S. 2010. “Estimation of Impact of Surgical Disease Groen, R. S., M. Samai, R. T. Petroze, T. B. Kamara, L. D. Cassidy,
through Economic Modeling of Cleft Lip and Palate Care.” and others. 2013. “Household Survey in Sierra Leone Reveals
World Journal of Surgery 34 (3): 391–96. High Prevalence of Surgical Conditions in Children.” World
Davies, M. C., S. M. Creighton, and D. T. Wilcox. 2004. “Long- Journal of Surgery 37 (6): 1220–26.
Term Outcomes of Anorectal Malformations.” Pediatric Gupta, A., S. Singh, P. Patel, J. Patel, and M. K. Varshney.
Surgery International 20 (8): 567–72. 2008. “Evaluation of the Utility of the Ponseti Method of
Dobbs, M. B., and C. A. Gurnett. 2009. “Update on Clubfoot: Correction of Clubfoot Deformity in a Developing Nation.”
Etiology and Treatment.” Clinical Orthopaedics and Related International Orthopaedics 32 (1): 75–79.
Research 467 (5): 1146–53. Hadley, G. P. 2008. “Perspectives on Congenital Abnormalities
Dobbs, M. B., J. A. Morcuende, C. A. Gurnett, and I. V. Ponseti. in the Third World.” African Journal of Paediatric Surgery
2000. “Treatment of Idiopathic Clubfoot: An Historical 5 (1): 1–2.
Review.” Iowa Orthopedic Journal 20: 59–64. ———, and M. Mars. 2008. “Postgraduate Medical Education
Eason, G., M. Langer, D. Birabwa-Male, E. Reimer, in Paediatric Surgery: Videoconferencing—A Possible
M. Pennington, and others. 2012. “Costs and Cost- Solution for Africa?” Pediatric Surgery International 24 (2):
Effectiveness of Pediatric Inguinal Hernia Repair in 223–26.
Uganda.” Abstract presented at the European Paediatric Hegazy, M., N. M. Nasef, and H. Abdel-Ghani. 2009. “Results
Surgeons Association and British Association of Paediatric of Treatment of Idiopathic Clubfoot in Older Infants Using
Surgeons Joint Congress, Rome, June. the Ponseti Method: A Preliminary Report.” Journal of
Ekenze, S. O., S. N. Ibeziako, and U. O. Ezomike. 2007. Pediatric Orthopaedics. Part B 18 (2): 76–78.
“Trends in Neonatal Intestinal Obstruction in a Developing Higashi, H., J. J. Barendregt, and T. Vos. 2013. “The Burden
Country, 1996–2005.” World Journal of Surgery 31 (12): of Congenital Anomalies Amenable to Surgeries in Low-
2405–09. Income and Middle-Income Countries: A Modeled
Ekenze, S. O., C. Ngaikedi, and A. A. Obasi. 2011. “Problems Analysis.” The Lancet 381: S62.
and Outcome of Hirschsprung’s Disease Presenting after Hoffman, J. 2013. “The Global Burden of Congenital Heart
1 Year of Age in a Developing Country.” World Journal of Disease.” Cardiovascular Journal of Africa 24 (4): 141–45.
Surgery 35 (1): 22–26. Ibrahim, A. I. 2007. “One Stage Posterior Sagittal Anorectoplasty
Elhalaby, E.A. 2006. “Primary Repair of High and Intermediate for Treatment of High and Intermediate Anorectal Anomalies
Anorectal Malformations in the Neonates.” Annals of at Birth.” Annals of Pediatric Surgery 3 (3–4): 11–124.
Pediatric Surgery 2 (2): 117–22. Islam, M., and M. A. Aziz. 2011. “Esophageal Atresia: Outcome
Elhalaby, E. A., F. A. Uba, E. S. Borgstein, H. Rode, and in 21 Cases.” Bangladesh Armed Forces Medical Journal
A. J. Millar. 2012. “Training and Practice of Pediatric 44 (1): 47–50.
Surgery in Africa: Past, Present, and Future.” Seminars in Jamison, D. T., J. G. Breman, A. R. Measham, G. Alleyne,
Pediatric Surgery 21 (2): 103–10. M. Claeson, D. B. Evans, P. Jha. A. Mills, and P. Musgrove,

146 Essential Surgery

 eds. 2006. Disease Control Priorities in Developing Countries, Mendez-Tompson, M., O. Olivares-Becerril, M. Preciado-
2nd ed. Washington, DC: World Bank and Oxford Salgado, I. Quezada-Daniel, and J. G. Vega-Sanchez. 2012.
University Press. “Management of Congenital Adduct Clubfoot with the
Janicki, J. A., U. G. Narayanan, B. J. Harvey, A. Roy, S. Weir, and Ponseti Technique. Experience at La Perla General Hospital.”
others. 2009. “Comparison of Surgeon and Physiotherapist- Acta Ortopedica Mexicana 26 (4): 228–30.
Directed Ponseti Treatment of Idiopathic Clubfoot.” Journal Mhando, S., B. Young, and K. Lakhoo. 2008. “The Scope
of Bone and Joint Surgery 91 (5): 1101–08. of Emergency Paediatric Surgery in Tanzania.” Pediatric
Jawadi, A. H. 2010. “Clubfoot Management by the Ponseti Surgery International 24 (2): 219–22.
Technique in Saudi Patients.” Saudi Medical Journal 31 (1): Mills, J. L., T. C. Carter, D. M. Kay, M. L. Browne, L. C. Brody,
49–52. and others. 2012. “Folate and Vitamin B12–Related Genes
Khemakhem, R., B. Haggui, H. Rahay, F. Nouira, A. Charieg, and Risk for Oomphalocele.” Human Genetics 131 (5):
and others. 2012. “Congenital Diaphragmatic Hernia in 739–46.
Neonate: A Retrospective Study about 28 Observations.” Miranda, M. E., E. D. Tatsuo, J. T. Guimaraes, R. M. Paixao, and
African Journal of Paediatric Surgery 9 (3): 217–22. J. C. B. D. Lanna. 1999. “Use of a Plastic Hemoderivative
Larrazabal, L. A., K. J. Jenkins, K. Gauvreau, V. L. Vida, Bag in the Treatment of Gastroschisis.” Pediatric Surgery
O. J. Benavidez, and others. 2007. “Improvement in International 15 (5–6): 442–44.
Congenital Heart Surgery in a Developing Country: The Mkandawire, M., and E. Kaunda. 2002. “An Audit of Congenital
Guatemalan Experience.” Circulation 116 (17): 1882–87. Anomalies in the Neonatal Unit of Queen Elizabeth Central
Laughon, M., R. Meyer, C. Bose, A. Wall, E. Otero, and others. Hospital. One-Year Study Period: 1st November 2000
2003. “Rising Birth Prevalence of Gastroschisis.” Journal to 31st October 2001.” East and Central African Journal of
of Perinatology: Official Journal of the California Perinatal Surgery 7 (1): 29–33.
Association 23 (4): 291–93. Mo Suwan, L., S. Isaranurug, P. Chanvitan, W. Techasena,
Lavy, C. B. D., S. J. Mannion, N. C. Mkandawire, A. Tindall, S. Sutra, and others. 2009. “Perinatal Death Pattern in the
C. Steinlechner, and others. 2007. “Club Foot Treatment Four Districts of Thailand: Findings from the Prospective
in Malawi: A Public Health Approach.” Disability and Cohort Study of Thai Children.” Journal of the Medical
Rehabilitation 29 (11–12): 857–62. Association of Thailand 92 (5): 660–66.
Loane, M., H. Dolk, and I. Bradbury. 2007. “Increasing Moore, S. W., A. Alexander, D. Sidler, J. Alves, G. P. Hadley, and
Prevalence of Gastroschisis in Europe 1980–2002: A others. 2008. “The Spectrum of Anorectal Malformations
Phenomenon Restricted to Younger Mothers?” Paediatric in Africa.” Pediatric Surgery International 24 (6): 677–83.
and Perinatal Epidemiology 21 (4): 363–69. Nacul, L. C., A. Stewart, C. Alberg, S. Chowdhury,
Loisance, D. Y. 2012. “Training Young Cardiac Surgeons in M. W. Darlison, and others. 2013. “A Toolkit to Assess
Developing Countries.” Asian Cardiovascular and Thoracic Health Needs for Congenital Disorders in Low- and
Annals 20 (4): 384–86. Middle-Income Countries: An Instrument for Public
Lourenço, A., and J. Morcuende. 2007. “Correction of Neglected Health Action.” Journal of Public Health. Advance online
Idiopathic Club Foot by the Ponseti Method.” Journal of publication. doi:10.1093/pubmed/fdt048.
Bone and Joint Surgery. British Volume 89 (3): 378–81. Nandi, B., C. Mungongo, and K. Lakhoo. 2008. “A Comparison
Magee, W., H. M. Raimondi, M. Beers, and M. C. Koech. 2012. of Neonatal Surgical Admissions between Two Linked
“Effectiveness of International Surgical Program Model Surgical Departments in Africa and Europe.” Pediatric
to Build Local Sustainability.” Plastic Surgery International Surgery International 24 (8): 939–42.
2012: 185725. doi:10.1155/2012/185725. Narasimman, S., M. Nallusamy, and S. Hassan. 2013. “Review
Makhdoom, A., M. A. Laghari, M. K. Pahore, P. A. L. Qureshi, of Oesophageal Atresia and Tracheoesophageal Fistula
I. A. Bhutto, and others. 2011. “Clubfoot Treatment by in Hospital Sultanah Bahiyah, Alor Star. Malaysia from
Ponseti Method.” Journal of the Liaquat University of January 2000 to December 2009.” Medical Journal of
Medical and Health Sciences 10 (2): 71–74. Malaysia 68 (1): 48–51.
Manson, J., E. Ameh, N. Canvassar, T. Chen, A. V. den Hoeve, Olivieri, C., K. Belay, R. Coletta, G. Retrosi, P. Molle, and A. Calisti.
and others. 2012. “Gastroschisis: A Multi-centre Comparison 2012. “Preventing Posterior Sagittal Anoplasty ‘Cripples’ in
of Management and Outcome.” African Journal of Paediatric Areas with Limited Medical Resources: A Few Modifications
Surgery 9 (1): 17–21. to Surgical Approach in Anorectal Malformations.” African
Martiniuk, A. L. C., M. Manouchehiran, J. A. Negin, and Journal of Paediatric Surgery 9 (3): 223–26.
A. B. Zwi. 2012. “Brain Gains: A Literature Review of Osifo, O. D., and C. J. Okolo. 2009. “Outcome of Trans-anal
Medical Missions to Low and Middle-Income Countries.” Posterior Anorectal Myectomy for the Ultrashort Segment
BMC Health Services Research 12: 134. Hirschprung’s Disease—Benin City Experience in 5 Years.”
Mayo, E., A. Cuthel, J. Macharia, C. Lavy, and T. Mead. Nigerian Postgraduate Medical Journal 16 (3): 213–17.
n.d. “Creating a Countrywide Program Model for Owen, R. M., J. N. Penny, A. Mayo, J. Morcuende, and
Implementation of a Ponseti Method Clubfoot Treatment C. B. Lavy. 2012. “A Collaborative Public Health Approach
Program in Developing Countries.” Unpublished. http:// to Clubfoot Intervention in 10 Low-Income and Middle-
cure.org/downloads/site/clubfoot/ccw-creating_a​ Income Countries: 2-Year Outcomes and Lessons Learnt.”
_­countrywide_program_model.pdf. Journal of Pediatric Orthopaedics. Part B 21 (4): 361–65.

Surgical Interventions for Congenital Anomalies 147

 Ozdogan, T., C. Durakbasa, M. Mutus, and M. Iscen. 2010. Initiative as a Model for Sustainable Collaboration.” World
“Congenital Diaphragmatic Hernia: A 4-Year Experience in Journal of Surgery 35 (1): 17–21.
a Single Centre.” African Journal of Paediatric Surgery 7 (2): Rao, S. G. 2007. “Pediatric Cardiac Surgery in Developing
105–06. Countries.” Pediatric Cardiology 28 (2): 144–48.
Ozgediz, D., D. T. Jamison, M. Cherian, and K. McQueen. 2008. Rattray, K. W., T. C. Harrop, J. Aird, M. Beveridge, J. G. Gollogly,
“The Burden of Surgical Conditions and Access to Surgical and others. 2013. “The Cost Effectiveness of Reconstructive
Care in Low- and Middle-Income Countries.” Bulletin of the Surgery in Cambodia.” Asian Biomedicine 7 (3): 319–24.
World Health Organization 86 (8): 646–47. Rayes, O. M. 2010. “Congenital Tracheoesophageal Fistula
Ozgediz, D., and D. Poenaru. 2012. “The Burden of Pediatric with or without Esophageal Atresia, King Abdulaziz
Surgical Conditions in Low and Middle Income Countries: A University Hospital Experience over 15 Years.” Journal of
Call to Action.” Journal of Pediatric Surgery 47 (12): 2305–11. King Abdulaziz University-Medical Sciences 17 (4): 59–72.
Panjavi, B., A. Sharafatvaziri, R. H. Zargarbashi, and S. Mehrpour. Rescorla, F. J., A. M. Morrison, D. Engles, J. W. West, and
2012. “Use of the Ponseti Method in the Iranian Population.” J. L. Grosfeld. 1992. “Hirschsprung’s Disease: Evaluation of
Journal of Pediatric Orthopaedics 32 (3): e11–14. Mortality and Long-Term Function in 260 Cases.” Archives
Patel, A., J. E. Clune, D. M. Steinbacher, and J. A. Persing. 2013. of Surgery 127 (8): 934.
“Comprehensive Cleft Center: A Paradigm Shift in Cleft Richmond, S., and J. Atkins. 2005. “A Population-Based Study
Care.” Plastic and Reconstructive Surgery 131 (2): 312e–13e. of the Prenatal Diagnosis of Congenital Malformation over
Patwardhan, N., E. Kiely, D. P. Drake, L. Spitz, and A. Pierro. 16 Years.” BJOG: An International Journal of Obstetrics and
2001. “Colostomy for Anorectal Anomalies: High Incidence Gynaecology 112 (10): 1349–57.
of Complications.” Journal of Pediatric Surgery 36 (5): 795–98. Rohana, J., N. Y. Boo, and C. R. Thambidorai. 2008. “Early
Penchaszadeh, V. B. 2002. “Preventing Congenital Anomalies in Outcome of Congenital Diaphragmatic Hernia in a
Developing Countries.” Community Genetics 5 (1): 61–69. Malaysian Tertiary Centre.” Singapore Medical Journal
Peyvasteh, M., S. Askarpour, H. Javaherizadeh, and 49 (2): 142–44.
T. Fatahian. 2011. “Evaluation of Epidemiologic Indices Sadler, T. W. 2010. “The Embryologic Origin of Ventral Body
of Neonate’s Diseases in the Pediatric Surgery Ward of the Wall Defects.” Seminars in Pediatric Surgery 19 (3): 209–14.
Ahvaz Jundishapur University Hospitals during the Period Sadoh, W. E., D. U. Nwaneri, and A. C. Owobu. 2011. “The Cost
1993–1996 and 2002–2005.” Annals of Pediatric Surgery of Out-Patient Management of Chronic Heart Failure in
7 (1): 7–9. Children with Congenital Heart Disease.” Nigerian Journal
Pirani, S., E. Naddumba, R. Mathias, J. Konde-Lule, J. N. Penny, of Clinical Practice 14 (1): 65–69.
and others. 2009. “Towards Effective Ponseti Clubfoot Care: Saha, A. K., M. B. Ali, S. K. Biswas, H. M. Z. Sharif, and
The Uganda Sustainable Clubfoot Care Project.” Clinical A. Azim. 2012. “Neonatal Intestinal Obstruction: Patterns,
Orthopaedics and Related Research 467 (5): 1154–63. Problems and Outcomes.” Bangladesh Medical Journal
Poenaru, D. 2013. “Getting the Job Done: Analysis of the (Kulna) 45 (1-2): 6–10.
Impact and Effectiveness of the Smile Train Program in Salomon, J. A., T. Vos, D. R. Hogan, M. Gagnon, M. Naghavi,
Alleviating the Global Burden of Cleft Disease.” World and others. 2012. “Common Values in Assessing Health
Journal of Surgery 37 (7): 1562–70. Outcomes from Disease and Injury: Disability Weights
Poenaru, D., E. Borgstein, A. Numanoglu, and G. Azzie. Measurement Study for the Global Burden of Disease Study
2010. “Caring for Children with Colorectal Disease in 2010.” The Lancet 380 (9859): 2129–43.
the Context of Limited Resources.” Seminars in Pediatric Saranrittichai, S. 2008. “Gastroschisis: Delivery and Immediate
Surgery 19 (2): 118–27. Repair in the Operating Room.” Journal of the Medical
Poenaru, D., J. Pemberton, C. Frankfurter, and B. Cameron. 2013. Association of Thailand 91 (5): 686–92.
“Establishing Disability Weights for Congenital Pediatric Sarrafan, N., S. A. M. Nasab, M. Fakoor, and A. Zakeri. 2012.
Surgical Disease: A Multi-modal Study.” The Lancet 381: “Short Term Outcome of Congenital Clubfoot Treated
S3115. by Ponseti Method.” Pakistan Journal of Medical Sciences
Poley, M. J., W. F. B. Brouwer, J. J. V. Busschbach, 28 (3): 459–62.
F. W. J. Hazebroek, D. Tibboel, and others. 2008. “Cost- Sekabira, J., and G. P. Hadley. 2009. “Gastroschisis: A Third
Effectiveness of Neonatal Surgery: First Greeted with World Perspective.” Pediatric Surgery International 25 (4):
Skepticism, Now Increasingly Accepted.” Pediatric Surgery 327–29.
International 24 (2): 119–27. Sekar, P., and V. Vilvanathan. 2007. “Telecardiology: Effective
Pratap, A., D. K. Gupta, A. Tiwari, A. Sinha, N. Bhatta, and Means of Delivering Cardiac Care to Rural Children.” Asian
­others. 2007. “Application of a Plain Abdominal Radiograph Cardiovascular and Thoracic Annals 15 (4): 320–23.
Transition Zone (PARTZ) in Hirschsprung’s Disease.” BMC Semer, N. 2001. Practical Plastic Surgery for Nonsurgeons.
Pediatrics 7: 5. Philadelphia, PA: Hanley and Belfus.
Prato, A. P., V. Rossi, S. Avanzini, G. Mattioli, N. Disma, Senga, J., E. Rusingiza, J. Mucumbitsi, A. Binagwaho, B. Suys,
and others. 2011. “Hirschsprung’s Disease: What about and others. 2013. “Catheter Interventions in Congenital
Mortality?” Pediatric Surgery International 27 (5): 473–78. Heart Disease without Regular Catheterization Laboratory
Qureshi, J. S., J. Samuel, C. Lee, B. Cairns, C. Shores, and ­others. Equipment: The Chain of Hope Experience in Rwanda.”
2011. “Surgery and Global Public Health: The UNC-Malawi Pediatric Cardiology 34 (1): 39–45.

148 Essential Surgery

Shah, G., M. Singh, T. Pandey, B. Kalakheti, and G. Bhandari. van Bosse, H. J. 2011. “Ponseti Treatment for Clubfeet, An
2008. “Incidence of Congenital Heart Disease in a Tertiary International Perspective.” Current Opinion in Pediatrics
Care Hospital.” Kathmandu University Medical Journal 23 (1): 41–45.
6 (1): 33–36. van der Linde, D., E. E. M. Konings, M. A. Slager, M. Witsenburg,
Sharma, S., and D. K. Gupta. 2012. “Hirschsprung’s Disease W.A. Helbing, and others. 2011. “Birth Prevalanece of
Presenting beyond Infancy: Surgical Options and Postoperative Congenital Heart Disease Worldwide.” Journal of the
Outcome.” Pediatric Surgery International 28 (1): 5–8. American College of Cardiology 58 (21): 2241–47.
Singh, K., C. Greaves, L. Mohammed, and A. Kumar. 2012. van Heurn, L. W. E., W. Cheng, B. de Vries, H. Saing,
“Epidemiology of Treacheoesophageal Fistula and Other N. J. G. Jansen, and others. 2002. “Anomolies Associated
Major Congenital Malformations of the Digestive System with Oesophageal Atresia in Asians and Europeans.”
among Newborns in an English Speaking Caribbean Pediatric Surgery International 18 (4): 241–43.
Country: A Population Based Study.” British Journal of Vincent, M. V., and S. U. Jackman. 2009. “Hirschsprung’s
Medical Sciences 1 (3): 16–25. Disease in Barbados—A 16-Year Review.” West Indian
Sinha, S. K., R. P. Kanojia, A. Wakhlu, J. D. Rawat, S. N. Kureel, Medical Journal 58 (4): 347–51.
and others. 2008. “Delayed Presentation of Anorectal Vu, L. T., K. K. Nobuhara, C. Laurent, and G. M. Shaw. 2008.
Malformations.” Journal of the Indian Association of “Increasing Prevalence of Gastroschisis: Population-
Pediatric Surgery 13 (2): 64–68. Based Study in California.” Journal of Pediatrics 152 (6):
Spiegel, D. A., O. P. Shrestha, P. Sitoula, T. Rajbhandary, 807–11.
B. Bijukachhe, and others. 2009. “Ponseti Method for Walker, I. A., A. D. Obua, F. Mouton, S. Ttendo, and
Untreated Idiopathic Clubfeet in Nepalese Patients from I. H. Wilson. 2010. “Paediatric Surgery and Anaesthesia in
1 to 6 Years of Age.” Clinical Orthopaedics and Related South-Western Uganda: A Cross-Sectional Survey.” Bulletin
Research 467 (5): 1164–70. of the World Health Organization 88 (12): 897–906.
Stoll, C., Y. Alembik, B. Dott, and M. P. Roth. 2001. “Risk Factors Warf, B. C. 2011. “Hydrocephalus Associated with Neural
in Congenital Abdominal Wall Defects (Omphalocele and Tube Defects: Characteristics, Management, and Outcomes
Gastroschisi): A Study in a Series of 265,858 Consecutive in ­Sub-Saharan Africa.” Child’s Nervous System 27 (10):
Births.” Annales De Genetique 44 (4): 201–08. 1589–94.
Swenson, O. 2002. “Hirschsprung’s Disease: A Review.” ———. 2013. “Educate One to Save A Few. Educate Few to
Pediatrics 109 (5): 914–18. Save Many.” World Neurosurgery 79 (2 Suppl.): e15–8.
Tefera, E., T. Teka, and M. Derbew. 2007. “Neonatal Warf, B. C., B. C. Alkire, S. Bhai, C. Hughes, S. J. Schiff,
Gastrointestinal Surgical Emergencies: A 5-Year Review in and ­ others. 2011. “Costs and Benefits of Neurosurgical
a Teaching Hospital in Addis Ababa, Ethiopia.” Ethiopian Intervention for Infant Hydrocephalus in Sub-Saharan
Medical Journal 45 (3): 251–56. Africa.” Journal of Neurosurgical Pediatrics 8 (5): 509–21.
Tindall, A. J., C. W. E. Steinlechner, C. B. D. Lavy, S. Mannion, WHO (World Health Organization). 2008. “Global Burden of
and N. Mkandawire. 2005. “Results of Manipulation of Disease 2004 Update: Disability Weights for Diseases and
Idiopathic Clubfoot Deformity in Malawi by Orthopaedic Conditions.” WHO, Geneva.
Clinical Officers Using the Ponseti Method: A Realistic ———. 2012. “Congenital Anomalies,” Fact Sheet No. 370,
Alternative for the Developing World?” Journal of Pediatric WHO, Geneva. http://www.who.int/mediacentre/factsheets​
Orthopaedics 25 (5): 627–29. /fs370/en/.
Toro, M. N. H., M. E. A. Rave, and P. M. J. Gomez. 2010. ———. 2013a. “Emergency and Essential Surgical Care:
“Management of Abdominal Wall Defects (Gastroschisis Congenital Anomalies.” http://www.who.int/surgery​
and Omphalocele) at Hospital Universitario San Vicente de /­challenges/esc_congenital_nomalies/en/.
Paúl, in Medellín, Colombia, 1998–2006.” [Spanish]. Iatreia ———. 2013b. “Global Health Estimates for Deaths by Cause,
23 (3): 220–26. Age, and Sex for Years 2000–2011.” WHO, Geneva.
Turowski, C., J. Dingemann, and J. Giilick. 2010. “Delayed Wickramasinghe, P., S. P. Lambabadusuriya, and S. Narenthiran.
Diagnosis of Imperforate Anus: An Unacceptable 2001. “Prospective Study of Congenital Heart Disease in
Morbidity.” Pediatric Surgery International 26 (11): 1083–86. Children.” Ceylon Medical Journal 46 (3): 96–98.
Uba, A. F., and L. B. Chirdan. 2003. “Omphalocoele and Wu, V., and D. Poenaru. 2013. “Burden of Surgically Correctable
Gastroschisis: Management in a Developing Country.” Disabilities among Children in the Dadaab Refugee Camp.”
Nigerian Journal of Surgical Research 5 (1): 57–61. World Journal of Surgery 37 (7): 1536–43.
Ugiagbe, E. E., and O. D. Osifo. 2012. “Postmortem Wu, V., D. Poenaru, and M. J. Poley. 2013. “Burden of Surgical
Examinations on Deceased Neonates: A Rarely Utilized Congenital Anomalies in Kenya: A Population-Based
Procedure in an African Referral Center.” Pediatric and Study.” Journal of Tropical Pediatrics 59 (3): 195–220.
Developmental Pathology 15 (1): 1–4. Yang, C. F., W. J. Soong, M. J. Jeng, S. J. Chen, Y. S. Lee, and
Upadhyaya, V. D., A. N. Gangopadhyaya, D. K. Gupta, others. 2006. “Esophageal Atresia with Tracheoesophageal
S. P. Sharma, V. Kumar, and others. 2007. “Prognosis of Fistula: Ten Years of Experience in an Institute.” Journal of
Congenital Tracheoesophageal Fistula with Esophageal the Chinese Medical Association 69 (7): 317–21.
Atresia on the Basis of Gap Length.” Pediatric Surgery Zupan, J. 2005. “Perinantal Mortality in Developing Countries.”
International 23 (8): 767–71. New England Journal of Medicine 352 (20): 2047–48.

Surgical Interventions for Congenital Anomalies 149