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The marked advantages and merit of pre-term and particularly pre-labor (PTPL) cesarean section (C-section) in the avoidance, and indeed, virtual elimination of severely disabling gastroschisis (GS) complications in infants diagnosed prior to birth by ultrasound has unfortunately remained controversial in the 10 to 12 years since it was first reported and strongly recommended by numerous authors. During this period, GS has remained one of the four major causes of the short-gut syndrome (SGS) in infancy and childhood and a major cause of prolonged, costly, complicated, and hazardous neonatal intensive care unit stays with requirements for total parenteral nutrition (TPN). The most serious and frequent complications of GS in infants born without PTPL C-section are the occurrence of the "peel", which greatly enlarges and rigidifies the eviscerated gut, and of "complicated GS" (intestinal atresia/s, stenosis, necrosis, perforations) (CGS). The "peel" occurs in 100% of these cases and CGS in approximately 20%. "Peel" enlargement and rigidification of eviscerated intestine in the presence of a reduced peritoneal cavity causes great difficulty in covering the eviscerated, enlarged, and rigidified gut with abdominal wall, skin, a prosthesis, etc., and frequently produces gut ischemia from excessive pressure, which may lead to necrotizing enterocolitis (NEC) and SGS as well as prolonged hospital stays. The presence of a "peel" greatly complicates the hazards of dealing with cases of CGS, as resection and anastomosis are virtually impossible in the presence of a "peel." The authors report personal experience with 77 cases of GS dating as far back as 1951; 44 of the infants were born after the onset of labor by vaginal or C-section delivery and all had some degree of "peel" formation. Of 320 cases from the literature (including some of the cases reported here), 61 (19.1%) involved CGS. Of the 33 cases born PT, and especially PL, there were no cases of "peel" and only 1 case of CGS (3.0%). This infant had a single atresia associated with a very small (1 cm) defect in the abdominal wall and no labor-induced "peel," which was easily and successfully repaired by resection and anastomosis. The 6.4-fold reduction in the occurrence of CGS by PTPL C-section (3.0% vs 19.1%) was statistically significant by the chi-square test (P < 0.05), as was the 100% elimination of the disabling "peel." If the single case of CGS associated with a very small defect and no labor or labor-associated "peel" is eliminated, the incidence of CGS in the remaining PTPL group of 32 cases falls to 0 (0% versus 19.1%, P < 0.007). PT and especially PL C-section may be expected to virtually eliminate "peel" formation and CGS and to remove GS as one of the four major causes of SGS. The findings of this report that PT labor prior to PT C-section may result in both "peel" formation and CGS further solidifies the role of labor in the production of both the "peel" and the equally disabling CGS. Failure to appreciate the central role of labor in GS complications has doubtless contributed to the persistent controversy concerning the value and importance of PTPL C-section for gastroschisis diagnosed in utero. The pediatric surgeon has an important responsibility with the obstetrician to monitor the possible occurrence of occult labor in the waning weeks of pregnancy and be prepared to do a prompt C-section if it occurs and there is adequate lung maturity. The achievement of "peel"- and CGS-free gut would greatly facilitate the use of the new Bianchi technique of gut reduction without anesthesia. The combination of the use of epidural anesthesia for the elective PTPL C-section with the Bianchi approach would spare both mother and baby any untoward effects of general anesthesia and present the potential for massive reductions in hospital costs with minimal patient manipulation and disturbance. For infants born with labor-associated "peel," re-evaluation of the suitability and effectiv

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Developmental anomalies of the pancreas and its ductal drainage system are an important cause of pancreatitis. Patients with such anomalies require appropriate surgical intervention. This report concerns an uncommon foregut anomaly that causes pancreatitis. A 9-year-old girl had recurrent pancreatitis secondary to a gastric duplication communicating with an aberrant pancreatic lobe. Appreciation of the relevant anatomy led to successful surgical management of the pancreatitis.

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The posterior sagittal anorectoplasty (PSARP) has become the primary surgical procedure for patients with high imperforate anus. Very few careful long-term follow-up studies have established the superiority of the PSARP procedure over other surgical repair techniques. The authors' goal was to evaluate the operation and to identify the factors associated with improved continence. The medical records, operative reports, and radiographs of 53 patients (46 male, 7 female) who underwent PSARP for high imperforate anus at Children's Hospital and Medical Center (CHMC) between 1982 and 1990 were reviewed retrospectively. Subjective follow-up data were collected by telephone questionnaire, assessing habits indicative of stool continence, and a "fecal continence score" (FCS) was calculated for each patient. A prospective, 7-day diary assessing similar patterns of fecal continence was completed by each patient's family. The mean age of the patients studied was 8.0 years, and the mean follow-up period was 6.8 years. The telephone questionnaire was completed for 48 patients (94%). Toilet training for bowel continence was successful in 20 patients (42%) and occasionally successful in another 20 patients (42%), but 8 patients (16%) had no awareness of impending stool. Forty-five (94%) were physically active, but 16 (33%) reported social problems related to offending odor. The mean fecal continence score for all patients was 3.0 +/- 1.4 (5=excellent, completely continent), which was nearly identical to published scores for the other types of surgical repair. The FCS did not improve with age. The parents' responses to the telephone questionnaire matched the results obtained from the prospectively collected continence diary data. The authors' results for PSARP in patients with high imperforate anus do not differ substantially from those achieved by other repair techniques. Previously cited prognostic factors such as fistula anatomy and sacral anomalies did not appear to alter the functional results in the authors' series. Aggressive postoperative bowel management should be anticipated in all patients who have high imperforate anus and may benefit those who otherwise would achieve less satisfactory continence.

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Giant omphalocele is a major clinical challenge for pediatric surgeons. Whereas small- to medium-sized defects can be repaired primarily, larger omphaloceles cannot be closed at birth because the liver and small bowel have lost the right of domain to the abdomen. Two divergent strategies have evolved for treating these giant defects: (1) use of a silon chimney with gradual reduction of the contents of the sac, and (2) initial nonoperative management (epithelialization) of the omphalocele followed by repair of the residual ventral hernia. In an 18-year retrospective study, we have reviewed our experience with these treatment methods. Ninety-four infants underwent treatment for omphalocele between 1975 and 1993. Primary closure (PC) was possible in 55 patients, silon chimney (SC) was used in 15, and 7 had nonoperative management (NM) with epithelialization. In the remaining 17 infants, surgery was believed to be inappropriate because of the lethality of their associated anomalies. Major (but potentially survivable) anomalies were present in 26% of PC, 13% of SC, and 71% of the NM group patients. The majority of the liver was present in 73% of SC- and 86% of NM-treated omphaloceles. There was a decrease in length of stay, time to enteral feeding, and mortality over the 18-year period. However, those patients whose defects could not be closed primarily had consistently longer hospital stays. This was particularly true for the SC patients. The decreased use of total parenteral nutrition seems to reflect a shift from SC to NM rather than a decrease in the interval to full enteral feeding in any given treatment group over time.(ABSTRACT TRUNCATED AT 250 WORDS)

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An 8-year review of neonates with left-sided congenital diaphragmatic hernia who presented in severe respiratory distress at birth was retrospectively reviewed looking at stomach position, thoracic or abdominal, as a predictor of survivability. Survival was 93% with the stomach below the diaphragm compared with 29% when the stomach was thoracic in location. Stomach position may be an important early in utero predictor of high mortality in neonates with left-sided congenital diaphragmatic hernias.

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Escherichia coli O157:H7 is most commonly linked to hemorrhagic colitis and the hemolytic uremic syndrome. Diagnostic ultrasound was used to demonstrate terminal ileum abnormalities suggestive of bacterial ileocecitis, a recently described entity that resembles acute appendicitis, in a patient whose stool culture yielded E. coli O157:H7. This case extends the spectrum of disease caused by E. coli O157:H7 and expands the number of organisms that can cause bacterial ileocecitis.

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Controversy regarding the management of inguinal hernias in young infants stimulated this study. It was our hypothesis that inguinal hernia in young infants can and should be repaired at the time of presentation. In order to address this issue, the following questions were examined: How frequently are these hernias incarcerated and how should this be managed? What is the best timing of repair? Should the asymptomatic contralateral groin be explored? How do the recurrence and complication rates compare with those in older children? Have advances in anesthesia affected the treatment of these infants? The records of 384 infants less than 2 months of age who underwent inguinal herniorrhaphy from January 1985 to January 1990 at Children's Hospital and Medical Center in Seattle were reviewed. Nearly one fourth (24%) of the patients had incarcerated hernias. Preoperative reduction was successful 96% of the time. The hernia was then repaired within 48 hours. Six patients required urgent operation, but bowel resection was not necessary in any. Contralateral groin exploration was performed 96% of the time and was positive in 85%. The recurrence rate was 1.0%. Complications occurred in 2.3% of patients. There were no hernia-related deaths. Spinal anesthesia was used in 63 patients with no postoperative apnea. An aggressive approach toward prompt repair of hernias in this age group can result in a very low incidence of hernia-related complications. Repair can be carried out safely in the first 2 months of life with recurrence and complication rates comparable with those in older children. Spinal anesthesia may lower the rate of postoperative apnea.

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Necrotizing enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. A 5-year review of NEC at Children's Hospital and Medical Center identified the unique features of this disease in the term neonate. Eighty-one patients with NEC were treated between January 1984 and May 1989. Ten full-term neonates with gestational age greater than 38 weeks were identified for study. Charts were reviewed for recognized risk factors, clinical course, surgical intervention, and outcome. Ninety percent had a birth weight greater than or equal to 2.7 kg, and all were above 2.1 kg. NEC developed early in this group, with onset of disease in the first 48 hours of life in 50% of the group and within the first 4 days of life in 90%. The recognized risk factors of asphyxia, hypoglycemia, polycythemia, and respiratory distress were absent in 60%. Seven of 10 patients required exploratory laparotomy, whereas 3 of 10 required only medical treatment. Indications for operation were perforation in three patients, peritonitis in three patients, and mass in one patient. All patients requiring operations had severe colonic disease, with perforation of the colon in five of seven and full-thickness necrosis without perforation in two of seven. Two patients required total abdominal colectomy. Only one patient with perforated meconium ileus and associated NEC had small bowel involvement. This patient was the only mortality of the group. Subsequent intestinal continuity was restored in all surviving patients with no late complications. Two patients required resection of additional NEC strictures prior to reanastomosis. Of the three medically treated patients, none required subsequent operation for colonic stricture. Our experience indicates that the presentation, clinical course, and operative findings in full-term neonates with NEC differ from those encountered in the premature infant with NEC.

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One hundred fifty-six children younger than 13 years of age with blunt abdominal injuries were reviewed, and those injured in accidents (89%) were compared with those injured by child abuse (11%). Abused children were younger (mean age, 2 1/2 years) and all presented late to medical attention with a history that was inconsistent with their physical findings. Only 65% of abused children had physical or roentgenographic signs of prior abuse, while 35% had no signs of prior abuse. The abdominal organs injured were different; 61% of accidentally injured children suffered injuries to a single, solid organ, and only 8% had hollow viscus injuries, while 65% of abused children had hollow viscus injuries. Physicians should suspect child abuse when children have unexplained injuries (especially young children with hollow viscus injuries) even when other signs of child abuse are absent, and they should suspect hollow viscus injury in abused children.

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Atresia of the colon represents the least common cause of neonatal intestinal obstruction, comprising less than 10 percent of gastrointestinal atresias. Eleven patients underwent surgical repair of colonic atresia during a 5 year period. Five of the patients were premature. Two had associated gastroschisis and other congenital anomalies were present in three patients. Hypaque enema was utilized to diagnose intestinal atresia and laparotomy was then undertaken. Primary repair could be performed in four patients, one with type I and three with type 3 atresias. These four patients did well and were discharged within 13 days after operation. Three deaths occurred in patients with associated anomalies, yielding a mortality rate of 28 percent. Operative therapy of colonic atresia should be individualized, based on the condition of the patient at presentation and the presence of associated birth defects.

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Forty-six neonates with omphaloceles seen at the Children's Hospital and Medical Center in Seattle from 1975 to 1985 were reviewed. There was an 87 percent survival rate in those surgically managed. The 23 neonates who underwent primary closure all survived. The 13 neonates with giant omphaloceles with the liver in the defect who received silon chimneys had a 46 percent mortality rate and a high complication rate, with prolonged hospitalization. Two neonates with giant omphaloceles were managed by leaving the sac intact, and silver sulfadiazine cream was used as an escharotic agent. We believe it is a safer alternative than the silon chimney in neonates whose defects cannot be closed primarily.

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Mesenchymal hamartoma of the chest wall is a rare lesion that presents as a chest wall mass. Four infants are reported, three of whom underwent extensive chest wall resection.

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The unusual problem of multiple intestinal atresias associated with jejunal atresia has been reviewed and seven cases have been summarized. Maintaining maximal bowel length without jeopardizing the patient is of utmost importance. The shish kebob technique has been illustrated in a patient with 15 obstructions. The other cases include one patient with the apple peel deformity and two with atresias associated with gastroschisis. Although these children are small in general, they are bright and otherwise healthy. The 90 percent mortality reported previously has been reversed by a combination of techniques and lessons learned from the leaders in pediatric surgery.

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Twenty-four cases of fetal gastroschisis referred between 1982 and 1984 were evaluated. The seven infants delivered by cesarean section all had primary closures and an average hospital stay of 18 days. Of the 17 infants delivered via the vaginal route, there were three deaths; primary closure was only possible in 11 and the average hospital stay was significantly prolonged. Until a large multicenter, prospective randomized study can be accomplished, the present data support the use of cesarean section for delivery of infants with gastroschisis.

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Evaluation of abdominal pain in children poses a major challenge for the pediatrician and pediatric surgeon alike. Simple appendicitis remains one of the most difficult diagnoses in children. There has been an alarming increase in the incidence of perforated appendicitis, and professional delay has been found to be a factor in this increased rate. Therefore, this discussion focuses on appendicitis and its differential diagnosis.

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A retrospective study was performed to identify factors associated with perforation in 150 children with acute appendicitis. The children's parents were interviewed about the nature and timing of care, family history of appendicitis, and history of abdominal pain episodes, and the children's medical records were reviewed. Delay in treatment--the interval between first recognized symptoms of abdominal pain and surgery--was most predictive of perforation. A treatment delay of more than 36 hours was associated with a 65% or greater incidence of perforation. Mean delay for the group with perforation of the appendix was 66.7 hours compared with 35.8 hours for the group having appendicitis without perforation (P less than .01). Mean professional delay was significantly longer in the group with perforated appendicitis than in the group having appendicitis without perforation (P less than .01), but mean parental delay was not. Children aged 1 to 4 years and those aged 5 to 8 years had a 74% and 66% incidence of perforation, respectively, compared with a 30% to 42% incidence in older children (P less than .01). Age had a significant effect upon perforation even when adjusted for delay in treatment. Other factors associated with perforation were family history of appendicitis, social class, advice given by the first health professional contacted, and the presence of fecaliths. When all factors were considered simultaneously by using logistic regression techniques, delay in treatment, age, and absence of a family history of appendicitis were all significant predictors of perforation.

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Appendicitis remains the most common intraabdominal indication for surgery in children. Recently, barium enema has been helpful in increasing the accuracy of diagnosis in difficult cases. Experience with 202 barium enemas in children with abdominal pain entertaining the diagnosis of acute appendicitis revealed some of the problems encountered in using this procedure as a diagnostic tool. Of the 66 children who underwent surgery, there were 32 children who were felt to have a positive barium enema and 31 of these turned out to have acute appendicitis. In the group that had negative barium enemas, there were 3 false negatives and 2 of these children had early perforations by the time of their surgery. The third group were equivocal barium enemas and they pointed out the difficulty in obtaining a normal barium enema in a child who does not have appendicitis. It was felt that the use of barium enema as an adjunct was helpful especially if it were positive, but a negative barium enema could not be relied upon to delay surgery in the child with right lower quadrant peritoneal signs.

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Until alternate diagnostic methods are discovered, the staging procedure seems to be the most reliable method to establish the presence or absence of abdominal involvement in Hodgkin's disease. Our experience with staging laparotomy in 22 children raises serious questions as to both the risk of operation and the prognostic value of a negative abdominal exploration. Routine use of the staging laparotomy may not be justified in clinical Stage IA patients with lymphocyte-predominant cell type. Because of the hazards and limitations of the staging procedure, vigorous attempts would seem to be indicated to identify subcategories of patients in whom the likelihood of intraabdominal involvement is so small as to negate the value of surgical staging.

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