Umbilical polyp in infants and children.

AIM OF THE STUDY: The umbilical polyp is a rare congenital lesion resulting from the persistence of omphalomesenteric duct (OMD) enteric mucosa at the umbilicus. Exploration of the abdomen to exclude the presence of associated OMD remnants is controversial. The aim of this study was to evaluate the need for peritoneal cavity exploration in children with umbilical polyp. METHODS: All umbilical lesions (n = 53) excised between 1995 and 2005 in a single institution were reviewed to identify patients with umbilical polyp (n = 13). This is characterised histologically by the presence of gastrointestinal mucosa. A follow-up study of patients with umbilical polyp was performed. Data are reported as median (range). RESULTS AND CONCLUSIONS: All 13 patients underwent excision of an umbilical polyp at a median age of 15.1 months (3.1 - 80.5). All presented with a discharging polyp (associated with bleeding in 9) which did not respond to topical silver nitrate. Median diameter of the lesions was 0.5 cm (0.2 - 1). Histology revealed the presence of small bowel mucosa in 11 (associated with pancreatic tissue in 1 and gastric mucosa in 1) and large bowel mucosa in 2. All patients underwent inspection and probing of the base of the polyp after its excision. In 6 patients an associated OMD anomaly was suspected and exploration of the peritoneal cavity was performed (mini-laparotomy in 5 and laparoscopy in 1). No OMD anomaly was found. The 7 children who did not undergo exploration of the abdominal cavity remain asymptomatic after 5.8 years (0.9 - 13.7) follow-up. An umbilical polyp can be present in the absence of other OMD anomalies. Exploration of the peritoneal cavity in children with an umbilical polyp does not seem to be necessary.

Laparoscopic surgery in children is associated with an intraoperative hypermetabolic response.

BACKGROUND: Laparoscopic surgery is thought to be associated with a reduced metabolic response compared to open surgery. Oxygen consumption (VO2) and energy metabolism during laparoscopic surgery have not been characterized in children. METHODS: We measured respiratory gas exchange intraoperatively in children undergoing 19 open and 20 laparoscopic procedures. Premature infants and patients with metabolic, renal, and cardiac abnormalities were excluded. Anesthesia was standardized. Unheated carbon dioxide was used for insufflation. VO2 was measured by indirect calorimetry. Core temperature was measured using an esophageal temperature probe. RESULTS: We found a steady increase in VO2 during laparoscopy. The increase in VO2 was more marked in younger children and was associated with a significant rise in core temperature. Open surgery was not associated with significant changes in core temperature or VO2. CONCLUSIONS: Laparoscopy in children is associated with an intraoperative hypermetabolic response characterized by increased oxygen consumption and core temperature. These changes are more marked in younger children.

Resection and primary anastomosis is a valid surgical option for infants with necrotizing enterocolitis who weigh less than 1000 g.

HYPOTHESIS: Primary anastomosis following intestinal resection is a valid surgical option in the treatment of infants with necrotizing enterocolitis (NEC) who weigh less than 1000 g. DESIGN: Retrospective case series. SETTING: Tertiary neonatal surgery referral center. PATIENTS: All infants with confirmed NEC weighing less than 1000 g admitted to our intensive care unit over 4 years. INTERVENTION: For infants requiring laparotomy and intestinal resection, primary anastomosis was performed whenever the clinical condition permitted. MAIN OUTCOME MEASURES: Short- and long-term survival, length of intensive care unit stay, and complications. RESULTS: Fifty-one infants with NEC who weighed less than 1000 g were admitted during the study period. Twelve infants underwent intestinal resection and primary anastomosis (median weight at surgery, 0.83 kg; range, 0.6-0.96 kg). One infant developed recurrent NEC, requiring further surgery, but there were no anastomotic leakages and no strictures. The median postoperative stay on our intensive care unit was 14 days (range, 2-49 days). All 12 infants survived their episode of acute NEC, and 8 are alive, with a median follow-up of 34.2 months (range, 4.7-48.4 months). Only 1 death was related to NEC. During the same period, 14 infants underwent intestinal resection and stoma formation. Ten survived the acute episode, and 6 are alive at a median follow-up of 24.0 months (range, 13.1-33.9 months). The median postoperative intensive care unit stay was 18 days (range, 2-74 days). Necrotizing enterocolitis-related complications occurred in 8 of these infants. CONCLUSIONS: The outcome of infants with NEC who weigh less than 1000 g and undergo primary anastomosis is comparable to that of infants treated using alternative techniques. It is, therefore, a valid surgical option in selected infants. The mortality in this group of infants is high.

Disruption of local retinoid-mediated gene expression accompanies abnormal development in the mammalian olfactory pathway.

We have evaluated the role of retinoid signaling in the early development of the olfactory epithelium and olfactory bulb. When retinoid-mediated gene expression is blocked briefly in mouse embryos at midgestation with citral (a general alcohol dehydrogenase antagonist that is thought to interfere with retinoid synthesis), the spectrum of morphogenetic abnormalities includes disruption of olfactory pathway development. It is difficult, however, to assess the specificity of this pharmacological manipulation, insofar as it also compromises several other aspects of central nervous system development. In homozygous Pax6 mutant mice (small eye: Pax6(Sey-Neu)), there is a more discrete lesion to the olfactory pathway: The epithelium and bulb cannot be recognized at any time during development, whereas other forebrain subdivisions can still be recognized. This loss of the entire primary olfactory pathway is accompanied by a failure of retinoid-mediated gene expression limited to the frontonasal region and forebrain. Retinoid receptors are expressed in the forebrain of Pax6(Sey-Neu)/Pax6(Sey-Neu) embryos, and the mutant forebrain remains responsive to exogenous retinoic acid. However, in Pax6(Sey-Neu)/ Pax6(Sey-Neu) embryos, retinoic acid (RA) is not produced by the frontonasal mesenchyme, which normally provides local retinoid signals to the placode and forebrain. Together, these results suggest that local retinoid signaling is essential for the normal development of the mammalian olfactory pathway.

Operative trauma to the genitourinary tract in the treatment of anorectal malformations: 15 years' experience.

OBJECTIVES: To determine the incidence of urologic trauma during surgical correction of anorectal malformations (ARM). METHODS: The records of 228 children with ARM were retrospectively reviewed. RESULTS: Operative trauma to the genitourinary tract was seen in 12 patients, all male. Of 133 patients with a high ARM, 92 underwent an abdominoperineal pull-through and 41 underwent a posterior sagittal anorectoplasty (PSARP); the incidence of urologic trauma was 12% and 0%, respectively. In the subgroup of male infants with a high ARM (99 patients), the incidence of genitourinary trauma was 11%; in those with a low anomaly, the incidence was 1 of 43 (2%). There were 3 urethral tears, 4 urethral strictures, 5 urethral diverticula, 2 vas deferens injuries, and 1 ureteric injury. Nine of the 12 patients do not have any long-term sequelae resulting from the injuries; however, 2 are now infertile and 1 has urinary incontinence. Four patients are additionally compromised because of an absent or nonfunctioning kidney on one side. CONCLUSIONS: In children with ARM, those undergoing a conventional pull-through repair are more likely to have a genitourinary injury than those undergoing the PSARP, and boys with high ARM are at greatest risk.

Associated urologic anomalies in low imperforate anus are capable of causing significant morbidity: a 15-year experience.

OBJECTIVES: To determine the incidence and morbidity caused by urologic anomalies in patients with low imperforate anus. METHODS: The case records and imaging studies of 95 patients who underwent surgery for low imperforate anus over a 15-year period were retrospectively reviewed. Follow-up ranged from 2 to 18 years (mean 5.4). RESULTS: Genitourinary abnormalities were seen in 29 patients (31%), whereas 10 (11%) had lumbosacral abnormalities. A micturating cystourethrogram was performed in 48 patients, 18 of whom were shown to have vesicoureteric reflux and 2 of whom required surgical correction. Three additional patients required bilateral ureteric reimplantation: 2 had megaureters whereas 1 had bilateral ectopic ureters. Four patients had evidence of chronic renal failure; these included 1 patient with reflux nephropathy, 2 with bilateral dysplastic kidneys, and 1 with neurogenic bladder. The patient with reflux nephropathy underwent renal transplantation at the age of 18 years. Two patients had proximal hypospadias and 4 had undescended testes. A neurogenic bladder was documented in 5 children, 4 of whom had lumbosacral abnormalities. One of these had a spinal lipoma and required excision of the lipoma and untethering of the spinal cord. CONCLUSIONS: We conclude that 30% of patients with low imperforate anus would have associated urologic anomalies, several of which are capable of causing significant morbidity. Thus, all patients with imperforate anus, whether high or low, need to be screened for associated urologic anomalies.

Neonatal thymic haemorrhage.

Thymic haemorrhage in a neonate is an exceedingly rare condition. A case is presented of a neonate presenting at birth with cyanosis, respiratory distress and an anterior mediastinal mass. The radiological findings are demonstrated. After surgical excision and histopathological examination the lesion was found to be spontaneous haemorrhage into normal thymic tissue. Spontaneous thymic haemorrhage should be considered in any neonate developing acute respiratory distress with widening of the mediastinum and pleural effusion on chest radiography. Ultrasound may be used to support the diagnosis.

Mediastinal teratomas: review of 15 pediatric cases.

One hundred fifty-three children with a teratoma presented to one hospital between 1970 and March 1992. The clinical and pathological features of 15 patients with mediastinal teratomas are reviewed; six were newborn and nine aged from infancy to 13 years. Thirteen patients including the six newborns presented with respiratory distress and all 15 patients had a mass on chest radiograph. A definite diagnosis of teratoma was not made preoperatively in any of these patients. At operation, a median sternotomy was used to approach seven anterior tumors and a lateral thoracotomy performed in the other eight patients. Histologically two were mature, 10 had immature elements, and three were malignant teratomas. The patients with malignant tumors were all over 12 years of age and died within 6 months of treatment. All six neonates had immature teratomas. Raised serum alpha-fetoprotein levels provided useful markers in two patients with recurrent tumors. Three conclusions can be drawn: (1) mediastinal teratomas are rare in children and frequently are not diagnosed before operation; (2) in newborns these tumors may be immature and present with respiratory distress; and (3) a median sternotomy gives excellent exposure for anterior mediastinal tumors.

Problems and advantages of acetylcholinesterase histochemistry of rectal suction biopsies in the diagnosis of Hirschsprung's disease.

One hundred fifty-seven children, aged 2 days to 15 years, had rectal biopsies for suspected Hirschsprung's disease from 1980 to 1987. Sixty had adequate suction rectal biopsies stained for acetylcholinesterase (ACE); 41 of these were reported as normal and eight showed atypical innervation in children who progressed satisfactorily without surgery. Two showed hyperganglionosis and nine were from children with Hirschsprung's disease. Four of the latter nine biopsies (44%) gave false-negative results in infants aged 9, 10, 13 days, and 6 weeks. The last had total colonic aganglionosis. There were two false-positive results, one in a normal child with atypical innervation and the other in a child with hyperganglionosis. Thus, six of 60 (10%) of ACE-stained suction rectal biopsies gave misleading results.

Oesophageal atresia: at-risk groups for the 1990s.

The authors analyzed the outcome for 357 infants with oesophageal atresia and 15 with H-type tracheoesophageal fistula treated from 1980 through 1992. Survival according to Waterston risk categories was 99% for group A, 95% for group B, and 71% for group C. Presently, with optimal management, virtually all infants in groups A and B should survive. When examining the risk factors in the infants who died, two criteria were found to be important predictors of outcome: birth weight of less than 1,500 g and the presence of major congenital cardiac disease. A new classification for predicting outcome in oesophageal atresia is proposed: group I: birth weight > or = 1,500 g, without major cardiac disease, survival 97% (283 of 293); group II: birth weight < 1,500 g, or major cardiac disease, survival 59% (41 of 70); and group III: birth weight < 1,500 g, and major cardiac disease, survival 22% (2 of 9).

Glutamine and glutathione counteract the inhibitory effects of mediators of sepsis in neonatal hepatocytes.

BACKGROUND/PURPOSE: Surgical neonates are at risk of sepsis-associated liver dysfunction. Hydrogen peroxide (H(2)O(2)) and nitric oxide (NO) are important mediators of sepsis, which impair neonatal hepatic metabolism. Glutamine has been shown to have beneficial effects on hepatocyte metabolism during neonatal sepsis. However, the molecular basis of these effects are unknown. The aim of this study was to test the hypotheses that (1) glutamine and its dipeptides counteract the inhibitory effect of septic mediators on neonatal hepatocyte oxygen consumption and (2) the effects of glutamine are specific and not shared by other amino acids. In addition, we wished to determine the metabolic pathways and mediators involved in the action of glutamine. METHODS: Hepatocytes were isolated from suckling rats, and O(2) consumption measured polarographically. Study A: the ability of 10 mmol/L glutamine to reverse the inhibitory effects of 1.5 mmol/L H(2)O(2) and 300 micromol/L S-Nitroso-N-acetylpenicillamine (SNAP; a nitric oxide donor) on O(2) consumption was examined. Study B: the ability of other amino acids and dipeptides of glutamine to reverse the effects of H(2)O(2) was examined. Study C: various concentrations of glutamine were tested for their ability to reverse the H(2)O(2) inhibition of O(2) consumption. Study D: the mechanism of action of glutamine was examined by incubating hepatocytes with either an inhibitor of entry into the Krebs cycle or an inhibitor of glutathione synthesis. Study E: the ability of glutathione to reverse the inhibitory effects of H(2)O(2) was examined. RESULTS: Study A: glutamine reversed the inhibition of hepatocyte O(2) consumption exerted by either H(2)O(2) or NO. Study B: glutamine dipeptides reversed the inhibition of hepatocyte O(2) consumption by H(2)O(2), but other amino acids did not. Study C: the counteracting effect of glutamine was proportional to the dose administered. Study D: blocking entry of glutamine into the Krebs cycle did not abolish the effects of glutamine, but blocking glutathione synthesis completely abolished the effect of glutamine. Study E: exogenous glutathione reversed the inhibitory effect of H(2)O(2) on hepatocyte O(2) consumption. CONCLUSIONS: This study found that glutamine and its dipeptides are unique in reversing the effects of septic mediators on neonatal rat liver oxidative metabolism. The effectiveness of glutamine appears to be mediated via glutathione synthesis. Addition of glutamine, glutamine dipeptides, or glutathione to total parenteral nutrition (TPN) may be beneficial in preventing liver damage in neonatal sepsis.

Double duodenal atresia/stenosis: a report of four cases.

Four neonates with double duodenal atresia/stenosis are described. Preoperative plain radiographs in two patients demonstrated atypical appearances suggestive of complex pathology. Cystic dilatation of the second part of the duodenum was observed at laparotomy in two patients and in one of these the "cyst" was palpable preoperatively, causing diagnostic confusion. All four patients underwent successful surgery but one infant with Down's syndrome subsequently died of congenital heart disease.

Colostomy for anorectal anomalies: high incidence of complications.

PURPOSE: The aim of this study was to characterize the type and incidence of complications related to colostomy formation in newborn infants with anorectal anomalies. METHODS: The authors reviewed a 5-year (1994 to 1999) experience of a single institution in the management of neonates with high and intermediate anorectal anomalies who required colostomy at birth. Patients with colostomy still in place have been excluded from the study to maximize the chances of detecting colostomy-related complications. RESULTS: There were 80 neonates with anorectal malformations, of whom, 49 (31 boys and 18 girls) were included in the study. The site of colostomy was sigmoid colon (n = 32), transverse colon (n = 7), and descending colon (n = 10). Thirty-nine colostomies were loop, and the remaining 7 were divided. The median birth weight was 2.96 kg (range, 1.46 to 3.88). The age at colostomy formation was 2 days (range, 1 to 210). Mechanical complications related to colostomy formation were observed in 16 infants (32%) with 3 infants having more than 1 mechanical complication. These included prolapse in 8 (50%), intestinal obstruction (adhesions, intussusception, and volvulus) in 7 (44%), and skin dehiscence in 3 (19%). One neonate had necrotizing enterocolitis (NEC) after colostomy formation. Urinary tract infection was observed after colostomy in 14 infants (29%). The incidence of urinary tract infection was not higher in infants who had loop colostomy (11 of 39, 28%) compared with infants who had divided colostomy (3 of 10, 30%). There were no differences in the incidence of colostomy-related complications and urinary tract infection between male and female infants. There were no deaths in this series. CONCLUSIONS: Formation of colostomy for anorectal anomalies should not be considered a minor procedure. In our experience the incidence of complications after colostomy formation is high. The incidence of urinary tract infections does not seem to be affected by the type of colostomy performed.

The clinical significance of thrombocytopenia in neonates with necrotizing enterocolitis.

PURPOSE: The aim of this study was to evaluate the clinical significance of low platelet count in the surgical management of neonates with necrotizing enterocolitis (NEC). METHODS: The clinical course of 58 consecutive neonates with advanced NEC (Bell's stages II or III) treated in the authors' hospital between 1995 and 1998 was reviewed. NEC in neonates who required operation was classified as isolated, multifocal, or pan-intestinal. Severe thrombocytopenia was defined as platelet count less than 100 x 10(9)/L. Rapid fall in platelet count was defined as fall greater than 150 x 10(9)/L within 24 hours to a level less than 100 x 10(9)/L. RESULTS: Median birth weight was 1,564 g (range, 550 to 4,270) and gestational age was 31 weeks (range 23 to 41). Twenty-two neonates (38%) were below 1,000 g. Age at the onset of the disease was 13 days (range, 1 to 62). NEC was treated medically in 7 neonates (12%). Indications for operation included pneumoperitoneum in 23 neonates (45%), clinical deterioration in 19 (37%), and intestinal obstruction in 9 (18%). The nadir platelet count (lowest level during the course of disease) was lower in patients with stage III disease than in patients with stage II disease (P <.05). The greater the extent of the disease, the lower the platelet count (P =.012). The nadir platelet count was lower in infants who died than in survivors (P <.05). None of the patients with platelet count greater than 100 x 10(9)/L died. In predicting intestinal gangrene, severe thrombocytopenia has a sensitivity of 69%, specificity of 60%, and positive predictive value of 89%; rapid fall in platelet count has a sensitivity of 32%, specificity of 89%, and positive predictive value of 92%. CONCLUSIONS: (1) A platelet count less than 100 x 10(9)/L or a rapid fall in platelet count represent poor prognostic factors. (2) Monitoring the platelet count during the course of NEC is useful; however, it cannot be used in isolation to predict the extent of the disease or survival rate.

Pelvic neuroblastoma: low mortality and high morbidity.

BACKGROUND/PURPOSE: Cervical, thoracic, and pelvic neuroblastomas are regarded as having a better outcome than abdominal primaries. The aim of the study was to analyze the results of treatment of pelvic neuroblastomas in our institution. METHODS: The authors reviewed the records of 284 patients with neuroblastoma treated in our hospital during the period 1983 through 1998 and identified 17 (6%) with pelvic tumors. The revised International Neuroblastoma Staging System was used. RESULTS: There were 6 patients with stage 1 disease, 8 with stage 2, 2 with stage 3, and 1 with stage 4 disease. Intraspinal extension of the tumor was present in 7 patients (41%). Except for one child with stage 4 disease, all patients underwent an attempt of tumor excision, and 6 had a complete resection. All 7 patients with intraspinal tumor survived. Permanent postoperative neurological complications occurred in 6 patients (35%). These included sciatic nerve palsy, urinary and fecal incontinence, neuropathic bladder, and leg weakness or nerve root injury L4-S1. Three of 17 patients died, but 1 fatality was unrelated to the tumor. The overall survival rate was 82% and was not influenced by the completeness of tumor resection. CONCLUSIONS: The survival of nonmetastatic pelvic neuroblastoma in our institution is good despite incomplete tumor resection. Intraspinal extension is not a negative prognostic factor. Considering the high incidence of permanent neurological damage after surgery and the generally favorable biological characteristics of these tumors, surgical treatment should not be overaggressive.

The management of oesophageal atresia in neonates with right-sided aortic arch.

BACKGROUND/PURPOSE: The management of oesophageal atresia (OA) in association with a right-sided aortic arch (RAA) is challenging. It is controversial whether right or left thoracotomy is the most appropriate surgical approach. The aim of this study was to determine the prevalence of RAA in patients with OA and the most appropriate surgical approach. METHODS: The case records of all the neonates with OA treated over an 18-year period (1980 through 1997) were reviewed. Patients with RAA were analysed with particular reference to the preoperative investigations, operative approach and findings, and postoperative complications. RESULTS: Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of these infants had major cardiac anomalies. The diagnosis was not suspected on preoperative chest x-ray in any of these infants. In only 1 of the 5 neonates who had a preoperative echocardiogram was RAA suspected. A right thoracic approach was performed in the 11 infants in whom a RAA was not suspected. Immediate conversion to a left thoracotomy with primary anastomosis was carried out in 2 infants. In 2 further infants, fistula ligation alone was performed because of a long gap atresia. Anastomosis via the right thoracotomy was performed in 7 infants. There were 3 anastomotic leaks, 1 intractable stricture, and 1 recurrent fistula. Three patients (25%) had a double aortic arch, and, as a result, a left thoracotomy had to be abandoned in 2. There were 3 deaths, 2 as a result of major cardiac anomalies and 1 related to prematurity and sepsis. CONCLUSIONS: RAA occurs in 2.5% of infants with OA. Preoperative chest x-ray and echocardiogram are unreliable as diagnostic modalities. Anastomosis via a right thoracotomy is associated with a 42% leak rate. The presence of a double aortic arch can make the oesophageal anastomosis via a left thoracotomy as difficult as via a right thoracotomy.

Late death after extracorporeal membrane oxygenation for congenital diaphragmatic hernia.

PURPOSE: The aim of this study was to determine the long-term outcome of neonates who required extracorporeal membrane oxygenation (ECMO) for respiratory insufficiency associated with congenital diaphragmatic hernia (CDH). METHODS: All neonates born with CDH, who required ECMO (oxygenation index over 40 in the presence of maximal support) during a 6-year period, were reviewed. The median duration of follow-up for survivors was 14 months (range, 9 to 22 months). RESULTS: Of 72 neonates with CDH, 11 (15%) required ECMO. The median age at the start of ECMO was 2 days (range, 1 to 4); duration was 9 days (range, 4 to 17); post-ECMO ventilation was 27 days (range, 8 to 86); and stay in intensive care unit, 33 days (range, 8 to 133). Three patients survived and are not oxygen dependent. There were three early deaths at 9 to 16 days and five late deaths at a median of 6 months (range, 2 to 32 months) secondary to chronic lung disease (n = 3) and infection (n = 2). Four of the five patients with late deaths were oxygen dependent at discharge from hospital. Three of the late death patients and one survivor underwent a fundoplication for gastrooesophageal reflux. CONCLUSIONS: Neonates born with CDH and requiring ECMO had a significant late mortality. Follow-up for more than 1 year is indicated to assess the benefit of ECMO for this diagnosis. The authors report only three long-term survivors from 11 ECMO patients selected from a total of 72 neonates with CDH.

Surveillance of Barrett's esophagus in children.

PURPOSE: The aim of this study was to review the need for regular endoscopic biopsy of Barrett's esophagus in children. METHODS: This was a retrospective case-notes review of 38 children with Barrett's esophagus treated between January 1982 and August 1997. The mean age at diagnosis was 6.3 years (range, 1 to 15 years). All had gastroesophageal reflux at diagnosis. Two patients were treated medically, and 36 underwent antireflux surgery (32 Nissen fundoplication, four Thal procedures). At follow-up, for a mean of 43 months (range, 0 to 13 years), 25 underwent repeated endoscopy and biopsies, two underwent further surgery and biopsies, and four underwent endoscopy only. Seven have had follow-up at their referring hospital. RESULTS: In the 27 patients who underwent rebiopsy, there was continued evidence of Barrett's esophagus in 15. There was reversion to normal tissue in 10 patients, and mild esophagitis was present in two. There was no evidence of any dysplastic or malignant change in any patient. All cases that reverted to normal esophagus or mild esophagitis had previously undergone a Nissen fundoplication. CONCLUSIONS: Dysplastic degeneration and malignant change did not occur in any of the authors' patients. In addition, 12 patients with Barrett's esophagus reverted to normal. The authors therefore question whether regular endoscopic surveillance is necessary in children under 16 years of age.

Management and long-term follow-up of infants with total colonic aganglionosis.

BACKGROUND/PURPOSE: Although the survival rate for the infants with total colonic aganglionosis (TCA) has improved significantly, problems with the surgical management continue and the long-term consequences for growth and continence are poorly documented. The aim of this study was to review the experience in the management of 48 patients over a 17-year period (1980 to 1996). METHODS: The medical records of all patients were analyzed with particular emphasis on the number and type of surgical procedures, the attainment of anorectal continence, number of stools per day, and physical development. RESULTS: There were 30 boys and 18 girls. Three (6%) patients died: one of sepsis, one of associated major congenital cardiac anomaly, and one of Moebius syndrome and brain stem dysfunction. Forty-one patients (85%) went on to undergo a pull-through procedure: 38 Duhamel with 13 having Martin modification and three with Soave procedure. Inappropriate surgery (47 procedures) were carried out in 19 (40%) patients before the definitive diagnosis; only four of these infants were admitted primarily to our unit. Thirteen patients had a stoma in aganglionic intestine. An ileostomy was closed in six infants before the diagnosis was established, and six had a previous "negative" laparotomy. Long-term follow-up was possible in 27 patients, of whom 19 (70%) required a total of 39 additional procedures. Ten patients underwent a total of 16 anal dilatations and six underwent sphincterotomy. A permanent stoma was necessary in six patients (two with Down's syndrome). Two patients with Martin modification required resection of the side-to-side anastomosis for intractable diarrhea. The number of the stools per day decreased yearly. Fecal incontinence was common at the 5-year follow up (82%). However, at 10 and 15 years, the rate of incontinence decreased to 57% and 33%, respectively. The proportion of patients below the second percentile for body weight were 25% at 5 years, 20% at 10 years, and 63% at 15 years of age. The equivalent statistics for body height were 15%, 0%, and 23%, respectively. CONCLUSIONS: Patients with TCA tend to undergo multiple procedures. Anorectal function improves gradually over time. There are no advantages of the Martin modification. In the long-term follow-up over half of the patients are below the second percentile for weight and one quarter are below the second percentile for height. Careful long-term follow-up is necessary for the patients with TCA.

Recurrent necrotizing enterocolitis.

In the decade 1981 to 1991, 16 infants developed recurrent necrotizing enterocolitis (NEC). They comprised 12 (6%) of 196 neonates referred for further management of NEC and four others referred with major congenital anomalies. Their median gestational age was 32 weeks (range, 27 to 40), median birth weight was 1,260 g (range, 790 to 3,230), and the sex distribution was equal. Recurrent NEC occurred after a median interval of 37 days (range, 11 to 163) from the onset of the initial episode. All but one of the 16 infants were either premature (n = 10) or mature with major congenital anomalies (n = 5). Nine patients had previously undergone surgery for NEC. Medical treatment was successful in 11 patients with recurrent NEC. Two infants died, both of liver failure related to parenteral nutrition; one of whom had suffered four separate episodes of NEC and was found to have a superior mesenteric artery occlusion. There was no consistent association between recurrent NEC and the type or timing of enteral feeds or the anatomical site or method of management of the original attack. The mortality of 12.5% was similar to that for primary NEC.