Pediatric Diagnosis: Atlas of Disorders of Surgical Significance Spencer W. Beasley digital version 2025
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    Pediatric Diagnosis: Atlas of Disorders of Surgical
             Significance Spencer W. Beasley
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                     Paediatric Surgical
                        Diagnosis
                     Atlas of Disorders of Surgical Significance
SECOND EDITION
                                                       Spencer W. Beasley
                                                  Paediatric Surgeon, Christchurch Hospital
                        Professor of Paediatric Surgery, Departments of Paediatrics and Surgery, University of Otago
                                                         Christchurch, New Zealand
                                                            John Hutson
                                            Paediatric Urologist, The Royal Children’s Hospital
                              Chair of Paediatric Surgery, Department of Paediatrics, University of Melbourne
                                                            Melbourne, Australia
                                                           Mark Stringer
                                                 Paediatric Surgeon, Wellington Hospital
                             Honorary Professor, Department of Paediatrics & Child Health, University of Otago
                                                        Wellington, New Zealand
                                                        Sebastian K. King
                                             Paediatric Surgeon, The Royal Children’s Hospital
                              Clinical Associate Professor, Department of Paediatrics, University of Melbourne
                                                             Melbourne, Australia
                                                       Warwick J. Teague
                             Paediatric Surgeon & Director of Trauma Services, The Royal Children’s Hospital
                             Clinical Associate Professor, Department of Paediatrics, University of Melbourne
                                                            Melbourne, Australia
           This book contains information obtained from authentic and highly regarded sources. Reasonable efforts have been made to publish reliable data and
           information, but the author and publisher cannot assume responsibility for the validity of all materials or the consequences of their use. The authors
           and publishers have attempted to trace the copyright holders of all material reproduced in this publication and apologize to copyright holders if
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                                        Names: Beasley, Spencer W., author. | Hutson, John M., author. | Stringer,
                                        Mark D., author. | King, Sebastian, author. | Teague, Warwick J., author.
                                        Title: Pediatric diagnosis : atlas of disorders of surgical significance /
                                        Spencer Beasley, John Hutson, Mark D. Stringer, Sebastian King, Warwick
                                        Teague.
                                        Description: Second edition. | Boca Raton : CRC Press, [2018] | Includes
                                        bibliographical references and index. |
                                        Identifiers: LCCN 2017052034 (print) | LCCN 2017052272 (ebook) | ISBN
                                        9781315279978 (eBook General) | ISBN 9781315279961 (eBook PDF) | ISBN
                                        9781315279954 (eBook ePub3) | ISBN 9781315279947 (eBook Mobipocket) |
                                        ISBN 9781138197329 (pbk. : alk. paper)
                                        Subjects: | MESH: Diagnostic Techniques, Surgical | Child | Infant | Atlases
                                        Classification: LCC RD137.3 (ebook) | LCC RD137.3 (print) | NLM WO 517 | DDC
                                        617.9/8--dc23
                                        LC record available at https://lccn.loc.gov/2017052034
              Paediatric surgery is age-related general surgery in a broad         photographs, x-rays and other imaging studies. In addition to
              sense, involving many specialty fields. This book represents         marvellous illustrations, good descriptive legends and a con-
              the essential elements of the multifaceted nature of the sur-        cise text present a list of differential diagnoses and at least one
              gery of infancy and childhood.                                       reliable approach to confirmatory studies where appropriate.
                  Three very experienced individuals with well-recognised             No attempt is made to be exhaustive in the treatment of
              expertise in clinical care, education and research wrote this        any disorder, which makes this book particularly useful for
              book. They have worked their entire careers in one of the most       the intended audience around the world. The authors advise
              important children’s centres in the world, so this book brings       their readers to use this book in conjunction with a stan-
              with it a level of authority which is unmatched.                     dard text for comprehensive coverage, but it would certainly
                  The authors’ goal was to develop an illustrated guide to         stand alone as a visual guide to rapid diagnosis of almost
              diagnosis of the important paediatric surgical conditions.           any paediatric surgical condition likely to be seen in routine
              The common, the occasional and some rare conditions are              practice. This book is well organised into sections, which are
              all here. Although it would be virtually impossible to include       comprehensively treated. In addition to conditions usually
              every conceivable rare disorder, the authors come close; and         treated by general paediatric surgeons, those treated by oph-
              certainly every condition likely to be encountered in a prac-        thalmologists, neurosurgeons, urologists, otolaryngologists,
              titioner’s professional life time is presented including entities    orthopaedists and plastic surgeons are covered. Neonatal as
              seen mainly in third world countries. There is a great need for      well as acquired disorders are included. A book like this could
              a book of this nature, which teaches the basics that trainees are    only have come from the life’s work of three such experienced
              frequently not taught today as high-tech medicine is empha-          paediatric surgeons.
              sised. The emphasis is on primary care and additional manage-
              ment geared for medical students, trainees and practitioners in                                         James A. O’Neill, Jr, MD
              paediatrics, obstetrics and various surgical specialities, as well                  C. Everett Koop Professor of Paediatric Surgery
              as nurses who might need a quick reference for diagnosis.                           University of Pennsylvania, School of Medicine
                  The particularly valuable aspect of this pictorial text is                Surgeon-in-Chief, Children’s Hospital of Philadelphia
              that it stresses physical diagnosis illustrated by beautiful                                                Philadelphia, PA, USA
vii
              This second edition of Atlas of Paediatric Surgery, now titled    assessment and intervention were possible previously, these
              Paediatric Surgical Diagnosis: Atlas of Disorders of Surgical     have become a standard aspect of care in the time interval
              Significance, is a superbly illustrated and well-organized        since the first edition was published. These areas are addressed
              guide to most common anomalies in children’s surgery as           in chapter one, while the second chapter is dedicated to major
              well as many that are uncommon. While this text is not            congenital anomalies which would be symptomatic or appar-
              intended to be encyclopaedic in scope, it does present in con-    ent in the neonatal period. Together these chapters will be
              siderable depth the broad spectrum of general and thoracic        quite useful to any who wish to understand newborn surgi-
              paediatric surgery, as well as offer an important perspec-        cal anomalies. The introductory chapters are followed by
              tive to any children’s provider in paediatric urology, head       anatomical and organ system presentations, concluding with
              and neck surgery, ophthalmology, orthopaedics, neurosur-          trauma and gynaecological conditions. The overall focus is
              gery and, indeed, all of children’s surgery. The emphasis is      on visual clarity and a pragmatic bedside clinical approach to
              on providing photographs and illustrations to assist in the       these children with surgical needs. The atlas is designed not
              recognition, clinical understanding and multidisciplinary         as a comprehensive surgical reference text or operating atlas,
              management of the various abnormalities. This edition             but as a companion or adjunct to such standard presentations,
              builds on the first; it is not only beautifully photographed      adding a depth of understanding and visual diagnostic clarity
              and illustrated, but it is improved in the organization and       that is unique. It will be a valuable addition to the libraries of
              clarity of the supporting text. Taken together, the succinct      institutions and individuals who care for children with sur-
              narratives and lucid visual presentations make the text use-      gical needs. This includes not just general and thoracic pae-
              ful to a broad audience. Children’s surgery encompasses           diatric surgeons but all children’s surgeons, as well as other
              such diverse pathology that collection of this information,       allied health professionals, those who are learning about these
              and particularly these photographs, would take most indi-         patients, including residents, fellows and students, and indeed
              viduals several careers to compile. The authors have done         anyone with an interest in the surgical problems of infants
              this for the reader with emphasis on diagnostic information       and children.
              including radiographic and other imaging, as well as intra-
              operative photographs. The presentation is uniquely infor-                                                Keith T. Oldham, MD
              mative in aggregate.                                                           Professor of Surgery, Division of Pediatric Surgery
                 The atlas is edited by five of the most senior and well rec-                                     Medical College of Wisconsin
              ognized children’s surgeons in the world. All are experts and                        Marie Z. Uihlein Chair of Pediatric Surgery
              bring a wealth of clinical experience to this work. The authors                                             and Surgeon-in-Chief
              have added new information in this edition, particularly                                        Children’s Hospital of Wisconsin
              related to antenatal diagnosis and treatment. While in-utero                                                Milwaukee, WI, USA
ix
              ACKNOWLEDGEMENTS FOR THE                                            copying (and maintaining in order) a large number of slides,
              FIRST EDITION                                                       and to produce a number of line drawings. Their full coopera-
                                                                                  tion in this project over many months has made the editors’
              No one surgeon, not even one in the busiest clinical practice,      task much easier.
              could collect the range and variety of conditions displayed             The secretarial staff of the Department of Surgery, Elizabeth
              in this atlas within a lifetime. Therefore, despite fairly exten-   Vorrath and Judith Hayes, now well used to the rigours of medi-
              sive collections of our own, we have been heavily reliant on        cal manuscripts, have confirmed their mastery of complex,
              the contributions of a number of colleagues. Many of these          numerous and ever-changing legends. Their efficiency and good
              have gone to considerable effort to provide us with as com-         humour has obscured any frustrations they might have had.
              prehensive a coverage of the specialty as possible, and to them         Our ever-patient families have provided the support that
              we are extremely grateful. They include: Alex Auldist, John         has allowed us to devote consecutive long weekends to the
              Barnett, Don Cameron, Tony Catto-Smith, Bill Cole, David            project. We are happy to return the time to them in full now
              Croaker, Paddy Dewan, Bob Dickens, James Elder, Roger Hall,         it is completed.
              David James, Peter Jones, Anne Kosloske, Julian Keogh, Geoff            Finally, we are grateful to Peter Altman who encouraged us
              Klug, Neil McMullin, Azad Najmaldin, Kevin Pringle, T.M.            to embark on the project and who has provided helpful guid-
              Ramanaujam, Barry Shandling, Errol Simpson, Arnold Smith,           ance throughout. The professional work of Chapman and Hall
              Durham Smith, John Solomon, Douglas Stephens, Keith                 has been a major factor contributing to this work, which we
              Stokes, Russell Taylor, Roger Voigt, Alan and Susie Woodward.       hope will be of use to paediatric surgical aspirants and teach-
              Ramanujam, in particular went to extraordinary lengths to           ers for many years to come.
              provide us with a fine series of slides of the most bizarre and
              rare conditions. We are grateful to our many registrars who         ACKNOWLEDGEMENTS FOR THE
              have had to organise the photography of many of the lesions.
                 Slides of interesting cases often are passed between col-
                                                                                  SECOND EDITION
              leagues and, with time, their origin may become obscure. It is      Once again we are indebted to The Royal Children’s Hospital
              quite possible that a number of illustrations included in this      Creative Studio (previously Educational Resource Centre) for
              atlas have not been acknowledged appropriately. To those who        their expert assistance in taking many of the clinical pho-
              have the original source of these illustrations, we are truly       tographs as well as dealing with the artwork and digital file
              grateful, despite their anonymity.                                  management.
                 An enormous contribution has been made by staff of the              We thank Shirley D’Cruz, Personal Assistant to Professor
              Educational Resource Centre of The Royal Children’ Hospital,        John Hutson, who worked tirelessly to prepare the base texts
              Melbourne, who have been happy to bear the burden of                and image legends for revision. Shirley then shouldered the
                           Fig. 1 The authors (from left to right): Spencer Beasley, Mark Stringer, Warwick Teague, Sebastian King
                           and John Hutson.                                                                                                       xi
           lion’s share of the responsibility for ensuring version control       to Peter Beynon and Paul Bennett, the meticulous care they
           throughout the complex task of revising the text and legends,         have taken assembling a substantial file of figures, and their
           and managing the nuances and needs of five editors and a              detailed understanding of what we have tried to achieve, has
           publisher across three time zones.                                    made our work so much easier.
              Many of the original photographs provided in the first e dition      Creating an atlas of this magnitude takes an enormous
           have been retained. In addition, we have included photographs         amount of time: this time is often at the end of an already
           and images provided by the listed contributors to this edition.       long day, and it is time that otherwise would have been spent
              The staff of CRC Press throughout have been wonderfully            with our wives and families. So it is to Christy, Susan, Alice,
           patient and understanding of us, particularly in the final            Charlotte and Kirsty, and our respective children, who have
           stages where the actual layout and accuracy of the figures            had to tolerate and forgive us our absences during the prepa-
           and their legends has become important. From Cherry Allen,            ration of this book, that we are so grateful.
xiii
           Victoria Scott
           Paediatric Surgeon                      Jonathan Wells
           Christchurch Hospital                   Paediatric Surgeon
           Christchurch, New Zealand               Christchurch Hospital
                                                   Christchurch, New Zealand
           Anne Smith
           Forensic Paediatrician                  Toni-Maree Wilson
           Victorian Forensic Paediatric Medical   Paediatric Surgeon
              Service                              Wellington Hospital
           Melbourne, Australia                    Wellington, New Zealand
              More than any other specialty, with the possible exception of        to 9 deal with the regions of the body sequentially, working
              dermatology, paediatric surgery lends itself to an illustrated       from the head and neck, through the trunk, to the limbs.
              guide to diagnosis. In the neonate, the dramatic appearance          Abnormalities of the respiratory, gastrointestinal and urinary
              of exomphalos, gastroschisis, bladder exstrophy and prune            systems may be associated with a variety of external clinical
              belly syndrome is obvious. Anorectal malformations pres-             manifestations but, more often than not, the definitive diag-
              ent with a spectrum of features, some of which are quite             nosis is made only after specialised radiological investigation
              subtle, but which can be demonstrated with careful clinical          or at operation. Consequently, the operative views illustrate
              examination. Even some internal lesions, such as volvulus,           those conditions in which a diagnosis is made at surgery, or
              meconium ileus and bowel atresia, have external features,            where the operative appearance is characteristic and clarifies
              such as abdominal distension. The majority of orthopaedic            the diagnosis. No attempt has been made to include details of
              deformities and inguinoscrotal lesions are diagnosed entirely       operative technique. In the chapter on trauma, which covers
               on clinical grounds, most of which can be illustrated clearly       all systems, special emphasis is given to non-accidental injury
               on photography. Likewise, abnormalities and lesions of the          and in particular sexual abuse, as this is an area of consider-
               head and neck, which are common in this age group, are usu-         able importance, and the clinician must be aware of the rel-
               ally superficial or structural, enabling easy clinical diagnosis.   evant features.
               The relatively obscure areas of the thorax and urinary tract             There are three major limitations to any pictorial guide to
               may have few or vague clinical features, but become apparent        diagnosis. First, given the limitations of length, it is impos-
               on appropriate radiological or other types of imaging.              sible to include all conditions or their variations. For example,
                  This illustrated guide to the diagnosis of paediatric surgical   the sections on anorectal malformations or disorders of sex-
               disorders sets out to cover the broad spectrum of abnormali-        ual differentiation are extensive but not comprehensive: they
               ties encountered in this specialty. Although we have concen-        could be vastly expanded, but for the sake of a relatively con-
               trated on the common lesions, we have deliberately included         cise book this is not feasible. Second, many well-recognised
               some extremely rare conditions to highlight the enormous            conditions occur extremely rarely and, even in a large institu-
               variation that one may encounter in a specialty that includes       tion, certain conditions may be seen only once every decade
               bizarre congenital abnormalities.                                   or so. If they are not captured on film at the time of presenta-
                  The first chapter focuses on antenatal diagnosis, in recog-      tion, and before their operative correction, it may take some
               nition of the fact that nowadays most structural congenital         time before another similar case presents. In this regard, the
               abnormalities are diagnosed antenatally, and the paediatric         editors are grateful to the many contributors who have helped
               surgeon becomes involved in the care of the unborn infant           ‘fill the gaps’. Third, some conditions have no external clinical
               and its family well before birth. In this chapter we have delib-    or radiological features that lend themselves to photography,
               erately included the type of ultrasound images encountered          which means that they tend to be ‘down-played’ in a pictorial
               by paediatric surgeons in their everyday practice rather than       book of this type. This is not to ignore or deny their impor-
               concentrating on some of the recently introduced but not            tance, but obviously those conditions that are easily demon-
               always widely available ‘state of the art’ imaging techniques.      strated photographically will tend to gain greater prominence.
               The second chapter deals with major neonatal abnormalities          For this reason, it is important that this book should be read
               that are apparent at, or shortly after, birth; some will have       in conjunction with a standard paediatric surgery text if com-
               been diagnosed several months prior to birth. Chapters 3            prehensive coverage of the specialty is required.
xv
              Fig. 1.1 An abortus with an intact amniotic sac containing         Fig. 1.2 Exomphalos in a 12-week fetus showing prolapse of
              a fetus within it. At least 10% of pregnancies abort in the        the liver into the sac.
              embryonic stage (the first 8 weeks of gestation), mostly from
              chromosomal anomalies or gross malformations.
           Fig. 1.3 Exomphalos in an aborted fetus demonstrated on            Fig. 1.4 Exomphalos in a fetus with trisomy 13. Doppler flow
           postmortem babygram. It displays the relatively small size of      is seen in the umbilical cord vessels (arrow).
           the abdomen compared with the volume of bowel and liver
           in the sac. This illustrates why return of the sac contents into
           the abdominal cavity after birth can be difficult.
           Fig. 1.5 Gastroschisis. Multiple loops of bowel have extruded      Fig. 1.6 Antenatal scan at 16 weeks’ gestation showing trunk
           through a defect in the anterior abdominal wall.                   and umbilical cord (arrow) with bowel loops protruding just to
                                                                              the right of the attachment of the umbilical cord.
Liver
Bowel
Stomach
           Fig. 1.7 Colour Doppler ultrasound scan in a                       Fig. 1.8 Gastroschisis. Transverse abdominal ultrasound scan
           32-week gestation fetus with gastroschisis showing blood           of a 19-week gestation fetus showing stomach and liver in
           flow in the extra-abdominal mesenteric vessels.                    the abdomen and extruded bowel.
Liver
              Fig. 1.9 A giant exomphalos containing the liver in a          Fig. 1.10 Scan at 24 weeks’ gestation showing a congenital
              16-week gestation fetus. Note the size of the exomphalos in    diaphragmatic hernia with a fluid-filled cavity (stomach, ST)
              comparison to the abdominal circumference.                     beside the heart (HRT).
Left
Stomach
               10
                                           Heart
              Fig. 1.12 Antenatal scan at 29 weeks’ gestation showing        Fig. 1.13 First postnatal chest radiograph of the same infant
              a fluid-filled stomach beside the heart in a left-sided        as in Fig. 1.12 confirming the diagnosis of a left-sided
              diaphragmatic hernia.                                          congenital diaphragmatic hernia.
           Fig. 1.14 4D ultrasound scan showing a cervical lymphatic   Fig. 1.15 EXIT procedure on the same infant with a cervical
           malformation. This baby was delivered by the ex-utero       mass lesion.
           intrapartum treatment (EXIT) procedure.
              Fig. 1.19 Same fetus as in Fig. 1.18 on 2D ultrasound          Fig. 1.20 Colour Doppler scan at 30 weeks’ gestation
              scanning showing the cleft lip and palate.                     showing an intra-abdominal fluid-filled cavity separate from
                                                                             the bladder, consistent with a duplication cyst.
                                                                             (B)
                                                                             Figs. 1.22A, B (A) Antenatal ultrasound scan through the
                                                                             trunk showing showing two fluid-filled cavities in the upper
                                                                             abdomen, consistent with duodenal atresia. (B) Same baby
              (A)                                                            at 3 hours after birth with classic double bubble on x-ray.
           Fig. 1.23 Scan at 28 weeks’ gestation showing a double-           Fig. 1.24 Coronal scan of a fetus at 18 weeks showing
           bubble sign in another fetus with duodenal atresia.               echogenic bowel. Echogenic bowel may be seen in
                                                                             otherwise normal fetuses but can also be a marker for cystic
                                                                             fibrosis, intrauterine viral infection and aneuploidy.
           Fig. 1.25 Antenatal scan showing echogenic masses in the          Fig. 1.26 Large multicystic ovary in a fetus at 34 weeks’
           liver view, consistent with fetal gallstones or biliary sludge.   gestation. Early postnatal follow-up is required.
Stomach
           Fig. 1.27 Transverse ultrasound scan of the upper abdomen         Fig. 1.28 Congenital pulmonary airway malformation
           at 14 weeks’ gestation showing a hepatic cyst.                    (CPAM) involving the right lower lobe in a 30-week gestation
                                                                             fetus on transverse section.
              Fig. 1.29 CPAM in the right lower lobe on a longitudinal         Fig. 1.30 Likely extralobar pulmonary sequestration as
              ultrasound scan of the fetus.                                    evident by the large vessel running directly off the aorta.
              Fig. 1.31 The fetal thymus is a large structure, here exposed    Fig. 1.32 Hemivertebrae (arrow) visible on a 28-week
              after removal of the left chest wall. The phrenic nerve can be   antenatal ultrasound scan.
              seen running behind it, over the surface of the pericardium.
              (A)                                                              (B)
              Figs. 1.33A, B (A) Ultrasound scan at 20 weeks’ gestation showing both feet, with a normal right foot and club foot on the
              left. (B) 3D reconstruction of the same fetus at 24 weeks clearly showing bilateral talipes equinovarus.
                                                                                                  Head
           Fig. 1.34 Twelve-week’ gestation ultrasound scan showing      Fig. 1.35 Spina bifida was diagnosed in this fetus at
           the head and face of a fetus with anencephaly.                18 weeks’ gestation. The lemon-shaped head is evident on
                                                                         ultrasonography.
           Fig. 1.36 Posterior view of the fetal skull showing           Fig. 1.37 Spina bifida of the lumbosacral spine on an
           dilatation of the posterior horns of the lateral ventricles   antenatal scan showing an obvious gap in the dorsal arches
           in hydrocephalus.                                             (between the + markers).
           Fig. 1.38 Spina bifida showing a lumbosacral defect, which    Fig. 1.39 Transverse scan of the trunk at 30 weeks’ gestation
           is probably myelomeningocele filled with cerebrospinal        showing the myelomeningocele containing CSF (arrow).
           fluid (CSF).
              Fig. 1.40 Ultrasound scan at 28 weeks’ gestation showing a          Fig. 1.41 Pelvicalyceal dilatation in the left kidney evident at
              duplex right kidney.                                                28 weeks’ gestation.
           Fig. 1.44 Duplex left kidney with dilatation of the lower pole   Fig. 1.45 Colour Doppler scan at 28 weeks’ gestation
           moiety.                                                          showing both kidneys with some dilatation of the left
                                                                            kidney and multiple cysts in the right kidney, suggestive of a
                                                                            multicystic dysplastic kidney.
           Fig. 1.46 Longitudinal view of the trunk showing                 Fig. 1.47 Scan of the fetal pelvis showing a dilated bladder
           hydroureteronephrosis and a full bladder in a fetus with         with a large ureterocele, which may prolapse into and block
           posterior urethral valves.                                       the urethra.
                                                                            Fig. 1.48 The fetal adrenal is almost the same size as the
                                                                            fetal kidney. In this 16-week fetus, sectioned at the mid-
                                                                            abdominal level, the right adrenal dominates the right
                                                                            kidney on which it sits.
11
                A fistula opening on the perineal skin is easily identified    or female. In the commonest autosomal recessive variety,
           when it transmits meconium or air, but the opening can be           deficiency of the adrenocortical enzyme 21-        hydroxylase
           tiny and requires a careful search with magnification and           causes low cortisol levels and a compensatory increase in
           good illumination. In high lesions in males, the presence of        secretion of pituitary adrenocorticotrophic hormone, result-
           a rectourethral or rectovesical fistula can be inferred if air or   ing in adrenal hyperplasia. The excessive androgens produced
           meconium is passed per urethram. The fistula is usually con-        in females cause virilisation of the external genitalia but
           firmed by subsequent contrast studies (distal loopogram and         the internal anatomy is normal for a female. Investigations
           cystourethrography), which show ‘beaking’ of the terminal           include estimation of serum electrolytes and blood glucose
           rectum where the contrast enters the urinary tract.                 (to detect hyponatraemia, hyperkalaemia and hypoglycae-
                The use of an ‘invertogram’ (or shoot-through lateral          mia), serum 17-hydroxyprogesterone and urinary pregnane-
           radiograph of the pelvis with the baby prone) at 12–24 hours        triol. Chromosomal analysis is mandatory and screening for
           of age, once gas reaches the distal bowel, may provide addi-        the known genes involved in sex development is becoming
           tional information on the level of the abnormality. The radio-      more common. A urogenital sinogram or urethroscopy will
           graph is taken in an exact lateral projection with the baby         show a masculinised urethra and the presence of a vagina and
           being placed prone over a padded wedge. Gas rises to the            cervix. A pelvic ultrasound confirms the presence of a uterus,
           apex of the blind-ending rectum, and the level is related to        Fallopian tubes and ovaries.
           various skeletal landmarks corresponding to the levator sling.          Some infants are born with normal male chromosomes
           There is less reliance on plain radiographs these days to deter-    and testes but with insensitivity to androgens. A genetic
           mine the level of the anorectal malformation because clinical       abnormality in the androgen receptor system prevents the
           examination with or without a perineal ultrasound scan is           normal target tissues for androgens (Wolffian ducts and exter-
           often sufficient.                                                   nal genitalia) from responding to androgen stimulation. The
                Neonates with an anorectal malformation require inves-         abnormality may be partial or complete. In the complete form
           tigation for associated anomalies: an ultrasound scan of the        (complete androgen insensitivity syndrome, CAIS), the exter-
           urinary tract and spine and radiographs of the chest and spine.    nal genitalia are completely female in appearance. The clue to
            Some also need an echocardiogram and/or          karyotyping.     the gonads being testes in a phenotypic female is their discov-
            Those with ‘high’ lesions usually require management with          ery in inguinal herniae. The normal secretion and response
            a temporary diverting stoma and are further investigated at a      to anti-Müllerian hormone (AMH) leads to regression of the
            later date by contrast studies ± endoscopy.                        Müllerian ducts, while failure to respond to androgens pre-
                                                                               vents the development of a vas deferens.
                                                                                   Disorders of sex development may be produced by dyspla-
           AMBIGUOUS GENITALIA (DISORDERS OF                                   sia of one or both testes. When both testes are dysplastic, defi-
                                                                               ciency of androgen may cause incomplete virilisation, and
           SEXUAL DEVELOPMENT [DSD])                                           deficiency of AMH may allow persistence of Müllerian duct
           Genitalia are considered ambiguous when one or more of the          structures. Usually, both testes are undescended because they
           following features are present:                                     are unable to secrete enough hormones to enable testicular
                                                                               descent. In the asymmetrical form of dysplastic testes (mixed
           1 The phallus is too large for a clitoris or too small for a        gonadal dysgenesis), one gonad may be a testis that has
             penis.                                                            descended with preservation of the ipsilateral Wolffian duct
           2 The urethral opening is proximal, near the labioscrotal           and local regression of the Müllerian duct. The more dysplas-
             (genital) folds.                                                  tic testis usually remains in the abdomen and has failed to
           3 The genital folds remain unfused, giving the appearance of        cause regression of the Müllerian duct or preservation of the
             labia or of a cleft scrotum.                                      Wolffian duct. Infants with mixed gonadal dysgenesis may
           4 The testes are either not descended or impalpable.                appear to be males with severe hypospadias and one palpable
                                                                               testis. These dysplastic testes have an increased risk of malig-
               There is variation in the degree of abnormality that may        nant degeneration.
           be present, making gender assignment at birth hazardous.                In severe hypospadias with undescended testes the phallus
           Prompt and accurate diagnosis is imperative because of              may be large enough to indicate that the infant is essentially
           the social implications for the parents of not being able to        male, and usually the chromosomal karyotype is normal
           announce the gender of the baby, and the malformation may           male (46, XY). A contrast urethrogram will demonstrate a
           be the outward sign of the life-threatening condition congen-       normal male urethra without a vagina or uterus. The testes
           ital adrenal hyperplasia. When congenital adrenal hyperpla-         may be impalpable (in the inguinal canal or abdomen) or pal-
           sia occurs in females, the appearance of the external genitalia     pable near the pubic tubercle, on one or both sides. Severe
           may cover a broad spectrum, suggestive more of either male          hypospadias with undescended testes has to be distinguished
[LU 1.5]
a) Kom ons lees oor nog ‘n arend. Luister en volg eers terwyl jou opvoeder
die volgende teks aan julle voorlees.
As gevolg van sy suksesse in die sakewêreld het hy een van die rykste mense
in die wêreld geword. Hy het die Rembrandt-tabakmaatskappy met slegs
R20 begin en dit tot ‘n reusebesigheid uitgebou. Later het hy ook Rothmans
International in Europa gevestig, en nog later ook Richemont gestig.
Laasgenoemde is ‘n maatskappy wat ander maatskappye besit, soos Dunhill,
Mont Blanc en Cartier, wat luukse goedere soos juwele verkoop. Sy
Richemont-besigheid is ongeveer R128 miljard werd!
In die laaste jare van sy lewe het hy amper al sy tyd gewy aan sy ideaal om
‘n netwerk oorgrensparke tussen lande in Suider-Afrika te vestig. Dit moes
toerisme bevorder en werk skep vir werklose mense. Daarom het hy in 1997
die Stigting vir Vredesparke saam met oudpresident Nelson Mandela en
wyle prins Bernard van Nederland begin. Behalwe sy bewaringswerk in
Suider-Afrika, het hy ook daarmee gehelp in Europa, Suid-Amerika en
Suidoos-Asië. Verder het hy in die 1960’s gesorg dat gratis mediese dienste
vir 25 jaar lank aan die mense van Lesotho gelewer word.
Baie van ons historiese geboue is deur sy toedoen gered, soos die
vissershuisies van Kassiesbaai op Waenhuiskrans, die Van Wouw-
huismuseum in Pretoria, die Reinethuis en die Drostdy-hotel op Graaff-
Reinet, die wit huise in die middedorp van Stellenbosch, die Bo-Kaap-
museum en talle, talle meer.
AKTIWITEIT 2
[LU 3.1.2]
b) Gebruik die leesstuk om in julle groepe hardop aan mekaar voor te lees.
Probeer veral om duidelik en vloeiend te lees en om die moeiliker woorde
korrek uit te spreek.
AKTIWITEIT 3
[LU 3.1.1]
c) Gebruik nou die leesstuk om die antwoorde op die volgende vrae neer te
skryf:
In watter provinsie is Graaff-Reinet? (Jy kan ook jou atlas gebruik om te kyk
waar dié dorp is).
Dink jy dr. Rupert was regtig ‘n groot Suid-Afrikaner? Gee minstens twee
redes vir jou antwoord.
Wenk: breek die woord eers in lettergrepe op en dink goed daaroor voordat
jy die betekenis hier neerskryf.
v) Ons het gelees van wyle dr. Anton Rupert en wyle Prins Bernard. Wat
beteken dit as die woord “wyle” voor iemand se naam geskryf of gesê word?
As jy nie weet nie, sal jy dit op bladsy 1405 in die HAT vind.
As mens ‘n stuk skryfwerk van goeie gehalte wil doen, is dit nodig om dit
deeglik voor te berei. Jy moet jou gedagtes eers agtermekaar kry en dit selfs
met iemand anders bespreek. Jy moet ook moeite doen om die regte inligting
te kry. Op die volgende bladsye gaan julle so ‘n proses volg.
AKTIWITEIT 4
[LU 4.2.1]
Stap 1:
Julle opvoeder sal julle in klein groepe verdeel (liewer nie minder as drie in
‘n groep nie). Julle eerste taak is om julle onderwerp te kies, naamlik die
naam van ‘n Suid-Afrikaner wat julle as “ ‘n arend” beskou en oor wie julle
wil skryf. Elke groep moet ‘n ander “arend” kies. Elke groeplid moet
minstens een naam voorstel en redes gee waarom hy / sy die persoon
voorstel. Die groepie moet alle voorstelle bespreek en uiteindelik een naam
kies.
Wat is belangrik?
Julle ouers mag julle vooraf help met name wat julle kan voorstel.
Wenk: Dink bv. aan Suid-Afrikaners wat presteer het in sport, politiek, die
vermaaklikheidswêreld, die mediese wetenskap of ander wetenskappe,
liefdadigheid, die versorging van mense, die kunste en musiek. Julle moet
egter seker maak dat julle meer as een bron van inligting oor die persoon kan
bekom.
AKTIWITEIT 5
[LU 5.3.3]
Stap 2:
Wanneer julle jul keuse gemaak het, moet julle besluit watter soort inligting
julle oor die persoon gaan soek, bv. kinderjare, opleiding, prestasies,
persoonlike eienskappe (soos Roland Schoeman se lojaliteit aan SA of dr.
Rupert se nederigheid). Maak daarvan aantekeninge sodat julle sal weet
waarna om te soek en uiteindelik ‘n inhoudsopgawe kan saamstel.
AKTIWITEIT 6
[LU 5.3.1]
Stap 3:
Elke groeplid bring die inligting (getik, geskryf, prente, knipsels, boeke,
sketse) wat hy / sy versamel het klas toe en deel dit met die ander groeplede
sodat almal oor dieselfde inligting beskik. Besluit sommer ook oor die
volgorde waarin julle die inligting gaan plaas en stel ‘n voorlopige
inhoudsopgawe op. Skryf dit onder mekaar neer.
Tot hier het jy saam met jou groeplede gewerk. Van nou af is jy eers weer op
jou eie.
AKTIWITEIT 7
OM ‘N KONSEPWEERGAWE TE SKRYF MET ‘N SENTRALE IDEE
EN GOED ONTWIKKELDE PARAGRAWE
[LU 4.2.3]
Stap 4:
Jy moet die inligting wat deur jou groeplede versamel is so deeglik bestudeer
dat dit vir jou voel asof jy die persoon self ken. Eers daarna kan jy begin
skryf. Jou eerste poging sal op los papier wees, sodat jy jou gedagtes goed
agtermekaar kan kry. Wanneer jy daarmee tevrede is, moet jy jou
konsepweergawe netjies op ‘n skoon vel papier of in jou werkboek
neerskryf, onder die opskrif: Inhoudsopgawe.
aktiwiteit 8
[lu 4.2.5]
Stap 5
Hersien jou werk deur dit self deeglik te lees. Gebruik jou woordeboek om
seker te maak dat jy spelfoute regstel en dat jy hoofletters en ander
leestekens reg gebruik het. Is jou inhoud korrek? Handel elke paragraaf oor
gedagtes wat bymekaar hoort?
AKTIWITEIT 9
[LU 4.2.5]
Stap 6
In hierdie stap moet julle nou julle skryfwerk aan die ander groeplede wys.
Julle moet probeer om mekaar goeie raad te gee oor hoe julle jul werk kan
verbeter en watter foute julle nog kan uitskakel. Julle opvoeder kan ook
hiermee help. Skryf die verbeteringe in ‘n ander kleur as jou
konsepweergawe neer.
AKTIWITEIT 10
[LU 4.2.5]
Stap 7
Dink ‘n gepaste naam vir jou werkstuk uit en skryf dit bo-aan. Dit word
gevolg deur jou inhoudsopgawe. Daarna skryf jy jou finale weergawe sodat
dit gereed kan wees vir assessering.
Taalstruktuur en -Gebruik
AKTIWITEIT 11
[LU 6.1.11]
a) Leenwoorde
b) Nuutskeppinge
Afrikaans is ‘n taal wat “lewe” en “groei”, want daar word gedurig nuwe
woorde in ons taal bygevoeg. Die meeste van hierdie nuwe woorde kom uit
leerareas soos Tegnologie en Elektronika, omdat daar gedurig nuwe
ontwikkelinge en uitvindings is. Dink maar aan nuwe woorde wat ontstaan
het uit die uitvinding en ontwikkeling van die sellulêre foon en die digitale
kamera. Die volgende verrassende voorbeeld kom in die 2005-uitgawe van
die HAT (bladsy 1051) voor:
Bemagtig:
Vigs:
CD-ROM:
(ii) Probeer om sinne te maak waarin elk van die volgende nuutskeppinge se
betekenis duidelik is:
voëlgriep:
biep:
AKTIWITEIT 12
OM DIE VERSKILLENDE KOMPONENTE VAN SINNE
(ONDERWERP, VOORWERP, GESEGDE) UIT TE KEN EN TE
VERSTAAN
[LU 6.2.1]
Julle het alreeds in graad 4 kennis gemaak met die verskillende dele
(komponente) waaruit sinne bestaan, naamlik die onderwerp, gesegde en
voorwerp. Kom ons hersien dit gou.
Om die komponente van ‘n sin uit te ken, is dit die maklikste om by die
gesegde te begin. Jy moet net onthou dat die gesegde die woord(e) in die sin
is wat aandui of die sin in die teenwoordige, verlede of toekomende tyd
geskryf is. Dit is dus gewoonlik ’n werkwoord (teenwoordige tyd), maar dit
word ook saam met ‘n hulpwerkwoord soos sal of het gebruik in die
toekomende en verlede tyd. Om die drie komponente uit te ken, moet jy drie
vrae vra.
Vraag 2: Wie of wat vang? Die antwoord op die vraag is die onderwerp,
naamlik: Die kaptein.
Vraag 3: Wat vang hy? Die antwoord op die vraag is die voorwerp, naamlik:
die bal.
Gesegde: vang
Hier volg nou drie enkelvoudige sinne. Gebruik die voorbeeld hierbo om die
drie vrae korrek te vra sodat jy kan vasstel wat die onderwerp, gesegde en
voorwerp in elke sin is.
Onderwerp:
Gesegde:
Voorwerp:
Onderwerp:
Gesegde:
Voorwerp:
Onderwerp:
Gesegde:
Voorwerp:
Maak nou drie enkelvoudige sinne van jou eie – een in die teenwoordige,
een in die verlede en een in die toekomende tyd. Breek elke sin in sy
komponente op en dui so aan wat die onderwerp, gesegde en voorwerp in
elke sin is.
Onderwerp:
Gesegde:
Voorwerp:
Sin 2 (Verlede tyd):
Onderwerp:
Gesegde:
Voorwerp:
Onderwerp:
Gesegde:
Voorwerp:
AKTIWITEIT 13
[LU 6.2.3]
Jy praat nie altyd in kort, enkelvoudige sinne nie. Jou sinne is dikwels
langer, omdat jy woorde byvoeg wat beter beskryf wat jy eintlik wil sê. In
die volgende sinne is die skuinsgedrukte woorde bygevoeg om ‘n duideliker
beskrywing te gee.
My persoonlike woordelys
                                  Table 1.15.
                         Woord Opgebreek Kort sin
Assessering
LU 1
LUISTER
LU 3
LEES EN KYK
Die leerder is in staat om vir inligting en genot te lees en te kyk en krities op
die estetiese, kulturele en emosionele waardes in tekste te reageer.
3.1.2 voer luidlees duidelik uit en pas leesspoed volgens doel en gehoor aan.
LU 4
SKRYF
4.2.7 lewer finale weergawe in en besin oor die assessering van die stuk.
LU 5
DINK EN REDENEER
Die leerder is in staat om taal vir dink en redeneer te gebruik en inligting vir
leer te verkry, verwerk en gebruik.
LU 6
TAALSTRUKTUUR EN -GEBRUIK
6.4.6 toon kennis van die gebruik van bekende idiomatiese uitdrukkings.
Memorandum
Aktiwiteit 1
a) Luisteroefening.
Aktiwiteit 2
b) Leesoefening.
Aktiwiteit 3
(iv) oor-grens-park
Taalstruktuur en -gebruik
Aktiwiteit 11
a) Leenwoorde
b) Nuutskeppinge
Infrastruktuur: Stelsels wat mekaar ondersteun sodat sake glad verloop, bv.
vervoer, gesondheidsdienste, bankwese, mynwese, ens.
CD-ROM: ‘n Kompakte skyf in die rekenaar wat data vertoon wat slegs
gelees kan word en nie verander kan word nie.
(ii) Voëlgriep is ‘n siekte wat onder voëls voorkom en wat tans na al hoe
meer lande versprei.
Die selfoon het ‘n biepgeluid gemaak om aan te toon dat daar ‘n SMS is.
a) Onderwerp: Petro
Gesegde: skryf
Voorwerp: ‘n brief.
b) Onderwerp: Hy
c) Onderwerp: Pappa
Voorwerp: my.
Eie sinne.
Aktiwiteit 13
Graad 5
SO MAAK MENS
Module 6
AKTIWITEIT 1
AKTIWITEIT 2
[LU 3.1.1]
[LU 3.5.2]
Luister eers hoe jou opvoeder die storie van Dommie en die goue gans
voorlees. Lees dit dan op jou eie en beantwoord daarna die vrae.
‘n Man en vrou het drie seuns gehad. Die jongste se naam was Dommie,
want almal het gedink hy is maar dommerig. Almal by die huis het altyd met
hom gespot en hom verkleineer.
Die oudste seun het eendag besluit om te gaan hout kap in die woud. Sy ma
het vir hom ‘n heerlike pastei gebak en ook vir hom ‘n bottel goeie
koeldrank gegee sodat hy darem lekker kon eet as hy sou honger word van al
die harde werk. In die woud kom hy ‘n klein ou mannetjie teë en dié sê vir
hom: “Goeiedag, jong man. Gee asseblief vir my ‘n klein stukkie van jou
pastei en ‘n bietjie koeldrank uit jou bottel, want ek is baie honger en dors.”
Maar die slim jong man sê: “Ek vir jou van my vleis en koeldrank gee? Nee,
ek sal nie genoeg vir myself oorhê nie,” en hy stap verder.
Hy begin kap toe aan die eerste boom, maar die ou mannetjie het sy byl
getoor, want skielik gly die byl en hy kap sy been raak. Die wond was so
groot dat hy nie verder kon werk nie en hy moes dadelik huis toe gaan sodat
die wond verbind kon word.
Die tweede seun besluit toe om te gaan hout kap in die bos. Sy ma gee vir
hom ook ‘n heerlike pastei en ‘n bottel koeldrank vir sy middagete. Hy
ontmoet dieselfde mannetjie langs die pad, met dieselfde versoek: “Gee my
asseblief so ‘n ietsie van jou pastei en koeldrank, want ek is honger en dors.”
Hierdie seun het ook gedink hy is slim, want hy sê: “Hoe meer jy eet, hoe
minder is daar vir my. Gaan weg!”
Die ou mannetjie het ook sy byl getoor, want toe hy die tweede hou daarmee
kap, kap hy ook sy been raak. Hy moes ook huis toe gaan om die wond te
laat behandel.
Dommie sê toe vir sy pa: “Pa, ek wil ook graag gaan hout kap.” Maar sy pa
antwoord: “Jou twee broers het hulleself beseer. Jy moet maar liewer by die
huis bly, want jy weet tog niks van houtkap af nie.” Maar Dommie hou aan
neul en uiteindelik stem sy pa in: “Nou goed dan; gaan tog maar, maar jy sal
gou jou les leer.” Sy ma gee toe vir hom ’n ou stuk droë brood en ‘n bottel
suur bier en so is hy daar weg.
In die bos ontmoet hy dieselfde ou mannetjie wat vra: “Kan ek asseblief van
jou pastei en koeldrank kry? Ek is baie honger en dors.”
Dommie sê: “Ek het net ou droë brood en suur bier, maar as jy dit wil hê,
kan ons saam daaraan eet en drink.” Hulle gaan sit op die gras en toe
Dommie sy ou brood uithaal, sien hy dat dit in die heerlikste pastei verander
het. Tot sy verbasing het ook sy suur bier in die lekkerste koeldrank
verander. Hulle het lekker geëet en gedrink en toe hulle klaar is, sê die
mannetjie: “Omdat jy so goedhartig is en gewillig was om alles met my te
deel, sal ek iets goeds vir jou doen. Sien jy daardie ou boom? Kap dit af en
jy sal iets daaronder vind.” Toe stap hy weg.
Dommie het dadelik begin kap en toe die boom val, kry hy ‘n gans met goue
vere onder die boom!
(Word vervolg)
                                 Table 1.16.
                                           Ja Nee
                          Goeie maniere
                           Eerbied
                           Respek
                           Selfsugtigheid
Dink jy Dommie se ma was goed vir hom? Gee redes vir jou antwoord.
                                  Table 1.17.
                                             Ja Nee
                          vriendelik?
                          mededeelsaam?
                          selfsugtig?
                          goedgemanierd?
Watter les dink jy kan ons uit hierdie eerste gedeelte van die storie leer?
Wat dink jy daarvan dat hulle die jongste broer Dommie genoem het?
AKTIWITEIT 3
[LU 3.1.2]
Oefen tuis om die storie hardop te lees. Gee veral aandag aan vlotheid en
duidelike uitspraak. Probeer ook om jou stem reg te gebruik in die dialoog-
gedeeltes, dit wil sê waar mense met mekaar praat. Kom lees dit dan in julle
groepe hardop aan mekaar voor. Jou opvoeder kan dalk ook versoek dat jy
dit aan die klas voorlees. Wees dus gereed daarvoor!
AKTIWITEIT 4
Bespreek in julle groepe wat julle van die jongste seun se bynaam dink. Gee
veral aandag aan die volgende:
a) Dink julle sy ouers het reg opgetree oor die benaming “Dommie”?
b) Watter mooi of aanvaarbare byname ken julle? Skryf dit neer. Dink aan
voorbeelde onder sportsterre, soos “Vinnige Fanie” (krieket), “Rocky”
(boks), “Super Sarel” (tydrenne), “Pistol Pete” (tennis), ensovoorts.
AKTIWITEIT 5
[LU 5.3.6]
Paragraaf 1
Paragraaf 2
Ek dink name kan mense seer maak
Taalstruktuur en -Gebruik
Woordsoorte
In hierdie module gaan ons vier verskillende soorte woorde probeer uitken.
Hulle is
Dit is woorde wat een of meer van die volgende eienskappe besit:
‘n verkleiningsvorm,
‘n enkelvoud en meervoud,
AKTIWITEIT 6
OM SELFSTANDIGE NAAMWOORDE TE IDENTIFISEER
[LU 6.2.11]
a) In elkeen van die vier sinne wat volg, is daar ‘n selfstandige naamwoord.
Kan jy hulle uitken? Skryf hulle neer en skryf ook die rede waarom jy sê dis
‘n selfstandige naamwoord, byvoorbeeld:
Ganse is “waghonde”
Ganse word beskou as uitstekende wagters in die nag. Sommige boere hou
ganse op die plaas aan en maak vir hulle slaapplek naby die kraal waar die
vee snags slaap. Indien daar ‘n bedreiging gedurende die nag is, is die ganse
altyd die eerste om dit agter te kom en hulle maak dan dadelik ‘n groot
lawaai. Sodoende word die boer gewaarsku en kan hy sy eiendom,
byvoorbeeld klein lammertjies, beskerm.
Verkleinwoorde
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