Outcome after ileoanal anastomosis in pediatric patients with ulcerative colitis.

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.

Long-term morbidity in total colonic aganglionosis.

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.

Congenital thymic cysts in children--mostly misdiagnosed.

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.

Is abdominal cavity culture of any value in appendicitis?

BACKGROUND: 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. METHODS: 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. RESULTS: 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; P < 0.0001). 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; P > 0.05). CONCLUSION: 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.

The diagnosis of malrotation during air enema procedure.

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.

Intussusception in the 1990s: has 25 years made a difference?

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.

Fecal incontinence in children: treatment with percutaneous cecostomy tube placement--a prospective study.

PURPOSE: To evaluate the technique used for and long-term results of percutaneous cecostomy tube placement for the treatment of fecal incontinence in children. MATERIALS AND METHODS: After an initial pilot study in 15 patients, 42 additional patients with fecal incontinence aged 2-20 (mean, 11.5) years and weighing 9.9-109.0 (mean, 39.2) kg underwent percutaneous cecostomy tube placement. Twenty-nine patients had spina bifida, nine had imperforate anus, three had cloacal anomalies, and one had Hirschsprung disease. Mean follow-up was 265 days (range, 8-503 days). RESULTS: Tube placement was successful in all patients. One patient developed local inflammation after accidental early retention-suture removal, which was treated with suture replacement and intravenous antibiotics. Another developed postprocedural ileus, which resolved. Late complications included constipation in one patient (treated with diet alteration), granulation tissue in seven patients (treated with silver nitrate cautery), and accidentally dislodged tubes in three patients (two successfully replaced at home and one replaced at the radiology suite). Vomiting related to the phosphate enema occurred in two patients. Resolution of soiling was achieved in all patients. CONCLUSION: Percutaneous cecostomy and antegrade enemas are very successful in achieving fecal continence and patient independence and acceptability, with minimal early and late complications.

The cecostomy button.

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.

Abdominal calcification in cystic fibrosis with meconium ileus: radiologic-pathologic correlation.

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.

Large adrenal cysts in teenage girls: diagnosis and management.

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.

Laparotomy or drain for perforated necrotizing enterocolitis: who gets what and why?

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 <.01). As the weight of the babies increased over 1,000 g, the survival in the laparotomy group increased to 67%. There was no significant increase in survival in infants over 1,500 g. The highest neonatal mortality risk is generally found among babies weighing less than 1,000 g at birth with a gestational age of less than 30 weeks. This risk increases even more when perforated NEC is added to the prematurity. With the use of peritoneal drainage, survival in this group can approach that of larger neonates.

Laparotomy or drain for perforated necrotizing enterocolitis: who gets what and why?

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 <.01). As the weight of the babies increased over 1,000 g, the survival in the laparotomy group increased to 67%. There was no significant increase in survival in infants over 1,500 g. The highest neonatal mortality risk is generally found among babies weighing less than 1,000 g at birth with a gestational age of less than 30 weeks. This risk increases even more when perforated NEC is added to the prematurity. With the use of peritoneal drainage, survival in this group can approach that of larger neonates.

A 45-year experience with surgical treatment of peptic ulcer disease in children.

Peptic ulcer disease (PUD) requiring surgical treatment has become rare with the availability of modern medical management. A retrospective study of all patients who required operations for PUD between 1949 and 1994 (n = 43) was done. The patients were classified into 3 groups: A (n = 38): pre-histamine-2 (H2) blocker era (1949-1975); B (n = 3): pre-hydrogen-potassium (H-K+) ATPase inhibitor era (1976-1988); C (n = 2): H-K+ ATPase inhibitor era (1989-1994). Data, analyzed using X2 analysis (P < .01), included preoperative medical therapy, surgical indications, type of operation performed, complications, and postoperative medical therapy. The indication for surgery in group A was bleeding (26), perforation (8), or obstruction (4); in group B the indication was obstruction (2) or perforation (1); in group C the indication was obstruction (1) or bleeding (1). The incidence of obstruction as an indication for surgery did not differ among the groups (P < .01). Two of the three patients who had surgery for obstruction in groups B and C had biopsy-proven Helicobacter pylori. The postoperative morbidity rate was lower for groups B and C, although not significantly. The relative mortality among the groups did not change (P > .01). Children with PUD can have complications similar to those of adults with PUD. Since the introduction of H2 antagonists, the recognition and treatment of H pylori, and the use of H-K+ ATPase inhibition, the incidence of operations for bleeding and perforation has decreased dramatically. However, the incidence of surgery for obstruction remains the same.

Is interval appendectomy necessary after rupture of an appendiceal mass?

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.

Percutaneous cecostomy: a new technique in the management of fecal incontinence.

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.

Initial experience with one-stage endorectal pull-through procedures for Hirschsprung's disease.

To evaluate our experience with one-stage endorectal pull-through (ERPT) procedures (without colostomy) for Hirschsprung's disease (HD), we compared 7 such patients to a cohrt of 20 consecutive patients undergoing EROT folowing colostomy. Reasons for exclusion from hte cohort group included: (1) bowel obstruction requiring operation before 1 month of age; (2) presentation with enterocolitis (EC), intestinal perforation, or massive fecal distension; (3) long-segment disease; or (4) severe associated anomalies. The 7 male patients in the single-stage ERPT group first presented at a median age of 16 days (4 days-2.5 years) and were managed by regular digital dilation or colonic irrigation for between 1 and 6 months (median 2 months) prior to surgery. Single-stage procedures were performed at a median age and weight of 4 months (2 months-2.5 years) and 6.4 kg (4.5-13.8 kg), respectively Median hospitalization for these patients was 9 dyas. Postoperative complications occurred in 2 patients (29%), and included anastomotic stricture requiring outpatient dilation, and 1 case or recurrent EC that responded to a course of anal dilations. The cohort group (14 M, 6 F) presented at a median of 15 months. All underwent colostomy as a primary procedure. ERPT was deferred until a median age of 21 months, and the hospitalization after pull-through averaged 10 dyas (20 days including stay after colostomy). Four patient (20%) developed complications requiring reoperation following the initial colostomy. Complications after ERPT occurred in 5 patients (25%) and included 1 death from fulminant Hirschsprung's EC. Other complications included 1 anastomotic stricture and 2 mucosal prolapses requiring anoplasty. Long-term functional results were similar in both groups. Despit our limited experience, we conclude that one-stage ERPT can be safely performed in infants, including those under 3 months of age, with rectosigmoid HD. Total duration of hospitalization is reduced and colostomy complications are avoided. Functional outcome appears to be comparable between patients treated in one or two stages.

Pure esophageal atresia: a 50-year review.

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, regardless of the type of repair used.

A new look at an old operation for aganglionosis.

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 in 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 withstanding); 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.

Continence following electrical stimulation and EMG biofeedback in a teenager with imperforate anus.

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.