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Background: To review the outcome after restorative proctocolectomy among children and adolescents with ulcerative colitis at a pediatric inflammatory bowel disease center. Methods: The records of all patients with ulcerative colitis undergoing colectomy and ileoanal anastomosis at The Hospital for Sick Children, Toronto, Canada, were reviewed. Questionnaires concerning functional results were sent to patients with restored transanal defecation. Results: Seventy three patients (mean age, 13.2 years; range, 2.6-18.8 years) underwent ileoanal anastomosis (19 straight ileoanal anastomosis, 41 J pouch, 13 S pouch) between January 1980 and June 1995 and were observed 5.8 ± 3.3 years. The ileoanal anastomosis is nonfunctional in 19 (26%) patients. Excision rates according to type of restorative procedure were J pouch, 7% (3 of 41); S pouch, 32% (4 of 13); and straight ileoanal anastomosis, 32% (6 of 19). Failure was usually attributable to intractable diarrhea among patients with straight ileoanal anastomosis but was caused by anastomotic leak or pelvic-perianal sepsis among patients with pouch procedures. Failure rates did not vary with age at ileoanal anastomosis. Among patients retaining ileoanal continuity, continence problems reported in the questionnaire were frequent and tended to be more extreme among younger patients. Overall, 90% of respondents reported satisfaction with the functional outcome of the restorative operation. Conclusions: The success rate of the ileoanal anastomosis/J-pouch procedure is comparable to that in adult series. The ileoanal anastomosis/J-pouch procedure is the operation of choice for children and adolescents who want ileoanal continuity restored after colectomy for ulcerative colitis.

This review encompasses 50 years (1942 through 1991) and 69 newborns (43 boys, 26 girls). Half the babies were premature (weighing less than 2.5 kg), and about one third had other anomalies. The procedures used in this series were late primary anastomosis (17), gastric tube reconstruction (16), staging esophagostomy and gastrostomy (13), gastric pull-up (13), early primary anastomosis (4), and colon replacement (3). Four neonates received no treatment. The most common repair in the 1940s and 1950s was the gastric pull-up; the gastric tube was the most popular in the 1960s and 1970s. Delayed primary anastomosis has been the operation of choice since the 1980s. Over the last decade, it has become apparent that primary repair is successful in three quarters of such infants if the wait is 3 months and/or the newborn weight has at least doubled. This repair appears to provide the best functional result, unless there is an anastomotic stricture. Before the 1970s, the survival rate was below 40%, but since the 1980s the rate has more than doubled, to 90% in our series, regardiess of the type of repair used.

Splenic, portal, or mesenteric venous thrombosis after splenectomy for hematologic disease has not been reported in the pediatric literature. It is a rare complication associated with significant morbidity and mortality in adult reports. Between 1981 and 1991, 3 patients (13-year-old boy with hereditary elliptocytosis [HE], 13-year-old boy with thalassemia intermedia [TI], and 18-year-old girl with idiopathic thrombocytopenic purpura [ITP]) presented with abdominal pain, nausea, with or without fever, at 4, 11, and 13 days postsplenectomy, respectively. Abdominal Doppler ultrasound (US) and/or computed tomography (CT) showed: (1) an intraluminal filling defect with partial obstruction to flow in the right branch of the portal vein with the remaining vessels patent (HE); (2) splenic vein thrombosis with complete occlusion of the main portal vein and proximal superior mesenteric vein (TI); and (3) complete thrombosis of the splenic vein, proximal superior mesenteric vein and portal vein (including central radicles), with retrogastric collateralization (ITP). Subsequent imaging showed either complete resolution of vascular obstruction on no treatment (patient 1), or portal venous cavernomatous transformation with hepatofugal flow after 6 months of systemic anticoagulation (patients 2 and 3), and all 3 patients are currently asymptomatic. Postoperative sonographic evaluation of a consecutive series of pediatric splenectomies for hematologic disease (n = 16), was performed at a median of 51 days (range, 3 to 124). This demonstrated one case of asymptomatic left portal venous thrombosis with subsequent recanalization in the absence of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Eleven newborns with pure esophageal atresia were treated between 1980 and 1989 inclusive; there were six girls and five boys. Their gestational age ranged from 31 to 40 weeks (average, 37 weeks) and weight from 1.1 to 3.0 kg (average, 2.2). The only associated anomalies were Down's syndrome, respiratory distress syndrome, and patent ductus arteriosus. All babies received an immediate gastrostomy. Several radiologic studies were done to see if the distance between the two esophageal pouches was decreasing. Dilatations of the upper pouch were carried out in two patients. After a wait of 1 to 7 months (average, 312) a primary anastomosis was attempted; the weight of six babies doubled during this time. Eight neonates had a primary repair (two were aided by a circular myotomy). Two had a staged gastric tube constructed, and one baby had a gastric pull-up procedure. Three of the infants with a primary anastomosis required a subsequent antireflux operation, and one needed her anastomosis resected 16 months later. Ten of these 11 newborns are alive and well; one of the gastric tube children died from an adhesive small bowel obstruction at age four years. We conclude that: (1) newborns who have a pure esophageal atresia occur at a ratio of one to every 15 neonates with the common type of esophageal atresia and distal tracheoesophageal fistula; (2) there are few associated congenital defects; (3) primary repair is successful in three quarters of such infants if the wait is past 3 months and/or the newborn weight is at least doubled; (4) one third of the primary repair babies will require antireflux surgery within 3 months of the primary anastomosis; and (5) survival rate is over 90%.

Enterocolitis is the most common cause of significant morbidity and death in Hirschsprung's disease. Although most cases respond to nasogastric decompression, antibiotics, and colonic evacuation, some children have an unusually fulminant or protracted clinical course. Four cases are reported of pseudomembranous colitis (PMC) that developed 1 to 18 months (mean, 8 months) after definitive surgery for Hirschsprung's disease (Soave endorectal pull-though, 2; Duhamel procedure, 2). While all children presented with fever, abdominal distention, and diarrhea, indistinguishable from typical Hirschsprung's enterocolitis, the clinical course was fulminant in two cases, both of whom died of septic shock. Postmortem examination in both showed extensive colonic pseudomembranes despite identification of Clostridium difficile toxin and subsequent vancomycin therapy (initiated late in the clinical course). Two children in the series had protracted hospitalizations and eventually required diverting enterostomy despite recognition of C difficile toxin and treatment with enteral vancomycin, in one child necessitating multiple courses of antibiotic therapy. Awareness of the virulence of PMC associated with Hirschsprung's disease (even after definitive resection) should prompt submission of stool specimens from any child who presents with enterocolitis for both C difficile culture and toxin levels. On the basis of our experience it is our policy to initiate a prompt course of vancomycin by rectal lavage or nasogastric tube in all children with Hirschsprung's enterocolitis, pending culture results, in view of the significant morbidity and mortality exemplified by cases in this review.

We have reviewed the outcome of all patients undergoing their first intestinal resection for Crohn's disease at this hospital between 1970 and 1987. Recurrence rates, defined by recurrent intestinal symptoms and radiological confirmation of mucosal disease, were calculated using survival analysis. Age, sex, anatomical location of disease, indication for surgery, preoperative duration of symptomatic disease, use of preoperative bowel rest, and pathological features of the resected bowel were analysed individually and jointly as potential risk factors influencing postoperative recurrence of disease. Eighty two patients (age, mean (SD) 14.8 (2.5) years) underwent intestinal resection and were followed postoperatively for a minimum of one year (mean 5.3 (3.3) years). Anatomical location of disease, indication for surgery, and preoperative duration of symptomatic disease were the only factors that significantly influenced the duration of the recurrence free interval. Patients with diffuse ileocolonic inflammation experienced earlier recurrence (50% at one year) than children with predominantly small bowel disease (50% recurrence at five years, p less than 0.0001). Failure of medical therapy independent of disease location as the sole indication for surgery was associated with an earlier relapse than when surgery was performed for a specific intestinal complication such as abscess or obstruction (p less than 0.003). Patients undergoing resection within one year of onset of symptoms experienced delayed recrudescence of active disease (30% recurrence by eight years) compared with patients whose preoperative duration of symptomatic disease was longer (50% recurrence by four years when preoperative duration of disease was one to four years and 50% by three years when disease had been present greater than four years preoperatively, p = 0.03). The mean height velocity of patients with growth potential increased from 2.4 (2.3) cm per year preoperatively to 8.1 (3.4) cm per year in the first postoperative year (p=0.0001). These results support an early approach to surgery in the management of ileal Crohn's disease with or without caecal or right colonic involvement, especially when complicated by persistent growth failure. The higher recurrence rates in more diffuse ileocolonic disease emphasise the need for alternative treatment strategies in these children.

Between 1974 and 1986, inclusive, over 400 newborns with clinical, radiological, and/or pathological evidence of necrotizing enterocolitis (NEC) were treated at the Hospital for Sick Children, Toronto, Ontario. Within this group were 37 babies who had a bowel perforation that was treated with peritoneal drainage under local anesthesia. Eighty-eight percent of the 41 weighed less than 1,500 g and 65% weighed less than 1,000 g; during the same time 40 other neonates (9% of the total) with perforated NEC had laparotomies. Twelve neonates (32%) required only drainage with complete recovery of their intestinal tracts. The remaining 25 (68%) fell into one of three groups: (1) nine (24%) had rapid downhill course, sepsis, and death without laparotomy; (2) nine (24%) had rapid downhill course, sepsis, and laparotomy (five deaths); (3) seven (20%) had slow development of bowel obstruction requiring operation (two deaths). The overall survival rate was 56%. These results continue to indicate that this method is effective in temporizing 88% of the small and/or very ill babies with a NEC perforation. However, an added bonus is that 32% of these newborns treated in this fashion had complete resolution of their disease.

This case report describes intraoperative anaphylaxis occurring in a fourteen-year-old female with spina bifida in which latex surgical gloves were incriminated as the aetiologic agent. The patient was non-atopic but since eight years of age she had developed localized angioedema and urticarial skin reactions on exposure to rubber. She had previously undergone several uneventful surgical procedures. Forty-five minutes following induction of anaesthesia and during laparotomy for elective cholecystectomy she experienced sudden onset of increased airway pressure, oxygen desaturation, tachycardia, profound hypotension and erythema consistent with an anaphylactic reaction. Resuscitation with manual ventilation and oxygen, intravenous fluids and an epinephrine infusion was successful. Subsequent investigations for allergies demonstrated a strongly positive skin prick test and RAST to latex antigen, with negative results to anaesthetic agents, antibiotics and inhalant allergens. During two later operations prophylaxis consisting of diphen-hydramine, ranitidine and hydrocortisone appeared to prevent further reactions. Latex should be considered as a cause of life-threatening intraoperative allergic reactions in patients with a history of rubber allergy or frequent exposure to latex products.

Between 1974 and 1988, 86 newborns with perforated necrotizing enterocolitis (NEC) were treated by either laparotomy (usually involving a bowel resection and a temporary stoma) or a peritoneal drain under local anesthesia. The survival of babies in the laparotomy group was 57% versus 59% in the drained group. However, for neonates less than 1,000 g survival in the drained group was 69% compared to 22% for the laparotomy group (P

Since 1980, the authors have operated on seven infants with total colon Hirschsprung's disease using the long Duhamel procedure. All infants were given an ileostomy when the diagnosis was made, and the long Duhamel procedure was performed between 6 and 24 months of age. This operation is the standard Duhamel procedure except that the sigmoid and descending colon form the fecal reservoir (as in the Martin variation of the Duhamel operation). A covering loop ileostomy was made in all the patients, and it was closed within 5 months; two infants had one episode of enterocolitis, 1 to 9 months after closure. The seven infants have had follow-up for 1 to 13 years; all are alive and well. The children are continent and toilet trained (age not with-standing); they have up to 10 formed stools per day. Two had some soiling at night. Whereas in the past the Duhamel procedure (in which a too-long anterior rectal pouch was left) often resulted in the formation of a fecal impaction, this does not occur when the material passing through the anus is at best semiliquid. With no long side-to-side anastomosis, the procedure is simple and complications are few.

A teenage boy with repaired high imperforate anus relied on daily enemas for social continence. After treatment with low intensity transcutaneous electrical stimulation and electromyographic biofeedback home programs, he achieved improved fecal continence requiring only one enema per month.

Thirty-six patients with benign neonatal sacrococcygeal teratoma (SCT) were treated in our medical center from 1972 to 1990. Mean gestational age was 38.6 +/- 3.3 weeks with a mean birth weight of 3,484.0 +/- 938.5 g. Twenty-nine patients (89%) were females. The majority of the tumors (75%) contained cystic components and 96% were Altman classification I and II. The initial surgical removal of the SCT (including the coccyx) was carried out during the first 7 days of life. Six patients (22%) developed recurrence of the tumor. Three were benign and reappeared locally after 12 +/- 3 months and were reexcised. The mean serum alpha-fetoprotein level in this group was 13 +/- 1 g/L. The malignant recurrence (all originally reported as being mature benign SCT) appeared at 20.3 +/- 1.5 months and had markedly elevated serum alpha-fetoprotein levels (7,320 +/- 4,630 micrograms/L). All the patients in this group had multimodal therapy including complete excision of the recurrent tumor. We conclude that SCT, although histologically benign, has an alarming potential to recur either as a benign or malignant tumor during the first 2 years of life. Close follow-up for at least 3 years (frequent examination, serum alpha-fetoprotein, and diagnostic imaging) is recommended for all patients who had undergone excision of SCT in the newborn period.

Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.

A case of isolated rupture of the thoracic trachea due to blunt trauma in a 4-year-old child is presented. The rarity of this injury and its initial presentation as massive subcutaneous emphysema and bilateral pneumothoraces warrant its description. This child was diagnosed as having a tracheal injury by computed tomography scan. The diagnosis was confirmed at bronchoscopy and was successfully treated by thoracotomy and primary closure of a 3-cm rent in the membranous portion of the trachea. There was difficulty with intraoperative ventilation due to the large air leak through the tracheal tear. This was controlled by intermittent tamponade of the defect with sequential closure. Postoperatively, the child did well and was discharged on the sixth postoperative day. Rigid bronchoscopy done 2 months later showed no abnormality. This case demonstrates that computed tomography is helpful in the diagnosis of tracheal injuries. The treatment may require early operative intervention and closure of the defect.

The management of perianal disease (PD) in children with Crohn's disease was reviewed. Of 325 patients (184 boys), 200 (62%) developed PD. The mean age at diagnosis of Crohn's disease was 11.2 years (range, 5 to 17 years). Skin tags were present in 114 (35%) patients, fissures in 165 (51%), fistulas in 41 (15%), and perirectal abscesses in 47 (13%). One hundred fifty-three patients had PD as one of the first symptoms; 71 of these had complete remission of PD and the remaining 82 had chronic symptoms. Forty-two patients were initially free of PD, only to develop it later. PD occurred in 50% of patients with gastroduodenal Crohn's disease, 57% with jejunal, 68% with ileal, 64% with ileocecal, 60% with ileocolic, and 51% with colonic. Local minor operation was necessary in 38 (12%) patients, fistulotomy in 12, and abscess drainage in 26. Four patients required either intestinal resection and/or enterostomy for progressive PD. Fifty-three patients received metronidazole, with amelioration in 38. It is concluded that PD is a common complication of Crohn's disease in children and adolescents. Its presentation may antedate that of the primary disease. Most PD follows a benign course. There is a limited role for operation, but a need for meticulous perineal care and a potential benefit with metronidazole therapy. We advocate conservatism in the management of perianal complications of Crohn's disease in pediatric patients.

Background/Purpose: No surgical treatment for total colonic aganglionosis (TCA) clearly has been proven superior. To identify clinical criteria associated with long-term functional outcome, a 28-year retrospective institutional review of this entity from 1969 through 1996 inclusive was undertaken. Methods: Total colonic aganglionosis (TCA), defined here as aganglionosis extending from the anus to at least the ileocecal valve but no further than 50 cm proximal to the ileocecal valve, was identified in 29 infants and children. Appropriate leveling ileostomy was performed in 28 of 29 patients, and definitive surgical reconstruction was performed in 26 of 29. Three groups were identified based on the definitive surgical repair performed: group 1, construction lacking or incorporating a short ganglionic-aganglionic common channel (modified Soave or modified Duhamel, n=8); group II, construction of an extended common channel (Martin-Duhamel, Martin-Soave; n=6); and group III, all others including an intermediate-length common channel (n = 13). Results: Functional outcome at extended follow-up (mean, 6.6 ± 5.6 years; range, 0.7 to 23) was determined based on survival, long-term ostomy requirements, growth, major complications, continence, and enterocolitis and bowel movement frequency. Although long-term functional outcome was deemed satisfactory in six of seven patients in group I, function was satisfactory in none of six group II patients. Group III results were intermediate (satisfactory in 6 of 13). Conclusions: Acceptable long-term outcome was most frequent in TCA patients whose definitive repair did not incorporate an extended ganglionic-aganglionic common channel. The use of extensive lengths of aganglionic bowel to maximize fluid absorption is frequently met with substantial morbidity.

Background/Purpose: Thymic cysts are rare lesions of the neck and mediastinum that are difficult to diagnose. Often considered inconsequential, these lesions can frequently be symptomatic. In this report the authors contrast their experience with that of the literature. Methods: From 1984 through 1997, the authors encountered 14 patients with this lesion. All cysts were completely excised. Patients that had an acquired cyst of the thymus were excluded from this series. Results: Of the 14 patients ranging in age from 2 weeks to 16 years, seven patients had cervical masses, five had mediastinal masses, and two children had both sites involved. Seven children were symptomatic with wheezing and upper respiratory infection, with cough and fever being the most common clinical features. Investigations included chest radiograph, contrast esophagram, sonography (US) and computerized tomography (CT). Displacement of vital mediastinal or neck structures was observed in eight patients. Only two patients received correct diagnosis before surgery. Successful and complete excision of all cysts was achieved. The cysts were benign and ranged in size from 2 to 22 cm in diameter. Conclusion: Often forgotten, thymic cysts are rare benign lesions that should be considered in the differential diagnosis of cervical and mediastinal masses in children.

Intraperitoneal culturing during appendectomy is a routine procedure. Significant decrease in the mortality and dramatic improvement in the morbidity were achieved by using antibiotics perioperatively. The value of intraoperative abdominal cavity culture was assessed in our study.A total of 499 patients formed two groups, those with acute nonperforated appendicitis (group A) and those with perforated appendicitis (group B). Intraoperative abdominal cavity culture were taken randomly in both groups. The perioperative morbidity, the validity, and the impact of positive culture on the antibiotic treatment were examined in both groups.Clinical diagnosed perforation was confirmed histologically in 176 patients (98.3% accuracy). Intraperitoneal cultures were obtained in 30.1% of the patients in group A and in 67.1% of group B. The majority of the patients in group A were treated preoperatively and postoperatively by a single antibiotic agent whereas 58.0% of the patients in group B were started on triple-agent antibiotics for significantly longer periods (22.4 +/- 9.4 versus 5.7 +/- 7.4 doses, respectively; P0.0001). No significant difference was found in both groups in the postoperative complication rate (wound infection, intra-abdominal abscess and small bowel obstruction) whether intra-abdominal culture was obtained or not (5.9% versus 4.7% in group A and 21.2% versus 21.9% in group B; P0.05).Traditional intraoperative abdominal cavity culture can be abandoned. In perforated appendicitis, colonic flora can be predicted, and antibiotic therapy should begun without any abdominal cavity culture results. This practical approach will save money and reduce laboratory work without affecting the patient's morbidity.

This paper describes the correct diagnosis of cecal malposition, suggesting midgut malrotation, during air enema examination in seven patients. It is possible to diagnose cecal malposition by air enema, even in the presence of a reducible intussusception.

To evaluate the current management of the infant and child with intussusception, the medical records of 188 consecutive intussusception patients over 5 years (1985–1990) were reviewed and compared to our series from 25 years ago (1959–1968). The peak months changed from May and June to January and July. Duration of symptoms and signs prior to diagnosis increased by one-third to 35 h with, however, a decrease in the incidence of pain, vomiting, abdominal mass, and rectal blood. Air was the only contrast used for the hydrostatic enema in the present series and was tried in every case with 81% success; this is a major improvement from 45% in the old series. There were three perforations (1.4%) with air-enema attempts compared with 1 (0.2%) 25 years ago. Recently only 19% of patients required operation but 30% needed resection; 55% of the patients in the older series required operation and 20% needed resection. Ten percent of intussusceptions continue to be found spontaneously reduced at operation. There were many less pathologic lead points in the newer series. The recurrences increased from 4% to 7%, but their reduction rate also increased from 31% with barium to 100% with air. There were no deaths in the last 25 years.

Large adrenal cysts usually occur in the fifth and sixth decades of a patient's life but are rare in the first two decades. This paper presents the clinical, cross-sectional imaging, surgical, pathological and follow-up data of three teenage girls with large adrenal cysts. Two had vague upper abdominal pain and, in the other, the cyst was found incidentally. The cysts were surgically removed in two patients, while in the third, follow-up imaging has shown no change in the cyst over a 4-year period, suggesting that conservative management is a reasonable option.

Background. There is confusion in the radiological literature as to the site of abdominal calcification in cystic fibrosis (CF) with meconium ileus (MI) in neonates. Purpose. To correlate the site of radiographic abdominal calcification with histologic and operative findings. Materials and methods. A review of clinical, radiographic, surgical and histologic data in 58 neonates with CF and MI. Results. Abdominal calcification was identified in 15 (26 %) neonates: on an abdominal radiograph in 8 (13 %), at laparotomy in 3 and histologically in 10 (37 %) of the 27 resected specimens. The radiographic pattern of calcification varied from small specks in three cases to small, better-defined areas in two. In the other three patients, the calcification was more extensive and curvilinear. Histologically, calcification was found to be intramural in ten resected specimens, of which two also had intraluminal and one serosal calcification. The more extensive, curvilinear calcification identified radiographically correlated with histologically proven dystrophic intramural calcification. The less marked flecks or discrete areas of radiographic calcification may represent intramural, serosal or intraluminal calcification. Conclusion. Intramural calcification is common microscopically in CF with MI. Extensive radiographic calcification in these patients is more likely to represent intramural rather than serosal or intraluminal calcification.

Since 1980, the authors have not routinely removed an appendix on an interval basis after treatment of a ruptured appendiceal mass (phlegmon and/or abscess). In the present group of patients, there were eight boys and two girls, two to 15 years of age (mean, 8.5 years). All presented with symptoms and signs typical of ruptured appendix, with a mass suspected by history and examination, and proven by radiological means (usually ultrasonography). The patients were treated for at least 1 week with intravenous triple antibiotics; three required drainage of their abscess (2 radiological, 1 surgical). The follow-up has been both clinical and sonographic. In all cases the inflammation disappeared with 1 month. One child (2 years old) returned in 2 months with symptoms and signs of a ruptured appendix, and appendectomy was performed. The other nine have remained well, for 6 months to 13 years. From this experience and a review of the literature, only a relatively small number of patients with a properly treated ruptured appendiceal mass (phlegmon and/or abscess) will return with a flareup (recurrence) of appendicitis (requiring appendectomy); the rest live a normal life, with their asymptomatic appendix intact.

A pilot study on the percutaneous introduction of a cecostomy tube for colonic irrigations in the treatment of children with fecal incontinence is described. The results were good, and the technique is recommended for certain patients.

Impalpable testes constitute approximately 20% of most series of undescended testes. From January 1986 to March 1991, we performed laparoscopies on 53 patients with impalpable testes. Thirty-two of them were found to have normal vasa and vessels entering each internal ring on the side in question. Of these, 14 were found to have “vanishing testes” at exploration, 12 others underwent successful orchiopexy, and the remaining 6 had excisional biopsies of fibrotic testicular remnants. Five patients had no visible vessels and a sixth had a blind-ending vas and vessels adjacent to the internal ring; in these cases no further investigations were deemed necessary. Fifteen patients were found to have abdominal testes and underwent high testicular vessel ligation and division at the time of the laparoscopy; 14 of them have undergone staged orchiopexy 6 months after laparoscopy and one is scheduled for this procedure. A 3-month follow-up of those who had orchiopexy showed excellent results in 10 patients and poor results in 3, all of whom had small testes that were unimproved or worse following vessel ligation. Four boys were spared operations as a result of findings at laparoscopy. Early in the series there was one failed laparoscopy, but it was successfully completed later. The first patient in this series developed peritonitis following the procedure, but it was successfully treated with antibiotics. There were no other complications. Laparoscopy is a safe procedure that allows accurate diagnosis and may prevent additional intervention in the treatment of the absent testes. It facilitates the locating of the impalpable testis and the planning and timing of subsequent orchiopexy. We believe that laparoscopy is the preferred procedure in the management of impalpable testes.

Chest wall motion during anaesthesia may differ from the awake state because of the effect of anaesthetic agents on the muscles of respiration. The purpose of this study was twofold (1) to describe the pattern of chest wall motion in infants and children during halothane anaesthesia (HA) using respiratory inductive plethysmography (RIP) and (2) to calibrate the voltage output of RIP in units of volume. Seven infants (2.3 ±1.7 mo, 5.9 ±0.7 kg) and five children (2.9 ±1.1 yr, 15.5 ±1.5 kg) were studied. Since results in both age groups were qualitatively similar they are presented as a single group. Respiratory excursions of the rib cage (RC) and abdomen (ABD) were measured using RIP. Airflow was measured with a pneumotachograph. During spontaneous breathing the analogue signals of airflow, pressure, RC and ABD were recorded. Measurements were taken during (1) halothane anaesthesia and (2) during emergence from anaesthesia. The XY plots of the RC and ABD signals were plotted for each period. In addition the voltage output of the respiratory excursions of the RC and ABD signals was converted to units of volume using the simultaneous solution of equation method. The accuracy of conversion factors was validated by regression analysis of the predicted and measured tidal volume using breaths sampled at random throughout the entire period of study. Regression analysis of this relationship gave a slope between 0.85 and 1.15 (r2 value > 0.7) in five of the twelve patients. The pattern of chest wall motion in the X Y plots showed synchronous motion between RC and ABD signals during HA in nine of the twelve patients. Three patients showed paradoxical inward motion of the RC during inspiration. This finding differs from that of previous studies which showed asynchrony between the RC and ABD signals during HA.

Eight-four children, all of whom had imperforate anus, were evaluated by measuring the anal sphincter force (ASF). Sixty-three were boys, 46 of whom had a high imperforate anus and 17 of whom had the low anomaly. There were 7 girls with cloacal malformations, 7 girls with high type and 7 with low type of malformation. In all the boys with a high imperforate anus the ASF ranged from 50 to 300 g. Those boys with a low type had an average ASF of 513 g. Normal values vary from 480 to 600 g. Twenty-five of the boys with a high imperforate anus had undergone posterior sagittal anorectoplasty and none were clinically continent. Their ASF measurements were lower than in patients with spina bifida. Patients with a low anomaly, both boys and girls, were continent and had high ASF readings. The ASF is a simple, inexpensive, and objective measurement of fecal incontinence when this is the consequence of muscular inadequacy. A reproducible quantification is now available to compare results of different operations and different centres.

We studied the clinical course of 506 children consecutively admitted with appendicitis at The Hospital for Sick Children from 1985 to 1989. One hundred eighty-one children (35%), ranging in age from 1 to 17 years, presented with perforation verified by histological examination. Ninety-six of them (53%) had generalized peritonitis, 47 (26%) had localized peritonitis, and 38 (21%) had abscess formation. Usually, triple antibiotics were begun preoperatively if perforation was suspected; otherwise, cefoxitin was started. Triple antibiotics were used postoperatively for 5 to 7 days in almost all children in the perforated group. Neither abdominal nor subcutaneous drainage was routinely used even in cases of intraabdominal abscess. The skin was closed primarily with steri-strips (63%), staples (20%), subcutaneous Dexon (11%), or silk (4%). Postoperative wound infection arose in 20 children (11%). Wound infections were noted from 1 to 14 days postoperatively (mean, 5.9 days). Whereas 9 of these were treated with local therapy only, 11 delayed the child's discharge or necessitated readmission. No patient suffered major complications from wound infection in that there were no cases of necrotizing fasciitis, reoperation for debridement, sepsis, or death. The intraabdominal abscess rate in this group of 181 children was 6% (n = 11). The low rate of infective complications fully justifies the policy of primary closure in contaminated wounds. This policy eliminates the necessity for painful and time-consuming dressing changes, shortens hospitalization, and obviates the trauma of delayed suturing of wounds in children.

Between 1958 and 1987, 13 children (aged 8 to 17 years) with 20 pheochromocytomas were diagnosed and treated. Five of these 13 had multiple (12) tumors and four patients had five recurrences in 6 years (1980 to 1986). There were three girls and one boy between 8 and 15 years of age. One girl had von Hippel-Lindau disease. Three of four original tumors were adrenal and less than 6 cm in diameter. All were excised without complications and went home normotensive. The five recurrent tumors were diagnosed and treated within 6 years of the original tumor when the children were 9 to 17 years; their presenting symptoms and signs (3 months) were hypertension plus others, not necessarily the same as previously. Computed tomography scan was again positive in the three that had this test; vanillylmandelic acid was the only uniformly positive biochemical test. Norepinephrines were positive in the three patients examined. The three girls had their recurrent tumors found in the opposite adrenal and the boy once again had two more tumors (adrenal and chest); all tumors were less than 3 cm in diameter and easily excised. They remain normotensive and well up to 8 years. Within this small group of recurrences, there was no specific pattern that would have led one to suspect that they would return with another pheo. There was nothing unusual about their recurrent tumor, except that it was usually in the opposite adrenal; only one of the four children has no remaining adrenal tissue. Although follow-up is important, recurrent pheochromocytomas seem to be heralded by recurrent symptoms and signs of hypertension.

The long-term results of thymectomy in 24 children with generalized myasthenia gravis are reviewed. Sixteen had complete remission and another seven were improved. This compares favorably with reported spontaneous remission rates of 30%. Because of the low morbidity in recent reports and the possibility that early thymectomy is more beneficial, we recommend thymectomy at the onset of juvenile generalized myasthenia gravis. ( J Child Neurol 1990;5:215-218).

Aluminum loading in children with chronic renal failure. Kidney Int 1984;26:201. 9. Griswold WR, Reznik V, Mendoza SA, Trauner D, Alfrey AC. Accumulation of aluminum in a nondialyzed uremic child receiving aluminum hydroxide. Pediatrics 1983;71:56. 10. Slanina P, Frech W, Ekstrom LG, Loof L, Slorach S, Cedergren A. Dietary citric acid enhances absorption of aluminum in antacids. Clin Chem 1986;32:539. l l. Trompeter RS, Polinsky MS, Andreoli SP, Fennell RS. Neurologic complications of renal failure. Am J Kidney Dis 1986;4:318-23. 12. Roodhooft AM, van de Vyver FL, Haese CD, van Acker K J, Visser W J, de Broe ME. Aluminum accumulation in children on chronic dialysis: predictive value of serum aluminum levels and desferrioxamine infusion test. Clin Nephrol 1987;28: 125-9. 13. McCarthy JT, Milliner DS, Kurtz SB, Johnson W J, Moyer TP. Interpretation of serum aluminum values in dialysis patients. Am J Clin Pathol 1986;86:629-36. 14. Bene C, Manzler A, Bene D, Kranias G. Irreversible ocular toxicity from single "challenge" dose of deferoxamine. Clin Nephrol 1989;31:45-8. 15. Lawson M, Milla P J, Harper JI. Aluminum and infant formulae. Lancet 1989;1:614. 16. BirchalI JD, Chappell JS. Aluminum, chemical physiology, and Alzheimer's disease. Lancet 1988;2:1008.

Percutaneous insertion of a cecostomy tube, performed under local anesthesia, to facilitate antegrade colonic cleansing, has been an invaluable advance in the management of fecal incontinence. However, the patient is left with a length of tubing (2 to 4 inches) protruding from the cecostomy site that has to be taped down to the abdominal wall. Available devices for insertion in place of the cecostomy tube are cumbersome and have a relatively high profile, projecting more than 1 cm from the surface of the abdominal wall. Worn under a swimsuit, they are clearly discernible. The inflated balloon within the cecum can occasionally break. Furthermore, in the individual with a relatively thick abdominal wall, such devices are too short to reach from the skin to the cecum. A new form of low-profile trapdoor device has been developed that overcomes the above shortcomings of other available "buttons." It has been successfully used in a clinical setting in 49 patients.

The policy of conservative nonoperative management of the injured spleen is reviewed, and recommendations are made to improve this mode of therapy. From 1981 to 1986, 75 patients were admitted with diagnosed splenic injury. Of thes, ten were operated on (four splenorrhaphies, three total splenectomies, one partial splenectomy; in two, the spleen had stopped bleeding spontaneously) and three died, all from causes unrelated to splenic trauma. Only 23% of the patients treated nonoperatively required blood transfusions, and the length of both hospital stay and time spent in the ICU was reduced. The results of this review show that, in comparison with our previous 5-year report, the number of patients treated without surgery increased from 70% to 87%, those receiving blood transfusions decreased from 36% to 23%, and the number undergoing a splenectomy decreased from 24% to 4%. These data suggest that almost all children with splenic injury can be successfully treated without an operation, those who are hemodynamically stable do not require ICU care, and the total hospital stay for uncomplicated splenic injury can be limited to seven days. A laparotomy can be safely reserved for patients with immediate massive hemorrhage or with transfusion requirements of greater than 40 mL/kg.

From 1968 to 1976 inclusive, 69 neonates with diaphragmatic hernias had corrective surgery within 18 hr of birth and the survival rate was 41%. During the same time, all babies with similar hernias who were operated on later than 18 hr from the time of birth survived. Our present interest has been focused on the pulmonary artery and its hypertension with the subsequent development of right to left shunting through the patent ductus arteriosus. During 1977 and 1978, we attempted to enter 19 consecutive newborns 18 hr of age or less with symptomatic Bochdalek diaphragmatic hernias into a "Collins protocol" for treatment. This included four stages: newborn nursery initial resuscitation, operation, cardiac catherterization, and ICU monitoring and pharmacological therapy. There was a total of seven survivors (36%), however for a number of reasons only eight babies really had a complete entry into this protocol and of these eight, five survived. Although this study is far from complete, some initial information and concepts are forthcoming. It is now apparent to us that there are three distinct groups into which these very early newborns fall: minimal pulmonary hypoplasia, unilateral hypoplasia and bilateral hypoplasia. The first group probably does not need pharmacologic support, while in the last it probably does not help. Further interest in other more specific pulmonary pharmacologic agents is now being considered as well as some way of early recognition of which baby is going to fit into which group, so that only the ones that need this treatment will get it.

Postoperative pain is a major problem following surgery in the ambulatory child. A study was undertaken to test the effect of intraoperative bupivacaine on postoperative pain in children undergoing outpatient hernia repair. Ninety-nine children aged 1 to 7 years underwent outpatient inguinal herniorrhaphy under general anesthesia. Each was randomly assigned to receive bupivacaine (group 1) or saline (group 2), infiltrating the ilioinguinal and iliohypogastric nerves. Drug administration and patient evaluation were double-blinded. The groups were similar with respect to age, sex, side of procedure, and length of operation. In the immediate postoperative period, 17 group 1 patients required analgesics compared with 39 in group 2 (P less than .01); total codeine dosage was lower in group 1 (4.0 +/- 7.1 mg v 11.8 +/- 10.5 mg, P less than .05). Activity level 45 minutes after surgery (using a standardized scale) was greater in group 1 (P less than .05). Acetaminophen requirements at home were lower in group 1 on the day of surgery (3.1 +/- 4.3 mL v 5.7 +/- 7.4 mL, P less than .05) and over the following 48 hours (1.5 +/- 3.4 mL v 4.9 +/- 10.7 mL, P less than .05). Activity level at home on the day of surgery did not differ significantly between groups, but activity level over the following 48 hours was higher in group 1 (P less than .05). The two groups were similar with respect to all other parameters. We conclude that intraoperative bupivacaine decreases post-operative pain and analgesic use, and promotes early ambulation in children undergoing hernia repair.

Severe stress can produce ulceration and uncontrollable hemorrhage in the pediatric age group. Modern angiographic techniques, using embolization of a Silastic balloon to occlude a visualized bleeding vessel, can successfully control hemorrhage and avoid surgical intervention and its resultant disordered gastric physiology. Recently this technique was used to control hemorrhage from the gastroduodenal artery in a 1-year-old child with severe respiratory sepsis. This is the youngest reported child in whom the technique has been used to control bleeding from this vessel.

We evaluated the causality of the association between intrauterine exposure to phenytoin and postnatal neuroblastoma using an in vitro lymphocyte toxicity assay for phenytoin-induced reactions in an unusual sibship. In addition, we investigated intrauterine phenytoin exposure in a case series of infants and children with neuroblastoma diagnosed over 17 years at our center. The response of lymphocytes from our index case with neuroblastoma exposed in utero to phenytoin was within the normal range, whereas the mother and a sibling with fetal hydantoin syndrome (FHS) exhibited an intermediate toxicity. None of the 188 cases of childhood neuroblastoma diagnosed between 1969 and 1988 had been exposed in utero to phenytoin, indicating that, statistically, the drug cannot be associated with neuroblastoma in more than two cases with this malignancy in our cohort, or in 1.5% of all cases of neuroblastoma. Although our data do not suggest an association between phenytoin in pregnancy and postnatal neuroblastoma, it is still possible that there is an increased risk for neuroblastoma in children with FHS.

Lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat with a tendency to local invasion but not to metastasize. To date, there have been 60 cases described in the international literature. There has never been a report in a child older than 8 yr. The main clinical characteristics are the presentation, usually below 3 yr of age, the rapid growth of the mass and the peripheral location, mainly in the extremities. Although CT scan may show a fatty tumor, there is no single test to make the differential diagnosis (which includes benign lipoma, liposarcoma and myxoliposarcoma) and the treatment should be based on clinical findings. We have treated four patients with this condition. Two were intrathoracic, one was intraperitoneal, and one was a tumor of the upper arm. All presented as rapidly-growing tumors and were clinically assumed to be malignant. We recommend complete but conservative excision of the tumor; there are reported recurrences after inadequate excision. This is a benign tumor and radical cancer surgery should be avoided.

Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.

In spite of recent reports of more aggressive investigation and treatment of malignant liver tumors in infants and children, there is still an alarming morbidity and mortality in the operative aspect of this tumor. Our operative approach to partial hepatectomy follows that laid down by many authors, but we seem to have the same intraoperative problems in removing the huge tumor confined to one liver lobe. The blood loss in such operations may equal or exceed the child's blood volume while intraoperative cardiac arrest is not unknown. The bleeding can be most acute in the course of the retrohepatic caval and hepatic veins dissection. Once bleeding becomes excessive, the dissection must continue in a hurried fashion leading to the above problems. Since 1977, six children have been admitted to our hospital with huge malignant liver tumors involving almost all of one liver lobe and part of the other. Each was explored through a long midline abdominal incision finding what was felt to be a resectable liver tumor. The incision was then carried upwards via a sternal split or a right thoracoabdominal incision and the liver mobilized. The patient was then put on cardiopulmonary bypass and cooled to a rectal temperature below 20 degrees C. Circulation arrest at this low temperature provided 1 hr or less of bloodless dissection, and an extended hepatic lobectomy was easily carried out. The patients were rewarmed on bypass and normal hemodynamics restored. Bleeding from the liver edge was controlled and the remainder of the operation completed. What was previously the most difficult aspect of a liver tumor operation has become the easiest part of the entire procedure. This operative approach is recommended in highly selected large tumor cases.

Fifty-eight infants with congenital diaphragmatic hernia presenting within the first 6 hours of life, who underwent surgical repair, were analysed prospectively in order to produce a reliable index of severity of disease that would reliably predict eventual outcome. All were treated with paralysis hyperventilation and intravenous (IV) isoproterenol for the first 48 hours. There were 30 survivors and 28 deaths in this series (mortality 48%). Using arterial PCO 2 values measured 2 hours after surgical repair and correlating them with an index of mechanical ventilation (mean airway pressure and respiratory rate), we have been able to clearly define two groups of diaphragmatic hernia based on their response to IPPV. The first group, with CO 2 retention and severe preductal shunting, was unresponsive to hyperventilation with high rates and pressures; the mortality was 90%. The second group responded well to hyperventilation and demonstrated reversable ductal shunting only. Survival in this group was 97%. Only four patients out of 58 exhibited the "honeymoon period," with a period of stability followed by severe ductal shunting. Arterial CO 2 accurately reflects the degree of lung development in this disease and separates those patients with severe pulmonary hypoplasia, where the outcome is invariably fatal, from those with a well-developed contralateral lung where there is excellent potential for survival.

A 20-year retrospective study was made of children with congenital posterolateral (Bochdalek) hernias presenting more than 8 weeks after birth. The records of 26 patients (16 boys and 10 girls) were evaluated. Sixteen infants and children (62%) were originally misdiagnosed clinically and radiologically as having either infective lung changes, congenital lung cysts, or pneumothoraces; inappropriate thoracentesis occurred in four patients misdiagnosed as having a pneumothorax. Five patients had previously normal chest radiographs. The most useful investigation was a plain radiograph following passage of a nasogastric tube. Coexisting abnormalities (in particular, gut malfixation and malrotation) were common. All patients except one were operated on within days of presentation, and as emergencies if symptoms were acute. More than one third of our patients were left with a smaller than normal ipsilateral lung after their diaphragmatic hernia repair, and these lungs must be considered hypoplastic to some degree. Chest tubes made no difference in the lung's eventual expansion. Two deaths occurred as a result of acute cardiorespiratory arrest in previously well children. Therefore, the symptoms, signs, and radiologic findings of patients with diaphragmatic hernias presenting after the neonatal period may be difficult to interpret, and may result in diagnostic delay, misguided therapy, and a potentially fatal outcome.

The results of operative manipulation of the vas deferens have unknown results. In order to document early and late structural changes in the vas after simple operative manipulations, the vasa deferentia of 120 Wistar rats were subjected to 4 different manipulations. Subsequently, they were removed at intervals (up to 6 mo) and examined after appropriate staining. Finger or vascular clamp grasping resulted in mild inflammation at 6 wk, which resolved thereafter. Forceps grasping disrupted the muscle layers in 30%-40% of the vasa. Hemostat clamping damaged muscle layers in 100% of the vasa and resulted in abscess formation in 30%. Healing in forceps grasped and hemostat clamped specimens was negligible and haphazard with minimal evidence of normal collagen or elastin at 6 mo. All differences were significant (p less than 0.05). These results suggest that operative manipulations of the vas should be minimized and limited to digital maneuvers. Damage from other manipulations is appreciable, long-term, and only minimally repaired at 6 mo.

In the past 10 years, the diagnosis of pseudoobstruction lasting more than two months was confirmed in 10 infants after sepsis, meconium ileus, and Hirschsprung's disease were excluded, and surgery or autopsy failed to demonstrate a site of mechanical intestinal obstruction. Four infants had undergone prior operation for another anomaly: gastroschisis (2) and ileal atresia (2). Five of the remaining infants also had megacystis. The lack of coordinated intestinal motility was best appreciated by radiocontrast small bowel studies, which showed degrees of aperistalsis or segmentation. Rectal manometric studies were not helpful. Histology of the intestine was normal in seven, while a gross deficiency of nerve fibres was noted in one patient and a myopathy of smooth muscle in another. A variety of drugs used to stimulate peristalsis were ineffective. Seven patients had 25 operations, often to exclude mechanical causes of obstruction. The mainstay of treatment was TPN and intestinal decompression. Six children survived; their ages ranged between 8 months and 9 years (median age, 16 months). There has been improvement in intestinal peristalsis in five children, three of whom now tolerate a regular diet and two of whom are on TPN and are currently increasing oral intake. Four infants died, two from sepsis, two from TPN-related hepatic failure. In contrast to previous reports, we conclude that intestinal pseudoobstruction may be self-limited in some neonates, including those with megacystis. Therapy should consist of long-term nutritional support and treatment of other anomalies that may be present.