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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.

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.

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.

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.

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.

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.

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.

Paediatric trauma care varies in different countries. In South Africa injury is the leading cause of death in the 5-14-year-old age group--1.5-3.8 times higher than in the USA. In 1978 the Child Safety Centre was established and prospectively collected data on paediatric injuries. The various types of injuries are discussed. Trauma is responsible for the highest percentage of years of life lost but the least amount of money is being spent on research and prevention of injuries. The Child Accident Prevention Foundation of Southern Africa has been constituted to research, prevent and reduce the risk factors of the injuries and to improve facilities for the injured child.

This study was undertaken to analyse admission data on all patients seen in the Red Cross War Memorial Children's Hospital Trauma Unit during the period 24 April 1984 - 31 March 1989. Data were retrieved from computerised records completed on admission and were reviewed descriptively. Variations in age, sex and population group pattern for different causes of injury were evaluated together with data on the nature and place of injury and time seen. Data for analysis of nature of injury by cause were also retrieved, but over a 2-year period only (1986-1987). Accuracy of recorded data was assessed from a random sample of hospital records. During the 5-year period 57,468 patients were seen in the Trauma Unit, of whom 17.1% were admitted. In addition 6,377 cases of poisoning and 119 of near-drowning were seen by the medical departments. Forty-three per cent of injuries were due to falls, which were the most important cause of injury in both admitted and non-admitted patients of all age, sex and population groups. Other main cause groups were bumps and blows (15%), transport (11%) and burns (11%). Transport and burn injuries had the highest admission rates. This is the first study of children's injuries in southern Africa and provides information essential to future design of paediatric trauma care systems and accident prevention programmes.

The epidemiological findings in 107 patients admitted to Red Cross War Memorial Children's Hospital, Cape Town, between 1976 and 1987 with a diagnosis of near-drowning are reported. Of these patients, 77% were less than 5 years of age (ratio of boys to girls 2.1:1) and 46% of accidents occurred in swimming pools, 18% in buckets and only 9% in the sea. The high percentage of bucket near-drownings is unique in published medical reports; this appears prevalent in socially disadvantaged communities. Seventy-six per cent of the children were normal on discharge from hospital, 12% died and 6% had neurological damage. In 6% the final outcome was not known. These results are in keeping with other hospital-based studies. Preventive strategies are discussed and the need for further research highlighted.

Polyploid features in some derivatives of Disa hybrids prompted an investigation of their chromosome numbers and those of their hybrid parents, the parental species, and a few close relatives. Disa uniflora Berg. has 2 n = 36; D. cardinalis Linder, D. racemosa L.f., D. tripetaloides (L.f.) N.E. Br. ssp. aurata and D. venosa Sw. have 2 n = 38, whereas D. sagittalis (L.f.) Sw. and D. uncinata H. Bolus have 2 n = 40. D. tripetaloides ssp. tripetaloides from Joubertsgat is a natural triploid with 2 n = 57. Two of the hybrid derivatives, viz. Disa Betty’s Bay ‘Helderberg’ and Disa Diores ‘Colette’ are near tetraploids with 2 n = 68, whereas three others, viz. Disa Foam ‘Jeanette’, Disa Helmut Meyer ‘Pat’ and Disa Unimeyer ‘Apricot’ are near triploids with 2 n = 48, 54 and 52 respectively. The hybrids and especially their robust polyploid derivatives are most attractive and are ideal for the cut-flower industry.

A child is reported in whom severe malabsorptive symptoms were present from a short time after birth together with increasing pseudo-obstructive episodes. Total neurofibromatous involvement of the smallbowel mesentery was found at laparotomy at the age of 4½ years. The child survived a further 2¼ years. The relevant literature is discussed.

Five neonates with uncomplicated meconium ileus were successfully managed by laparotomy and T tube ileostomy. This method seems to offer advantages over other surgical techniques used in the management of uncomplicated meconium ileus that fails to respond to decompression by Gastrografin enema.

Previous erratic use of intermittent intramuscular and intravenous morphine or pethidine for postoperative analgesia prompted a prospective trial of continuous intravenous morphine delivered by an infusion pump. The rate was adjusted to keep the patient free of pain--as assessed by observation in the infant and enquiry in the older child. Serum morphine levels were monitored for the first 36 hours. The results of a cohort of 20 patients (aged 3 months-12 years) are presented. Thanks to positive parental and nursing staff support, and absence of complications, administration of postoperative analgesia with continuous intravenous morphine infusion is now standard practice in this unit.

Eighty-four patients with congenital jejuno-ileal atresias and stenoses admitted over a 20-year period (1959-1978) are reviewed. The overall survival rate of 88% is a reflection of the improvement in diagnosis and supportive care and refinements in technique. Our current ideas on this subject are expressed and a detailed account of the presentation, diagnosis and management is given.

Three sets of thoraco-omphalopagous conjoined twins were investigated. The clinical findings indicated a complex shared heart in two sets, and separate cardiovascular systems in the other. These assessments were confirmed by dynamic imaging after bolus injection of Tc-99m(Sn)colloid or Tc-99m millimicrospheres. Static images were then used to establish the configurations of the shared livers. The results of these studies were in keeping with the angiographic and autopsy findings in the two sets with complex cardiac anomalies and with the surgical findings during successful separation of the third set.

During a 5-year period, 1970 to 1974, a total of 2 118 new patients were admitted to the Burns Unit of the Red Cross War Memorial Children's Hospital in Cape Town. There were 35 deaths. The main complications and causes of death in these 35 patients are critically analysed and standard management methods are reviewed to highlight mistakes in the management of burns.

The presenting symptoms and the clinical findings in 4 children with histologically proven thyroid carcinomas are described. The child with a single nodule in the thyroid gland, the child with a dyshormonogenetic goitre, the child with a thyroglossal duct remnant and the child with thyroid carcinoma and metastatic cervical lymph node involvement are discussed separately.

One-hundred-and-one normal neonates were examined manometrically in the first day of life to assess anorectal function. In order to overcome some of the difficulties in subjective interpretation of results, the stimuli used and the response pattern seen in the anal canal were measured. In the unstimulated state, all subjects demonstrated rhythmical changes in anal canal tone. Mean maximal intraluminal pressure in the anal canal decreased significantly after 10 hours of age and after the first meconium stool. Only five out of the 101 examined on the first day of life had an abnormal manometric response. It is significant that none of these had passed meconium at the time of examination, and that all had a normal response pattern at the age of 28 hours. In 20 of these babies the sensitivity of the rectoanal reflex was measured and found to be significantly increased on the third day of life compared with the first. The quantitative changes in anorectal reflex function in the newborn, are thought to be related to the state of physiological `constipation' which exists in utero and in the early neonatal period. The results indicate that a normal response pattern can be obtained in healthy babies after the first day of life. Consequently, anorectal manometry will be meaningful as a diagnostic method thereafter in the neonatal period. Further, it is suggested that measurement of the stimulus and the response will add useful information and reduce subjective error in interpretation of results.

The surgical results in 39 children operated on for biliary atresia at Red Cross War Memorial Children's Hospital between January 1975 and January 1985 are reported. The mean age at operation was 12.8 weeks (range 6 weeks-6 months). In the first 4 years no patient had sustained bile drainage after operation. Since 1979 16 of 32 patients (50%) have had significant bile drainage. Of these, 8 are alive and have been followed up from 6 months to 6 years after operation. Five are jaundice-free and well. The other 3 are mildly jaundiced, have evidence of ongoing liver damage, and have a poor prognosis. The deaths were due to progressive liver damage with liver failure, portal hypertension, and intercurrent infection. Cholangitis after 'successful' porto-enterostomy was the main cause of poor prognosis and can be related to the age at referral and the presence of cirrhosis of the liver at the time of surgery; thus bile drainage does not necessarily equate with cure. Early referral of infants with conjugated hyperbilirubinaemia to a major centre is of paramount importance if we are to improve the prognosis.

We report the clinical and biochemical course of 2 patients who underwent portacaval shunting for the relief of homozygous type II hyperlipoproteinaemia. In our first patient, the initial favourable clinical and biochemical response subsequently deteriorated and a splenoportogram revealed the presence of a blocked splenic vein with a large collateral blood supply to the liver. The second patient has responded quite dramatically; there has been relief of her preoperative anginal attacks and a significant fall in her plasma cholesterol concentration. The present role of portacaval shunts in the management of this conditions is discussed.

Methicillin-resistant Staphylococcus aureus strains (MRSA) have become increasingly prevalent as nosocomial pathogens, especially in burn wounds. MRSA constituted 38% of all S. aureus isolates in our 25-bed burns unit despite the utilization of a combination of 1% silver sulfadiazine and 0.2% chlorhexidine as topical therapy. Mupirocin, a new antibiotic, has proved in vitro and in vivo to be highly effective in the treatment of MRSA infections. A prospective clinical trial with mupirocin ointment in MRSA burn wound infection was untertaken. Forty-five children with 59 discrete burn wounds and from whom MRSA were isolated were treated with 2% mupirocin ointment under occlusive dressings, applied twice daily for 5 days. The average burned area treated was 8% (range, 2 to 20%) of the total body surface area. The burn wounds were assessed clinically and bacteriologically daily. Mupirocin eliminated MRSA in all 59 wounds treated, with the maximum therapeutic response seen within 4 days. In three wounds, gram-negative organisms persisted after 5 days of topical therapy. Treatment was well tolerated by all children. We recommend that mupirocin in its present polyethylene glycol base should be used only on a selective basis, when current prophylactic topical therapy has failed to control MRSA infection in burns of less than 20% of the total body surface area, and that it should be applied only for a limited period of 5 days. The safety and the efficacy of mupirocin in burns exceeding 20% of the total body surface area need to be established.

In this article the causes of burn injuries in a series of 236 children admitted to the burns unit of Red Cross War Memorial Children's Hospital are investigated. Special reference is made to housing conditions as a contributing factor. The findings suggest that overcrowding and inadequate bathing and cooking facilities are conditions permeating almost all other contributory factors. More pertinent preventative measures are recommended.

Research literature on the psychosocial aspects of cystic fibrosis (CF) is reviewed. The findings indicate that patients with CF and their families are subject to major stresses, yet many manage to function without observable dysfunction. However, indications are that the coping mechanisms of these families need to be enhanced and developed in order to prevent possible dysfunction.

The operative management of oesophageal atresia is reviewed. The operative technique and the approach to management, based on antomical classification of oesophageal atresia and classification into 3 different risk groups, are stressed. The embryology, aetiology, clinical features, symptoms and signs and radiological diagnosis of this condition are also briefly discussed.

Three newborn infants who developed gastric perforation are reported. One infant survived and 2 died. The aetiology of neonatal gastric rupture is discussed, as well as some diagnostic features. The importance of early diagnosis and prompt surgical intervention is stressed.

Takayasu arteritis with renovascular hypertension in an 8-year-old girl is described. At the onset both renal arteries were affected, but 2 years later one renal artery had been recanalized and the uncontrolled hypertension was cured by contralateral nephrectomy.

A spectrophotometric method is described for the quantitative biochemical assay of total cholinesterase in rectal biopsy tissue. Significantly increased enzyme activity was found in this tissue from patients with Hirschsprung's disease, suggesting that the assay has diagnostic value.

Suppurative mesenteric lymphadenitis is so rarely encountered and its symptoms and findings so nonspecific tht it has not yet been diagnosed preoperatively. It seems to occur mainly in children between the ages of 3 and 13 years and presents in a manner suggestive of acute appendicitis. A palpable mass may further support this diagnosis (peri-appendiceal abscess) or encourage the consideration of other diagnoses such as lymphoma or inflammatory bowel disease. Even at laparotomy the diagnosis may not be immediately obvious. The organism is usually a haemolytic streptococcus, but a variety of other organisms have also been isolated. In the second case reported here, a pure growth of Staphylococcus aureus was obtained. Treatment consists of operative drainage with incidental appendicectomy followed by a course of the appropriate antibiotic. The postoperative course is usually uneventful and recovery rapid. The aetiology and mechanisms still have to be explained.

Thirty babies discharged from the maternity home undiagnosed were subsequently found to be suffering from cystic fibrosis (12 cases), Hirschsprung's disease (13) or an anorectal malformation (5). The efforts of parents in this respect and the factors influencing the establishment of a diagnosis were investigated. Maximum periods of 3,9 years, 3,75 years and 5 years respectively were required to confirm a diagnosis in patients with the above three conditions. Although the severity of symptoms is an important factor in speed of diagnosis, other mediating variables had influenced the outcome in our sample; patients referred to a children's hospital were diagnosed sooner. For this reason patients from the lowest social class were diagnosed earlier than those from the higher socio-economic groups. The problems experienced by parents during this phase are reviewed.

The parents of 90 babies diagnosed as suffering from cystic fibrosis (17 cases), Hirschsprung's disease (30), oesophageal atresia (23) and anorectal malformations (20) were interviewed to investigate their reactions at the time of learning the diagnosis. The majority of parents were satisfied with the manner in which the diagnosis had been conveyed to them: the most pervasive factor in this respect was the attitude of the teller. Where this was sympathetic, parents responded positively even when too little information had been given or one parent had been told alone. The largest proportion of dissatisfied parents were from the cystic fibrosis group, in which, in contrast to the surgically correctable anomalies, the element of hope was not present.

Disc batteries are ingested by children with ever-increasing frequency as they become more widely available for a multitude of small electronic devices in the home. Because of their unique properties their ingestion cannot be managed in quite the same expectant way as most other foreign bodies swallowed. Experience with 13 cases is briefly recorded, the literature is reviewed, and based on our findings a plan of management is suggested. The potential hazards of button-battery ingestion should be publicized, and prevention encouraged by parental education, manufacturer's packaging and safe disposal of used batteries.

Twenty-seven patients with gastroschisis were seen at the Red Cross War Memorial Children's Hospital between 1960 and 1977. Twenty-five children were operated on either by primary closure, by skin closure alone, or by the insertion of a reinforced Silastic pouch or patch. The mortality rate has been reduced from 62% to 33% over the past 6 years. Despite better metabolic and respiratory care and intravenous alimentation, serious complications still occur, particularly when prematurity and associated anomalies such as atresia or meconium ileus exist. Other problems were respiratory complications, ileus, perforation, gangrene, intestinal obstruction, enterocolitis and disaccharide intolerance. The long-term follow-up of some of these patients is described.

Deformities can either be congenital in origin or acquired after an accident or particularly after extensive surgery for malignant disease. Many congenital anomalies, especially if associated with Down syndrome, or when major residual handicaps are anticipated, raise the ethical question of whether or not to treat them initially. The effect of major handicaps on the child, family and society are discussed. Certain congenital anomalies can now be prevented by amniocentesis and termination of pregnancy in the high-risk group, and some of the acquired deformities can also be prevented to a certain extent. Lastly, some aspects of the legal situation are mentioned.

Posterolateral diaphragmatic hernias and cystic hamartomatous malformations of the lung can cause actue respiratory distress in the immediate postnatal period. The clinical and radiological features of these two conditions can be similar. Owing to misdiagnosis, the surgical approach was inappropriate in 2 of 26 patients diagnosed as having posterolateral diaphragmatic hernias on admission. During the same period, 5 cases of congenital cystic lung disease were treated surgically. Three of these patients were admitted in severe respiratory failure within 48 hours of birth, and in 2 of these cases the wrong diagnosis was made. The clinical features of these two easily confused congenital anomalies are discussed, and the main differentiating points on the chest radiographs are presented.

Congenital anorectal deformities present a therapeutic challenge to the paediatric surgeon. The anomalies encountered range in complexity from the most simple to the most intricate. It is for this reason that three clear phases in management are defined and applied in every instance, i.e. absolute clarification of the anatomy of the defect, its appropriate operative correction, and, finally, a protracted postoperative period of care during which time voluntary stool control is acquired. Anal continence is the ultimate functional objective. The meticulous treatment required by the unfortunate baby born with this correctable abnormality is emphasized.

Regurgitation of stomach contents is common in babies. One of the causes is gastro-oesophageal reflux due to an incompetent lower oesophageal sphincter, which may or may not be associated with a sliding hiatus hernia. Persistence of this defect will result in a pathological entity leading to reflux oesophagitis. If this symptom complex is not recognized, early disabling complications will result. The purpose of this study is to draw attention to the symptomatology of gastro-oesophageal reflux and hiatus hernia in infancy and childhood so as to improve our understanding of this entity and consequently allow earlier diagnosis, appropriate treatment and prevention of complications.

There is a high incidence of primary colonic intussusceptions in infants and children in Africa. The case histories of 37 patients are reviewed. Of the varieties described, the caecocolic intussusception (16 patients) presents as an intestinal upset, often mild, with symptoms of colic and vomiting. In many of these patients there is known to be an intestinal infestation with Ascaris lumbricoides. This often leads to a delay in establishing the correct diagnosis. Colocolic intussusception (13 patients) gives rise to more acute abdominal symptoms. On clinical assessment, signs of intestinal obstruction are found and there is usually an intra-abdominal mass which can be palpated in the left colon. Further confirmatory evidence of intussusception is the finding of occult blood in stools. There is an unusually high incidence of sigmoid intussusceptions in infants (8 patients). The diagnosis of this form of intussusception is often delayed owing to inadequate clinical assessment of prolapsed bowel at the anal orifice. The length of the prolapsed bowel, the curved nature of the prolapse and the possible demonstration of a sulcus between the prolapsed bowel and the anal canal wall, aid in diagnosis.

An investigation study of 90 cases in which a baby had been born with cystic fibrosis, oesophageal atresia, Hirschsprung's disease or an anorectal malformation was undertaken to establish the duration of the prediagnostic phase and the difficulties experienced by parents before discharge from the maternity home. The congenital anomalies requiring immediate surgical intervention were diagnosed very soon after birth. A wider diversity of problems were encountered in the case of those conditions with a longer prediagnostic phase. The problems encountered by parents, viz. lack of communication with staff, fantasies of gross anomalies with fear for the baby's life, and the lack of viewing and mothering of the baby are discussed, and some long-term effects are highlighted.

During a 2-year period 176 stoma operations were performed at the Red Cross War Memorial Children's Hospital, for various conditions. About 50% were performed on neonates and a further 30% on infants under 1 year of age. The majority of stoma operations in this age group are performed as emergencies and the stomas are temporary. Only 10% are permanent. The specific stomas, the indications for these, some special points in technique, and the complications are considered and discussed. The management of an infant or child with a stoma is reviewed and the value of a stomatherapist in a surgical unit is stressed.