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Liver transplantation for infants and children has been available in South Africa at a single centre, the only established service in Sub-Saharan Africa, for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long-term. Materials and methods Since 1985, 225 infants and children have been assessed, with 146 accepted for transplantation. Sixty-nine have had 71 orthotopic liver transplants (OLTx). Biliary atresia was the most frequent diagnosis (54%) followed by acute liver failure (ALF) (15%). Waiting list mortality has remained high (23%), particularly for the ALF group (50%). Forty-three were reduced size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1. Twenty-seven were Results Fifty (74%) survive 1 month-12 years post-transplant. Actuarial survival after 1996 since HBV core antibody positive donor livers were refused and prophylactic IV ganciclovir used has been >82%. Early post-OLTx mortality was low (5%), one primary non-function, one IVC thrombosis, one PV thrombosis, but late morbidity and mortality (20%) was mainly due to viral infection: de novo hepatitis B (five patients, three deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (eight patients, six deaths) and CMV disease (11 patients, five deaths). Tuberculosis prophylaxis, required in six cases, resulted in major morbidity in two and mortality in one. Poor compliance played a significant role in seven deaths. Hypertension requiring medication along with some compromise of renal function has been present in all but two patients. However, all those of school-going age (25) attend school normally and remain in good health and only three of the survivors have abnormal liver function tests. Conclusions Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent re-transplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems. Intense education of the caregiver and close follow-up, particularly of those living at long distances has partly addressed the compliance problem.

The subject of malrotation and midgut volvulus in infancy and childhood is reviewed from the perspective of experience with 138 patients evaluated in a published series and a further 82 cases seen since. Embryology, historical aspects, clinical presentation, investigation, surgery, and outcome are discussed. The diagnosis of malrotation and volvulus should always be kept in mind when assessing any infant or child with symptoms of vomiting and pain, particularly when the vomiting is bile-stained. The diagnosis cannot be excluded if diarrhea is present, when clinical findings are bland, or even with normal abdominal radiographs. A contrast meal and follow-through should be done and, if correctly interpreted, should be diagnostic. Ultrasound examination may be helpful but is not secure enough to exclude the diagnosis. Laparotomy or laparoscopy is the only way to be sure. Malrotation with its propensity for volvulus is truly a time bomb lying within.

This paper records our experience in the management of 25 sets of conjoined twins seen over a 32 year period (1964 - 1996). The twins were classified into 14 complete and symmetrical sets and 11 incomplete or heteropagus. The 14 symmetrical sets included 9 thoracopagus, 2 ischiopagus, 1 craniopagus and 1 omphalopagus twins. In the incomplete heteropagus group there was 1 ischiopagus, one twin being anencepahlic, 2 dipygus, 5 parasitic, 2 fetus-in-fetu and I cranial and caudal. The management is detailed case by case. Overall 10 of 14 symmetrical sets underwent attempts at separation with 16 surviving the procedure, but there were 3 late deaths. In the incomplete group 10 of 11 were operated on with 9 survivors. The importance of a multi-disciplinary approach, the extensive investigations required pre-operatively to define areas of organ and bony conjunction, congenital anomalies of each twin and surgical teamwork is emphasized. Specific problems encountered were identified. In thoracopagus twins the hearts were of paramount importance as conjuction was usually fatal, being associated with major congenital defects. The greater the extent of thoracic cage fusion the greater the chance of associated severe anomaly. Skin expansion to assist coverage of the defects after separation was of great assistance, as was the use of collagen coated vicryl. Evaluation of the liver and pancreatico-biliary systems with isotope excretion scanning was crucial to pre-operative planning. Where there was fusion of the duodenum a single pancreatico-biliary system could be expected and prior strategies for separation and Roux-en-Y enteric drainage of both pancreatic and biliary secretion should be planned. Gastro-oesophageal reflux led to considerable morbidity in both twins of a thoraco-omphalopagus set. In ischiopagus and dipygus conjoined twins bilateral posterior iliac osteotomies were an essential component to anatomic reconstruction of the pelvic ring and wound closure. Also in this group, due to the frequency and extent of shared genital, urinary and ano-rectal structures, long-term morbidity was expected and a component of this might be due to spinal cord tethering, or as in one of our cases, a progressive hydrosyringomyelia. Timing of separation was ideally set at between 5 and 9 months with 6 to 8 weeks of prior tissue expansion but earlier operation was frequently required because of cardio-respiratory problems or organ failure in one twin. In most cases the goal of obtaining separate, independent and intact individuals was achieved.

One hundred seventy-eight of 330 patients were recalled after undergoing surgery for histologically proven Hirschsprung's disease (HD). One hundred fifteen were older than 4 years at interview (Mean age, 10 years). This sample appeared to be representative of the whole in terms of demographic features such as ethnic group, sex, length of aganglionic segment, timing of presentation and surgery performed. Anthropomorphic indices for weight and height were comparable to norms, but many younger patients were below expected weight for age. In general, weight and height for age was regained with time. Nine patients had delayed developmental milestones, which were owing to specific causes in four. Nine patients had a poor functional outcome, of which two had neurological impairment. Satisfactory school performance was achieved in all but 19 (26%) of the remaining patients. Long-term functional results were comparable for the Soave and Duhamel procedures with less favorable results noted following the Swenson procedure. Assessment of complications demonstrated a significantly (P < .01) lower incidence of constipation, sexual dysfunction, and micturition disturbance following the Soave procedure when compared with the Duhamel and Swenson procedures. Neurological impairment and length of aganglionic segment beyond the rectosigmoid area appeared to influence functional outcome, as did persisting enterocolitis. Enterocolitis was observed in 16.6% of patients on presentation, but continued in only 6%. Constipation was particularly associated with the Duhamel procedure, and a higher incidence of micturition disturbance, abdominal distension, and cuff stricture was noted following the Swenson procedure. Functional assessment by three different scoring methods showed that 86 (74.7%) of the 115 patients over the age of 4 had excellent anorectal function and appeared to be well adjusted. Twenty-two patients (19.2%) had relatively minor long-term problems but seven (6.1%) had persistent fecal soiling with resulting psychosocial maladjustment.

Iatrogenic esophageal perforations in children are rare. To evaluate patterns of injury, clinical presentation, and treatment options for such patients, the authors reviewed the case records of 11 children who had sustained transmural injury to the esophagus during a dilatation procedure at their institution between 1967 and 1994. Strictures requiring dilatation were attributable to caustic ingestion in eight, esophageal atresia repair in two, and congenital stenosis in one. Eight were penetrating injuries, and three were disruptions. Two involved the cervical esophagus; and nine involved the thoracic esophagus. Pain, pyrexia, and tachycardia were early signs. Proximal thoracic perforations led to signs in the left chest region (effusion/pneumothorax); with distal perforation the signs were on the right side. Treatment along conventional lines (local drainage, gastrostomy, primary repair after early recognition, and antibiotic therapy) was successful, with two major complications, both empyemas. The most recent case, a disruption of a tracheoesophageal fistula stricture, was successfully treated with oral water irrigation and antibiotics only. Six had colonic interposition (all caustic ingestion), one required endoesophageal resection of a distal stricture, and four had resolution of the stricture, three without additional dilatation. The majority of children with iatrogenic injuries of the esophagus can be managed successfully by conservative measures and pleural drainage, with surgical procedures reserved for large disruptions of the esophagus, intraabdominal perforations, and cases that do not respond to appropriate conservative measures. Patients with caustic injury to the esophagus have a greater risk for the development of penetrating injury, and this may be one indicator of the severity of scarring. There are distinct clinical patterns of presentation, which depend on the level at which the esophagus is perforated. Dilatation disruption of a localized stricture has a good long-term prognosis for the esophagus and may even cure the stricture. The role of oral irrigation still must be fully evaluated where disruption has occurred as distinct from a penetrating injury.

The significance of dysplastic features in the surgical pullthrough segment of bowel in patients with Hirschsprung's disease (HD) has not yet been clarified. The aim of this study was to evaluate prospectively the ganglionated proximal bowel in 26 patients with HD (January 1988 through January 1991). The significance of dysplastic features and their influence on post operative outcome were evaluated by means of a newly devised histological scoring system based on the morphological features. Functional outcome was assessed clinically at follow-up interview. Comparison was with control specimens from 22 patients undergoing unrelated bowel surgery and a further 5 patients with neuronal intestinal dysplasia (NID). Results indicated a wide spectrum of histologically identified dysplastic features in patients with NID, the ganglionated bowel of HD and controls. Although individual abnormal features were noted in the control group, significant degrees of dysplasia were absent. The overall degree of dysplasia was less striking than that observed in NID and in the 5 patients in whom NID co-existed with HD. Dysplasia of the ENS in residual bowel could be correlated with postoperative dysfunction in 4 out of 5 patients (80%) with HD and features of co-existing NID. In addition, milder symptoms were noted in 50% of patients having a borderline score (5-6/12). This study emphasizes the relationship between clinical obstructive symptoms and a high degree of dysplasia within the ENS. A histological grading system is of value in evaluating the spectrum of abnormal findings and prospectively identifying those with functional significance in patients with NID co-existing with HD.

Duodenal obstruction typically occurs in the ampullary region and presents in the early neonatal period. If the obstruction is incomplete, as with a fenestrated duodenal membrane, the presentation may be delayed and the diagnosis overlooked. Sixteen patients with fenestrated duodenal membranes presented over a 23-year period. Six presented in the neonatal period, with vomiting; an abdominal x-ray was diagnostic in four. Ten presented later (5 weeks to 14 years) with nonspecific symptoms, ie, failure to thrive, postprandial epigastric distension, and recurrent chest infections. Vomiting occurred in all, but was of short duration (1 week). Two patients in the delayed group had Down's syndrome. Diagnosis was confirmed on barium meal, and optimal surgical treatment was a bypass duodeno-duodenostomy. The diagnosis of fenestrated duodenal membrane must be considered in all cases with symptoms and signs suggestive of upper gastrointestinal tract obstruction, and excluded by contrast radiology.

Primary palmar hyperhidrosis can be a most unpleasant and distressing affliction. When normal daily activities, viz. writing, schoolwork, are interfered with and other treatments have failed, surgery is indicated. Between 1983-1987 9 children (6 F, 3 M), mean age 10.2 years (range 5-14 years) underwent bilateral transaxillary sympathectomy, 4 simultaneous and 5 1-4 weeks apart. On 14 sides a standard excision of dorsal ganglia (DG) 2, 3, and 4 was performed. The technique of lateral displacement of the sympathetic chain after transection distal to DG 4 and division of preganglionic fibres of DG 4, 3 and 2 was used on 5 sides. In addition to clinical evaluation a pilocarpine stimulation test was performed on the palms of the hands before and at follow-up 3-7 years after surgery. 17/18 hands appeared sympathectomised at early follow-up. One inadequate result required reoperation. Other complications included 2 Horner's syndromes--1 transient and 1 mild but permanent; 3 temporary intercostobrachial paraesthesias, 2 mild late recurrences of sweating and 2 compensatory increases in sweating. In 14 palms where sweat volume was measured before and 3-7 years after surgery there was a mean decrease in sweat of 84% (mg), those sweating the most prior to surgery having the best response. In 2 further palms insufficient sweat was obtained for testing. All but 1 considered the procedure worthwhile. Transaxillary transpleural sympathectomy is a safe, effective, and cosmetically acceptable operation to control symptoms of excessive palmar sweating.(ABSTRACT TRUNCATED AT 250 WORDS)

One hundred seventy-eight of 326 patients had surgery for Hirschsprung's disease at Red Cross Children's Hospital (1957 to 1990) agreed to participate in a recall follow-up study. Assessment of the postoperative outcome of the Swenson, Duhamel, and Soave procedures included manometric evaluation in 43 patients by perfused-tube and balloonseries techniques. Rectal suction biopsies were performed for patients who had persistent problems with stool evacuation. One hundred fifteen of the 178 patients were more than 4 years of age, and long-term functional outcome could be assessed. Although good results were obtained in 94% overall, 16 had clinical evidence of a degree of perrsisting obstruction. Results of manometric assessment of anorectal function in these patients were not significantly different from those of 28 patients who had normal stool evacuation. A biopsy was performed in 14 of the 16 patients who had symptoms of obstruction postoperatively, and abnormal histological features were noted. There was aganglionosis in four, features of neuronal intestinal dysplasia in nine, ganlioneuromatosis of the colon in one. The results of two biopsies were entirely normal. Implications of Postoperative dysfunction after surgery for Hirschsprung's disease are discussed, and a protocol for investigation and management is proposed.

In a series of 142 children treated for corrosive burns at the University of Cape Town teaching hospitals between 1957 and 1990, 55 developed strictures of the oesophagus. Prograde dilatations with or without a guiding trans-stricture string proved successful in 22 children while 33 had an oesophageal bypass procedure. The complications following dilatations, particularly the 10 perforations of the oesophagus, are reviewed. Three of these later responded successfully to further dilatations. Since 1969, a single-staged left colon interposition was employed as the procedure of choice. The operative technique of the left colon interposition is described and the early and late complications following the bypass procedures are detailed and discussed, stenosis and strictures at the upper anastomosis being the most significant.

Although the therapeutic approach to gastroesophageal reflux in children is well established, there are differences of opinion regarding the management of esophageal strictures, viz bougienage with medical therapy, fundoplication without dilatation, preoperative dilatation followed by fundoplication with intraoperative and postoperative dilatation, or resection and interposition. Sixteen consecutive children (mean age, 30.2 months) with reflux strictures were evaluated, constituting 12% of children operated on for gastroesophageal reflux. The strictures became clinically apparent 22.4 months (mean) from the onset of symptoms and were diagnosed by contrast studies and endoscopy. At first endoscopy all the patients had well-established fibrotic strictures. The strictures were mostly situated in the middle or lower esophagus and 7 were longer than 3 cm in length. All 16 were treated with antacids, H 2 -receptor blockers (Cimetidine), prokinetic agents, and intense nutritional resuscitation, together with preoperative stricture dilatations (average, 3.6 times). This was followed by fundoplication when nutritional parameters had been restored, esophagitis improved, and the strictures dilated to adequate size. Seven children required concomitant gastrostomies for prograde esophageal dilatations. Twelve children needed postoperative esophageal dilatations. The results were satisfactory in 14 (88%). Two required endoesophageal resection for localized unyielding strictures. One child responded only after failed reflux surgery was corrected at a second procedure. During an average follow-up of 8.2 years (range, 3 to 11) there has been no stricture recurrence and growth velocity was restored in all. We conclude that our preferred method is preoperative in-hospital management of gastroesophageal reflux with maximum nutritional support and careful evaluation of the degree and extent of esophagitis and fibrous scarring. Antire-flux surgery is delayed until nutritional deficiencies have been corrected, the esophagus adequately dilated, and acute esophagitis controlled. Long-term follow-up with endoscopic surveillance is advocated.

A new topical antiseptic agent, 5 per cent polyvinylpyrrolidone-iodine (PVP-I) cream, with altered physicochemical properties, incorporated in a different carrier base has proved in vivo to be more effective in controlling burn wound infections than 10 per cent PVP-I ointment. Important biodynamic properties of the new formulation have not, however, been elucidated in vivo. Hence the need for a controlled study to evaluate the bioavailability of the active component after penetration through burn eschar; the bactericidal efficacy of the cream and determination of the bactericidal time of the cream in comparison with 10 per cent PVP-I ointment. A modified Walker burn wound model was used to define the rate of trans-eschar penetration, biodynamic availability and bactericidal efficacy of 5 per cent povidone iodine cream in established Pseudomonas aeruginosa burn wound infection. In vitro penetration confirmed the effective diffusion of PVP-I cream through 1.5 mm eschar within 6 h. A single topical application of PVP-I cream resulted in a 98.8 per cent (6.088 x 10(9) c.f.u./g of tissue to 7.367 x 10(7) c.f.u./g of tissue) reduction in intra-eschar viable organisms within 18 h after application. A second topical application of PVP-I cream at 18 h resulted in a total reduction of 99.8 per cent in viable organisms (2.90 x 10(9) c.f.u./g of tissue to 7.009 x 10(6) c.f.u./g of tissue) within 48 h. Comparing the in vitro bactericidal time of povidone iodine ointment with cream against Pseudomonas aeruginosa, Staphylococcus aureus and a Klebsiella pneumoniae revealed that the PVP-I cream killed organisms ten-fold more quickly than the ointment.(ABSTRACT TRUNCATED AT 250 WORDS)

Conjoined twins represent a rare but fascinating congenital condition, the aetiology of which remains obscure. Over the past four decades, the paediatric surgeons at Red Cross Children's Hospital have been involved in the management of 46 pairs of conjoined twins, of which 33 have been symmetrical and 12 asymmetrical. Seventeen symmetrical twins have undergone separation with 22 children (65%) surviving; all of the live asymmetrical twins survived separation. We describe the important features of this unique cohort, outline our approach to management and present the results of this approach. We consider some of the ethical and moral dilemmas we have confronted, and discuss the prenatal diagnosis, obstetric implications and postnatal care of these children, including the relevant investigations and anaesthetic and surgical management. Specific aspects related to the cardiovascular system, hepatobiliary and gastrointestinal tracts, urogenital tract, central nervous system and musculoskeletal system are highlighted.

Background The liver transplant program for infants and children at the Red Cross Children's Memorial Hospital is the only established pediatric service in sub-Saharan Africa. Since 1985, 250 infants and children have been assessed and 155 accepted for transplantation. Methods Since 1987, 76 children (range 6 months to 14 years) have had 79 liver transplants, with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (n = 44), metabolic (n = 7), fulminant hepatic failure (n = 10), redo transplants (n = 3), and other (n = 15). Three combined liver/kidney transplants have been performed. Forty-nine were reduced-size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1, and 29 children weighed Results Fifty-six (74%) patients survived 3 months to 12 years posttransplant. Cumulative 1- and 5-year patient survival data are 79% and 70%, respectively. However, with the introduction of prophylactic intravenous gancyclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the projected 5-year pediatric survival has been >80%. Early (

L'hydatidose pancreatique primitive est rare. Le diagnostic peut etre difficile lorsque l'aspect clinique est celui d'une masse epigastrique inexpliquee en depit des renseignements radiologiques et ultrasoniques suggestifs. Les auteurs decrivent l'observation d'une jeune fille de 12 ans chez qui le diagnostic definitif fut seulement fait lors d'une operation iterative ce qui a permis de faire un traitement chirurgical efficace et approprie

A 10-year-old girl had hemobilia and a biliocutaneous fistula after blunt abdominal trauma. Embolization of the right hepatic artery occluded the hepatic artery aneurysm, but the cutaneous biliary fistula persisted despite prolonged (8 months) conservative management including sphincter decompression with an endoscopically placed biliary stent. Roux-en-Y fistuloenterostomy eventually cured the bile leak.

A total of 124 children aged less than 14 years with a liver abscess were seen in a 16-year period (1974–1990) and treated by non-operative initial management. Of the abscesses 98 occurred in the right liver and 26 in the left. The abscesses were solitary in 93 patients. Overall, 77 of the solitary and 21 of the multiple abscesses were confined to the right liver. In 78 of the right-sided and 20 of the left-sided abscesses the infection was primarily pyogenic in nature with Staphylococcus aureus being the usual organism cultured. The remainder were of amoebic origin. Clinical features were similar in patients with amoebic and pyogenic abscesses. Clinical and ultrasonographic follow-up demonstrated successful non-operative management and healing in 37 per cent of all patients submitted to an initial protocol of medical supportive care and antibiotic therapy. Of the multiple abscesses 60 per cent responded to non-operative management. Fourteen of the 16 solitary left-sided liver abscesses required drainage and three left-sided abscesses ruptured before drainage. Patients with a solitary left-sided abscess warrant early operative intervention.

Liver transplantation for infants and children has been available in South Africa for more than a decade. Current concerns have shifted from an initial target of early post-transplant survival to quality of life in the long term. Since 1985, 175 infants and children have been assessed, with 104 accepted for transplantation. Fifty have had orthotopic liver transplants (OLTx), 48 (3 retransplants) in Cape Town and 2 abroad. Biliary atresia was the most frequent diagnosis (52%) followed by acute liver failure (ALF) (16%). Waiting list mortality has remained high (15%), particularly for the ALF group (50%). Thirty-four patients survive 1 month--14 years post-transplant. Early post OLTx mortality was low (7%) but late morbidity and mortality (23%) were mainly due to viral infection: de novo hepatitis B (5 patients, 2 deaths), EBV-related post-transplantation lymphoproliferative disease (PTLPD) (6 patients, 4 deaths) and CMV disease (9 patients, 4 deaths). Tuberculosis prophylaxis, required in 6 cases, resulted in major morbidity in 1. Hypertension requiring medication along with some compromise of renal function has been present in all but 2 patients. However, all those of school-going age (20) attend school normally and remain in good health and only 3 of the survivors have abnormal liver function tests. Successful liver transplantation is possible in a developing country with limited resources. Scarcity of virus-free donors (HBV and HIV) leading to waiting list mortality and infrequent retransplantation along with long-term consequences of immunosuppression (infection, lymphoma and renal toxicity) remain problems.

An increasing incidence of colorectal carcinoma has been noted at this institution. We report seven children with colorectal carcinoma. The average delay between onset of symptoms and diagnosis was 41/2 months, and in five patients distant metastases were present at the first operation. Initial symptoms were ignored in all cases and in only one was the serious nature of the condition realized at first presentation. In five lesions the histology was mucin secreting adenocarcinoma, a poor prognostic variant. All seven died on average 11 months after diagnosis. These three factors--delay in diagnosis, advanced stage of disease, and poorly differentiated histology--contribute overall to a poor prognosis in the young.

Bleeding from esophageal varices is a common cause of major upper gastrointestinal tract blood loss in children with portal hypertension but usually ceases spontaneously or is satisfactorily managed by nonoperative measures. Massive hemorrhage from gastric fundal varices may be difficult to control with compression and sclerotherapy; in these cases, a direct surgical approach may be indicated. Since 1984, 27 children have undergone aggressive injection sclerotherapy for bleeding esophageal/gastric varices. Nine (6 with portal vein thrombosis) bled from gastric fundal varices. In 5 of these, medical management and sclerotherapy failed to control the acute bleed. In all 5 there was "rupture" of a large gastric fundal varix or "pile" and bleeding was controlled at emergency laparotomy by underrunning the varices through a high anterior gastrotomy. Four have subsequently been successfully managed by continued sclerotherapy and one patient with cirrhosis has died of liver failure. In 3 of the survivors both esophageal and gastric fundal varices have been completely obliterated. No further life-threatening hemorrhage has occurred in any case during a follow-up period of 1 to 5 years. Bleeding from gastric varices is more common than previously recorded and more difficult to control by nonoperative management, including injection sclerotherapy. In uncontrolled hemorrhage from gastric varices, surgical underrunning offers a means of providing initial control. Thereafter, the inevitable variceal recurrence may be successfully treated with sclerotherapy.

In October 1994 we changed our management of neonatal Hirschsprung's disease from a staged procedure after initial colostomy to a neonatal single-stage Soave endorectal pull-through (SERPT) without a colostomy. We report on our experience with the first 12 neonatal SERPTs, comparing them retrospectively with a consecutive series of 12 patients managed with initial colostomy, followed by a SERPT months later. A neonatal SERPT proved to be an easier and quicker procedure than the same procedure in the older child. Patients could be discharged earlier and fewer complications were encountered; in addition a colostomy was avoided. The cosmetic result and parental acceptance were excellent. At the same time the cost was less than half compared with the staged group. At this stage of follow-up functional results are comparable with previous staged pull-through procedures.

The accidental ingestion of corrosive agents is a major cause of oesophageal strictures in children. The mainstay of treatment is repeated dilatations. Despite this, a significant number of patients eventually require oesophageal bypass. We reviewed the records of all cases managed with this condition at the University of Cape Town teaching hospitals between 1976 and 1994. Dilatation therapy alone was successful in 14 out of 39 patients (41%). Morbidity of failed dilatation therapy included repeated hospital admissions over an average 11.5 months and 17 dilatations each. Oesophageal perforations occurred in 7 cases (18%). Early factors predictive of failure of conservative treatment were: delay in presentation of more than 1 month; severe pharyngo-oesophageal burns requiring a tracheostomy; oesophageal perforation; and a stricture longer than 5 cm on radiological assessment. The size of dilators accepted during early bougienage also correlated with eventual outcome. These criteria may be useful in predicting which patients will not respond to repeated dilatations. Early surgical intervention in such cases will prevent fruitless dilatations and related complications.

Controversy persists with regard to the diagnosis and treatment of the acute scrotum in children. The differential diagnosis includes torsion of the testis, torsion of one of the appendices testis and epididymo-orchitis. Clinical differentiation is notoriously difficult and our policy has been to explore the scrotum of all who present with signs of an acute scrotum. The medical records of 199 boys aged less than 13 years who presented with an acute scrotum at Red Cross War Memorial Children's Hospital in Cape Town during the period 1970-1996 were retrospectively reviewed. Diagnosis was made clinically in all cases. Sixty-two boys (31%) were found on exploration to have torsion of the testis (mean age 6.3 years), the left side being affecting 2.5 times more frequently than the right (1 neonate had bilateral torsion), 62 (31%) had torsion of testicular appendages and 56 (28%) had epididymo-orchitis. Nineteen were grouped separately and 13 (6.5%) of these were treated conservatively. Boys needing an orchidectomy (N = 38) for testicular torsion presented three times later than those who did not (48 v. 16.5 hours). Successful conservation of the testis was directly related to the time interval of symptom development and surgical derotation. Boys with torsion of a testicular appendage presented later (mean 51 hours) and were older (mean 10 years) than boys in the other two groups. Those with epididymo-orchitis were younger (mean 3.3 years) and 40% were younger than 1 year. Only in one-third was there any clinical supportive evidence of the diagnosis. Surgical complications were infrequent. We advocate expedient surgical exploration and derotation with contralateral orchidopexy, since clinical differentiation between torsion of the testis, testicular appendages and epididymo-orchitis is inexact, even with highly specialised investigation such as Doppler ultrasound and radionuclide scanning, which may not be widely available, may delay definitive treatment and suggest a lack of urgency where time is of the essence.

Impalpable testes constitute up to 20% of cases of undescended testes and present both a diagnostic and a therapeutic problem. Knowledge of testicular site prior to orchidopexy is useful in planning surgical strategy. Misdirected blind inguinal exploration can be avoided. Many imaging techniques are time-consuming and expensive and can neither confidently locate an impalpable testis nor exclude its absence. Recently laparoscopy has been advocated as a safe and efficient method of identifying presence and site of an impalpable testis. The first stage of a divided-vessel orchidopexy may also be performed simultaneously. Fifty children with 60 impalpable testes--40 unilateral and 10 bilateral--underwent laparoscopic examination prior to groin exploration. In 5 (8%) an inguinal testis was found which had been missed on pre-operative palpation. The testis was absent in 23 (38%), of whom 13 had a limited negative groin exploration because cord structures were seen entering the internal ring. In 30 (50%) an intra-abdominal testis was identified; 14 were relocated into the scrotum with a standard orchidopexy, 16 required a divided-vessel orchidopexy. In 2 (4%) intra-abdominal testicular remnants were removed. There was no operative morbidity. We conclude that laparoscopy is a useful technique in the management of children with impalpable testes. Open perumbilical insertion of the trochar is a safe method of introducing the telescope into the peritoneal cavity. In addition to accurately identifying a testis in 60%, 40% were spared the pain and morbidity of an extensive groin exploration.

Patients with congenital diaphragmatic hernias (CDH) usually present soon after birth with respiratory distress. Occasionally presentation is delayed. Twelve patients with delayed presentation (outside the neonatal period) of a total of 91 with CHD were seen over 17 years (1977-1994). Case records were reviewed to identify reasons for late presentation, evidence of morbidity, treatment and outcome. Age at presentation ranged from 6 weeks to 30 months (mean 8 months). Ten patients were aged 6 months or younger and there was an equal number of boys and girls. Seven cases were left-sided, 4 right-sided and 1 was anteriorly placed and bilateral. Ten patients presented acutely, 5 with small-bowel obstruction and 5 with respiratory distress, but all of the latter had a history of previous recurrent chest infection. Diagnosis was confirmed by radiographic examination of the chest in 10, barium meal in 1 and a 5-month-old patient had an inappropriate laparotomy and gastrojejunostomy for duodenal obstruction at an outlying hospital. Two had documented normal chest radiographs 3 and 6 months prior to diagnosis. Patients who presented with gastro-intestinal symptoms were older (mean age 1 year) compared with those with predominantly respiratory symptoms (mean age 19 weeks). None had any evidence of bowel strangulation. All were repaired via an abdominal approach. A hernial sac was found in half the cases (4/7 left and 2/5 right). Five had non-rotation of bowel. Primary repair without patch was carried out in all. One child with lung hypoplasia and multiple cardiac abnormalities died; the others recovered well. Delay in presentation of CDH is not uncommon (14% in this series). Most patients will have respiratory symptoms. A normal previous chest radiograph does not exclude the diagnosis. All patients with recurrent chest infection should have a contrast meal investigation early on. Long-term prognosis is favourable and postoperative morbidity is minimal, despite late presentation.

Abdominal aortic aneurysms are rare in children. Causes include mycotic aneurysms, vasculitides (eg, Takayasu's arteritis), connective tissue diseases (eg, Marfan's syndrome, Ehlers-Danlos syndrome, and tuberous sclerosis) and traumatic false aneurysms. Four cases are described. Case 1 was a 12-year-old boy who presented with an acute unheralded rupture of the subdiaphragmatic aorta accompanied by lower limb paralysis and ischemia. Attempted repair failed because of extensive friability of the large arteries. Histological evaluation confirmed cystic medial necrosis despite Marfanoid phenotype. Cases 2 and 3 were boys aged 12 and 11 with Takayasu's arteritis who presented with hypertensive encephalopathy and heart failure. Although both had involvement of the origins of the renal arteries, one aneurysm was predominantly suprarenal and the other infrarenal. Currently both children are being managed successfully with antihypertensive therapy. Case 4 was a 5-year-old girl who presented with hypertension and a pulsatile abdominal mass after treatment of infective endocarditis 18 months previously. Arteriography and three-dimensional computed tomography confirmed an aneurysm (6 x 5 x 4 cm) arising from the aorta and involving the right renal artery. Aneurysmectomy, removal of a small ischemic right kidney, and Gore-Tex grafting resulted in cure of the hypertension and uneventful recovery. The present series confirms that rupture is a fatal complication, renovascular complications are common, and medical control of hypertension is an essential part of management. Management strategies need to be highly individualized, and may be successful without surgical intervention. Close clinical and ultrasound follow-up of those managed nonoperatively is essential.

An experimental model in the pig rendered incontinent of feces was developed to assess the sphincteric activity of the transposed antropylorus. In the control group, normal defecation was studied clinically, radiologically, and manometrically. Nineteen 7- to 10-week-old pigs were rendered incontinent by resection of 20 cm of colon and rectum to below the dentate line. The antropylorus was prepared on its own blood supply and transposed to the anus, initially with a colostomy, which was closed 15 to 21 days later. Clinically these pigs passed semisolid stool in a piecemeal fashion. Contrast defecography showed hold-up at the pylorus, reflux of contrast into the colon, with pyloric contraction independent of antral stimulation. Manometry showed pyloric contraction with rise in antral pressure and independence. The authors conclude that transposition of an antropyloric segment to the anus provides a sphincter-like mechanism and could have application in fecal incontinence.

Ambulatory surgery has become an important component in the provision of surgical care. In 1987 a day-case surgical unit was established at Red Cross War Memorial Children's Hospital, Cape Town. Experience over a 6-year period (1987-1992) is reviewed in relation to the nature and total number of surgical procedures, pre-operative preparation, daily utilisation of the facility, postoperative care and complications. During this period 16,538 patients (mean age 3 years) were operated on in the unit. Nine surgical disciplines participated. Pre-operative assessment and preparation reduced the rate of cancellations on the day of surgery to less than 6%, with a 90% effective use of schedules operating time. Scarce nursing resources were also maximally utilised. Most procedures took less than 30 minutes and only 0.8% exceeded 1 hour. The average ward stay was 6 hours. No major complications were encountered and acceptance by patients and parents was excellent. Only 16 children who were not scheduled to do so needed to stay in hospital overnight. Recommendations to establish and improve day-stay surgical services are presented. It is concluded that a practical, efficient service can be established and that the authorities should be encouraged to expand appropriate facilities.

This is the report of a newborn girl who was treated by "trial of life" before surgical repair of her left-sided diaphragmatic hernia. Surgery was performed 60 hours after birth, at which time she was found to have an organo-axial volvulus of the stomach. Her postoperative course was complicated by a jejunal perforation on the fourth postoperative day, necessitating another laparotomy.

Eleven females who had a posterior anal transfer (PAT) for a vestibular anus were reviewed in order to: (1) assess the long-term functional success of this operation clinically; (2) evaluate the anorectal manometry profile; and (3) assess defaecation by video proctography. The cosmetic appearance was excellent in all patients. Seven had a good result, 2 a fair result, and 2 a poor result when assessed clinically (Kelly score) in terms of constipation and soiling. The anorectal manometry profile remained within normal limits except in the 2 with a poor result who had diminished sensory awareness of stool in the rectum. PAT does not appear to damage the muscle-sphincter complex, and the children with a large rectum full of faeces seemed to behave like children with acquired megacolon and constipation. Video proctography showed normal defaecatory patterns in all patients. Patient selection is important for this procedure. When PAT was used as the initial treatment a good result was obtained; when it was used to relieve constipation following previous surgery the result was less satisfactory.

A familial occurrence of Hirschsprung's disease and neuronal intestinal dysplasia (NID) is reported. Familial occurrence of NID in a parent and long-segment Hirschsprung's disease in two children in a family as well as a further report of NID in monozygotic twins is described. The linkage of these neurodevelopmental conditions is explored and a common etiologic mechanism for the two conditions is postulated.

Very narrow oesophageal strictures, although visible on contrast examination, may not be amenable to safe prograde dilatation. A technique used in four recent cases where attempts at prograde dilatation had failed is described. The strictures were easily negotiated from below via a gastrostomy and the distal oesophagus using an Arrow Duoflex guide wire for subsequent string-guided dilatation. Access to the lower oesophagus was easily achieved using a Portex blue-line endotracheal tube. This method appears quick, safe, and reliable.

The controversy over the need to explore the asymptomatic contralateral groin for the existence of a hernia sac in children who undergo inguinal hernia repair is not easy to resolve. To detect the patency of the contralateral processus vaginalis, we used intraoperative nonpuncture laparoscopy. By insufflating carbon dioxide and inserting a laparoscope through the open hernia sac, the opening of the contralateral processus vaginalis was clearly seen. A patent processus vaginalis on the opposite side is considered to be a potential hernia. A total of 74 children, 56 boys and 18 girls, were involved in the study. All of them had inguinal dissection on the symptomatic side and laparoscopy was performed through the opened hernial sac. A contralateral patent processus vaginalis was found in 22 children, 14 boys and 8 girls, by laparoscopy. The results were confirmed by surgical exploration of the opposite groin. There were no false-positive and no false-negative results. Intraoperative non-puncture laparoscopy is a simple, safe, and accurate method of detecting patency of a contralateral processus vaginalis. When used instead of routine bilateral exploration, this procedure can avoid many unnecessary surgical operations.

The syndrome of degenerative leiomyopathy causing intestinal obstruction clinically manifests in young indigenous African children as massive megacolon without aganglionosis. Eighteen children have been seen over a 16 year period from a localized geographic area. There were 10 males and 8 females having a mean age of 9.5 years at presentation and a mean duration of symptoms of 4.3 years. The youngest was 6 months old. All had progressive abdominal distension and infrequent stooling but 11 had intermittent diarrhoea and 9 had colicky abdominal pain. Gross gaseous distension of the large bowel with extension into small intestine occurred in 9 and this extended into stomach and oesophagus in 4. Biopsy of the dilated, thin walled bowel showed smooth muscle degeneration and necrosis with replacement by fibrous tissue. Neuronal cells of Auerbach's plexus tend to be displaced into the circular layer of smooth muscle with mild inflammatory changes. Some small arteries show medial fibrosis with subintimal fibroblastic proliferation. Acetylcholinesterase and immunohistochemical staining with neural and muscle markers are within normal limits. One child died while 14 have been maintained on prokinetic agents, low residue diets, laxatives and enemas. Nine children have required surgical intervention of whom 4 had volvulus and 3 adhesive bowel obstruction. Degenerative leiomyopathy is a distinctive entity with classical clinical and histological features. The aetiology is still obscure.

A survey of the current practice of general paediatric surgery in South Africa and Namibia was conducted by means of a postal questionnaire. One hundred and eighty-one replies were obtained (52%). Analysis showed that although surgeons are located predominantly in the major cities, 18% practised in the larger towns or rural areas. Although paediatric surgery represents a small volume of their work, most surgeons do attend to the surgical needs of children. The majority of surgeons have received some paediatric surgical training but this was considered inadequate by nearly half of all surgeons and by 60% of those who qualified in the last 10 years. Current postgraduate training at most of our universities involves a 3-6-month rotation, but from our survey this is considered insufficient in practice and there appears to have been some deterioration in paediatric surgical training in recent years. Steps needed to maintain paediatric surgical standards are discussed.

The use of concomitant tube cecostomy remains an option for decompression after distal large-bowel surgery but has been criticised because of a reported high complication rate. Two hundred and three patients who underwent a Soave endorectal pull-through procedure for Hirschsprung's disease (1974-1990) were evaluated. Operative technique included a modified sutured Soave endorectal pull-through procedure and a Stamm tube cecostomy utilising a large-lumen catheter. One hundred and twenty patients who had a modified two-stage procedure plus concomitant tube cecostomy were compared with 83 patients who underwent a three-stage procedure with colostomy cover. Early postoperative complications occurred in 8 patients (6.6%) in whom cecostomies were performed. A distal cuff abscess developed in 1 patient (0.8%), an anastomotic leak in 1 (0.8%) and 3 patients (2.5%) had colo-anal stenosis. The cecostomy tube dislodged in 2 patients and 1 required operative closure of a faecal fistula at the cecostomy site. Colonic venting was adequate and little nursing care was required. In 2 instances colonic distension developed after cecostomy clamping. Decompression was achieved by opening the cecostomy tube; this resulted in relief of symptoms and a good subsequent recovery. In contrast, there were 11 postoperative complications in the 83 patients undergoing a three-stage procedure (13.2%). In 2 patients (2.4%) an anastomotic leak occurred and 5 distal cuff abscesses (6%), 3 (3.6%) early strictures and 1 (1.2%) neorectal retraction developed. The use of a concomitant tube cecostomy with a two-stage Soave procedure is an effective and safe means of providing proximal colonic venting and did not add to mortality or morbidity.(ABSTRACT TRUNCATED AT 250 WORDS)

Over a 14-year period 587 children under 13 years of age were admitted with blunt injury to the abdomen. Twenty-nine (4.9 per cent) of these were found to have bowel rupture. Evidence of peritonitis was present at initial evaluation in 11 children (38 per cent). Radiological evidence of perforation (pneumoperitoneum) was present in only five of 27 (19 per cent) with a further six of 27 (22 per cent) showing dilated loops of bowel or fluid levels. Thus 59 per cent of radiographs were not diagnostic. The mean time from admission to laparotomy was 17 h. Proximal bowel perforation was common and perforation at multiple sites occurred in five patients; 59 per cent had a concomitant injury which resulted in two deaths (from head injury). Initial clinical and radiological evidence of bowel perforation can be misleading and reliance on such indicators may result in significant diagnostic delay. Frequently repeated clinical examination is advocated; progression of abdominal signs should alert the clinician to proceed to laparotomy.

The syndrome of degenerative leiomyopathy (DL) causing intestinal obstruction clinically manifests in young indigenous African children as massive megacolon without aganglionosis. We report on a clinico-pathologic study of 18 children seen over a 15-year period. All patients came from the same geographic area. Mean age at presentation was 9.5 years with mean duration of symptoms 4.3 years. The youngest infant was 6 months old. Eighteen children presented with progressive abdominal distention and infrequent stooling; 11 had intermittent diarrhoea and 9 colicky abdominal pain. Gross colonic gaseous distention was noted with proximal extension into the small bowel in 9 and into the stomach and oesophagus in 4. The rectosphincteric reflex was present in all 7 patients studied with anorectal manometry. The diagnosis, confirmed on full-thickness rectal biopsy, showed characteristic features distinguishing it from chronic idiopathic intestinal pseudo-obstruction, progressive systemic sclerosis, and mixed connective-tissue disease of childhood. The bowel was dilated, thin-walled, and on microscopy showed smooth-muscle degeneration and necrosis with replacement fibrosis of muscular layers, displacement of ganglion cells, mild increase in numbers and thickness of nerve fibres, mild inflammatory change, and medial fibrosis of small muscular arteries. Immunohistochemical studies were within normal limits. One child died; 14 have been maintained with a combination of prokinetic agents, low-residue diet, laxatives, and enemas; and 3 patients did not respond to this medical therapy on short-term follow-up. Nine children required surgical intervention — 2 diagnostic laparotomies, 4 volvulus, and 3 adhesive bowel obstructions. Four of these required subsequent surgical procedures. DL is a distinctive entity with a classical clinical and histologic picture whose exact etiology is still obscure, Exploratory laparotomy should be avoided.

Central venous catheters can be of major benefit to children in whom prolonged intravenous access is required. For their everyday use, however, the known associated morbidity needs to be at an acceptably low level. Experience with 44 central venous catheters in 31 children (3318 catheter days over a 40-month period) is reported. The complication rate of catheter sepsis (0.3/100 catheter days), thrombosis (1 catheter), and technical problems (17, of which 6 required removal) justified catheter usage. Provided catheters are correctly inserted and the patients meticulously nursed, these catheters offer major advantages to infants and children requiring long-term intravenous access, particularly for parenteral nutrition.

From 1978 to 1988 228 children under 13 years of age with liver injury following blunt abdominal trauma were studied prospectively. Motor vehicle related accidents were responsible for 85 per cent of the injuries. Isotope liver scan established the diagnosis and identified the pattern of injury. Multiple injuries were seen in 157 patients--predominantly head injuries. The liver was the only intra-abdominal organ injured in 119 patients. There were 69 associated splenic, 33 renal, 21 pancreatic and 3 bowel injuries. There were two deaths (0.88 per cent). Management was non-operative in 215 patients, with resolution of the liver injury in 214. Blood was transfused in 92 patients (40 per cent) (mean volume 20 ml/kg). There were 10 complications; two liver abscesses responded to antibiotics. Follow-up showed complete resolution of the liver injuries. Eight underwent laparotomy because of instability, deterioration or other extrahepatic visceral injuries. All required blood transfusion (mean volume 30 ml/kg). There were eight complications following surgery.

Six children have been admitted over the last 10 years with blunt abdominal trauma causing diaphragmatic rupture. Four were acute presentations, with the emphasis on cardio-respiratory symptoms. There was a high incidence of associated injuries. Early laparotomy and repair of the diaphragm was performed successfully, and three out of four patients survived. Two patients presented late with gastrointestinal tract symptoms. A thoracotomy was necessary to free the adherent herniated bowel in one case. The peripheral nature of the lesion in children suggests that avulsion, aggravated by the increased elasticity and recoil of the thoracic cage, may play a major role in pathogenesis. Diaphragmatic rupture must be excluded in all severe cases of blunt abdominal trauma.

Twenty-eight of 370 patients (14 families) treated for Hirschsprung's disease (HD) over a 34 year period had a family member with histologically proven HD. These 14 represented 4% of the 351 families: more than one affected child per family in 10 (2.8%) and both parent and child in 2 families. Neuronal intestinal dysplasia (NID) in a parent was associated with total colonic aganglionosis in two siblings of one family which suggests a similar genetic inheritance pattern to HD. Aganglionosis extended beyond the rectosigmoid in 61% of the familial group as opposed to 27% of the non-familial group. A significantly higher number of total colonic aganglionosis (TCA) was noted in those with a family history; 11 out of 28 (39%) as opposed to 19 out of 342 (5.6%) without a family history (p less than 0.001). Fifty percent of males with TCA had a family history but in only 2 cases was this transmitted through a female sibling. Although no significant difference was noted between male and female probands, a three times higher incidence of familial occurrence was noted in females with rectosigmoid disease than in males. Progression of length of segment in succeeding generations was noted in two families. Associated anomalies occurred in 16% without familial occurrence and 11% of the familial group.

Over an 11-year period, 333 patients aged 6 months to 13 years were investigated for suspected blunt renal trauma. Ninety-one renal injuries were demonstrated. All patients who had preexisting pathology sustained major (ie, grade III or IV) injuries and all those who required surgery presented with 4+ or macroscopic hematuria with or without loin signs. Intravenous pyelography (IVP) showed no injury in 140 (89%) of 157 patients who had 0 to 3+ microscopic hematuria, and did not influence management in the remaining 17 patients. Seventy-eight (84%) patients were treated nonoperatively, with one death and few complications. Thirteen (14%) patients underwent early laparotomy, with a nephrectomy rate of 92%. No patient with a renal pedicle injury was considered suitable for vascular reconstruction. We conclude that (1) contrast studies are of little value in pediatric patients with asymptomatic microscopic hematuria after blunt trauma; (2) IVP remains the most cost-effective means of investigating renal injuries; and (3) laparotomy is only indicated for ongoing hemorrhage from the severely injured kidney and in a few selected patients with renal pedicle injuries.

During the period 1980–1989, 95 patients, mean age 5 years, with abdominal tuberculosis (ATB) were seen at the Red Cross Children's Hospital. Eighty per cent were malnourished. Apart from fever, loss of weight, and failure to thrive, symptoms of abdominal pain, vomiting, and diarrhoea predominated. Abdominal distension (86%) and a palpable mass (57%) were the most common physical findings. Sixty-three per cent had radiological evidence of chest disease. Abdominal ultrasound was useful in identifying ascites and distribution of lymph node masses. Fifty of the 95 patients were managed on the surgical unit and the predominant involvement was: peritoneal 21, nodal 15, enteric 11, and undetermined 3. Thirteen of this group developed one or more complications — perforation (4), obstruction (7), abscess or fistulae (5), and haemorrhage (1). Surgery involved diagnostic laparotomy, extra-abdominal biopsy, and management of the complications. Emergency surgery was conservative. Definitive surgery for stricture-plasty, resection, and stoma closure was delayed at least 8 weeks to allow for chemotherapeutic effect. Uncomplicated TB responded rapidly to therapy. There were no deaths in this group. Thirty-seven of the 45 “medical” cases made an uncomplicated recovery on anti-TB therapy. Three died due to generalised disease, 5 had complications (chylous ascites 2, protein-losing enteropathy 3) and 3 had relapse of disease due to poor compliance. The morbidity and mortality of this ubiquitous disease can be greatly reduced by timely diagnosis, which often requires early recourse to diagnostic laparotomy, and appropriate surgery and supportive care for complicated disease.

Fifty consecutive paediatric patients with objective clinical signs of blunt abdominal trauma underwent both computed abdominal tomography (CT) and liver-spleen scintigraphy (LSS). All were managed non-operatively with no morbidity or mortality. Five splenic and 13 liver injuries were visualised on CT while LSS revealed 9 splenic and 29 liver injuries. Where injury to either organ was detected by both modalities, there was good correlation anatomically and also as to the extent of injury. LSS demonstrated injuries not shown on CT, which appeared to detect only more extensive injuries. We conclude that scintigraphy may be the more sensitive and therefore more appropriate method when blunt injury to the liver and spleen alone is clinically suspected. Abdominal CT remains the early investigation of choice in patients with clinical evidence of haemoperitoneum associated with shock or multiple injuries, particularly when injury to the kidneys as well as to the liver and spleen is suspected or when a concomitant brain scan is indicated.