Liver transplantation for children: Red Cross Children's Hospital experience.

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 < 10 kg. 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 (<1 month) post-liver-transplant mortality was low, but included: primary malfunction (n = 1); inferior vena cava thrombosis (n = 1); bleeding esophageal ulcer (n = 1); and sepsis (n = 1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths); Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disease (12 patients, 7 deaths); and cytomegalovirus disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in three patients was complicated by chronic rejection (n = 1) and TB drug-induced subfulminant liver failure (n = 1). CONCLUSIONS: Despite limited resources, a successful pediatric program has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors due to HBV and human immunodeficiency virus (HIV) leads to significant waiting list mortality and infrequent transplantation.

Heterogeneity and low detection rate of RET mutations in Hirschsprung disease.

Mutations in some exons of the RET proto-oncogene were recently observed in Hirschsprung patients. Using DNA polymorphisms and single-strand conformation polymorphism analysis for the whole coding sequence of the RET proto-oncogene, 82 unrelated Hirschsprung patients were screened systematically. A total of 4 complete deletions of RET and 12 point mutations were identified, each present in no more than one patient and distributed along the whole gene. De novo mutations could be documented in 4 patients. Southern blot and fluorescence in situ hybridization analysis carried out in a restricted number of patients did not reveal any deletion of RET. The low efficiency in detecting mutations of RET in Hirschsprung patients (20%) may originate mainly from genetic heterogeneity.

The use of radionuclides in the investigation of conjoined twins.

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.

Massive small bowel resection in a neonate. Four-year follow-up.

All but 17 cm of small intestine was removed in the neonatal period from a child now aged 4 years. Satisfactory growth was achieved by 2 years of age without prolonged intravenous hyper-alimentation. On return to his home environment progress was not maintained, and by 4 years of age, mental and physical retardation were found. Cholestyramine resin was beneficial in reducing stool bulk but had no substantial effect on fat absorption. Compensatory changes in the remaining bowel are recorded.

Malrotation and volvulus in infancy and childhood.

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.

Bactericidal efficacy of 5 per cent povidone iodine cream in Pseudomonas aeruginosa burn wound infection.

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)

Gastrostomy tube stabilizer.

A new method of gastrostomy tube fixation is described using a metal-ring appliance. This maintains the position of the tube, preventing progression of the tube towards the pylorus, and avoids movement, thus diminishing leakage around the tube. The wound and skin are exposed, easily accessible, and avoid the sogginess encountered with the nipple stabilization. The incidence of fungal infections is also reduced.

Inadequate pouch emptying following Martin's pull-through procedure for intestinal aganglionosis.

Diarrhea is an expected problem following Martin's operation. Its cause is obvious. With medical management, the phase of intolerance to enteral feeding can be bridged. Adaptation is usually reached within 3-6 mo of surgery. This is confirmed by our experience with five patients treated in this manner. In two further patients, episodic bouts of diarrhea continued to mar their recovery. It was then appreciated that this was related to pouch content stasis. The nonoperative management of this complication proved unsuccessful. A death at 20 mo post-pull-through due to "enterocolitis" and an operation 13 mo post-pull-through to repair an acute pouch perforation followed. Attention is drawn to this complication. The following points aimed at preventing stasis, which in our series has been the most difficult problem to treat, are made: a low anal anastomosis--0.5 cm above dentate line; limit pouch size; and use normally innervated bowel. With the use of these changes, early postoperative diarrhea may be difficult to control. For this reason it is proposed that parenteral feeding be used routinely in the immediate postoperative period, and, as rapid fluid loss is better tolerated, surgery be deferred until the patient is 8 mo or more of age.

Fecal incontinence: a simple pneumatic device for home biofeedback training.

A simple pneumatic biofeedback device has been designed for patients who have failed to develop social fecal continence following sacroabdominoperineal pull-through operations for anorectal anomalies. The components that make up the device are easily and cheaply available. This device was designed to be used by the patient at home and needs only the assistance of an adult.

The use of a lateral skin-flap perineoplasty in congenital anorectal malformations.

A lateral skin-flap perineoplasty has been used in 15 patients born with an absent anal canal. The aim behind the introduction of this modification was the prevention of mucosal prolapse. On early assessment, this has been achieved in 11 of the cases. This represents a 50% improvement on our previous results.

Potential danger of 'trial of life' approach to congenital diaphragmatic hernia.

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.

The flaccid trachea and tracheoesophageal congenital anomalies.

The patient with an esophageal atresia associated with a distal tracheoesophageal fistula, is often found to have an abnormally soft trachea. The use of contrast material as an aid in the diagnosis of this congenital anomaly demonstrates radiologically the compressive effect that a dilated and hypertrophied proximal esophageal pouch has on the trachea of the newborn. It is postulated that this chronic compressive force plays a part in retarding the development of the trachea during fetal life. The important decompressive effect of a distal tracheoesophageal fistula allowing nonphysiologic escape of lung fluid, supplements this effect. Attention is drawn to the fact that tracheal compression plays an important part in the respiratory distress seen in these patients, both before and after surgical correction of the tracheoesophageal anomaly.

Yersinia pseudotuberculosis affecting the appendix.

Yersinia pseudotuberculosis is an uncommon cause of abdominal pain. It has a much lower incidence than Yersinia enterocolitica, and most reports have emanated from Europe or North America. This report is about a patient with Yersiniosis affecting the appendix alone, in contrast to the usual picture of mesenteric adenitis or septicemia associated with this organism.

Blunt liver trauma in children: nonoperative management.

Since 1978, we have treated 19 of 23 (83%) children with blunt liver trauma nonoperatively. Management consisted of observation in an intensive care unit, repeated physical examination, frequent reevaluation of laboratory values, special investigations, and bed rest. The 19 patients all remained stable, required no surgical intervention, and showed resolution of the hepatic injuries with no early or delayed complications. Ultrasonography, although not as reliable a method as computed tomography or liver isotope scans for identification of hepatic trauma at first presentation, provided a very useful method for documenting subsequent progress and eventual healing of the lesions. The presence of an isolated hepatic injury is insufficient indication for surgery. If there is significant extrahepatic injury requiring surgery, or if the patient with hepatic trauma is deteriorating, operative intervention is mandatory.

Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease.

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.

Transposition of the antropylorus for anal incontinence--an experimental model in the pig.

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.

Investigation and management of blunt renal injuries in children: a review of 11 years' experience.

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.

Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung's patient.

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 balloon-series techniques. Rectal suction biopsies were performed for patients who had persistent problems with stool evaluation. 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 persisting 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, ganglioneuromatosis 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.

Serum and erythrocyte acetylcholinesterase activity in Hirschsprung's disease.

Acetylcholinesterase activity was measured in the serum and erythrocytes from 22 children. The 12 patients in whom the diagnosis of Hirschsprung's disease was confirmed, had a significantly higher concentration of enzyme in both serum and erythrocytes than the 10 children in whom the diagnosis was excluded. This systemic manifestation, may prove of value in the diagnosis of Hirschsprung's disease.

Portal vein obstruction by ectopic liver tissue.

The causes of portal vein obstruction in children with extrahepatic portal hypertension are variable. We report on a case of portal vein obstruction by ectopic liver tissue in an 8-year-old boy. The ectopic liver had a normal architectural pattern. The clinical management and radiology are reviewed.