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Objective The objective was to present a case series of pediatric patients presenting with small bowel obstruction secondary to both congenital and acquired internal mesocolic hernias, and the use of imaging technology in the management of this condition. Methods A retrospective review of patients treated at the Yale–New Haven Children's Hospital for small bowel obstruction from 1998 to 2008 (n = 6) who presented with acute small bowel obstruction secondary to internal mesocolic hernias was performed. Results We present 6 patients with small bowel obstruction caused by congenital (n = 4) and acquired (n = 2) mesocolic hernias after previous surgery. The median age at presentation was 13 years. Small bowel obstruction with a mesocolic hernia was identified by preoperative abdominal computerized tomography in 3 patients (50%) and at operation in the others. The mean length of stay was 6 days, with no recurrent episodes in the follow-up period. Conclusion Small bowel obstruction secondary to mesocolic hernias, although rare, may be considered in the differential diagnosis of patients with history of malrotation or abdominal wall defects owing to their association with congenital mesenteric anomalies. This condition requires special attention from the clinician because of its catastrophic consequences. Imaging studies are an important asset because of the difficulty in making an accurate clinical diagnosis and the rarity of internal hernias.

Small bowel volvulus in the absence of a congenital anomaly is rare but has been described in post-operative patients after abdominal surgery and is usually related to inflammatory conditions. The authors describe an adolescent girl with small bowel volvulus secondary to an oral fiber bolus after laparoscopic appendectomy that required emergency laparotomy and reduction. She subsequently developed early post-operative complete bowel obstruction requiring re-laparotomy and resection.

We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood.

A girl 5 years 11 months of age, belonging to an extensive kindred with multiple endocrine neoplasia, type IIA (MEN IIA), was found to have multifocal medullary thyroid carcinoma with metastasis in one paraglandular lymph node after positive findings on a calcium-pentagastrin stimulation test. Her sister, 3 years 8 months of age, also had an elevated calcitonin level, and thyroidectomy revealed C-cell hyperplasia and a focus of medullary thyroid carcinoma. These two cases underscore the need for prophylactic thyroidectomies in MEN IIA patients as young as 5 years of age and strict yearly provocative screening beginning at age 1 year.

Study Objective: To elucidate risk factors for apnea in preterm infants discharged from the hospital and in full-term healthy infants. To determine the efficacy of real-time cardiopulmonary monitoring versus computerized storage and retrieval for infants at risk. Study Design: Prospective study. Setting: Operating rooms and pediatric patient care units of university medical center. Patients: 27 preterm infants and 20 full-term infants no more than 60 weeks' postconceptional age, who were admitted for elective herniorrhaphy. Interventions: Infants were monitored before and after herniorrhaphy with general anesthesia using an infant apnea impedance monitor, pulse oximetry, and nursing observation. Measurements and Main Results: Demographic information and medical history were correlated with postoperative apnea. The sensitivity and specificity of nursing observation and oximetry were compared with computerized apnea monitors. Five patients (11%, four preterm, one full-term) were apneic postoperatively as recorded by computerized pneumocardiography. Previous apnea history, gestational age at birth, and postconceptional age at operation positively correlated with postoperative apnea. Nursing observation failed to detect 4 of 5 patients with documented apnea (sensitivity 20%, positive predictive value 50%). Pulse oximetry failed to detect 3 of 5 patients with apnea (sensitivity 40%, positive predictive value 66%). Conclusions: Although it is easier to predict postoperative respiratory dysfunction in previously sick or very young infants, absolute predictability for all neonates remains elusive. Clinical monitors with both storage and retrieval capabilities and real-time monitoring increase our ability to detect significant events in children at risk for apnea after herniorrhaphy.

The risk of postoperative reflux and pulmonary aspiration with straight colon or gastric tube esophageal replacement in children prompted us to reevaluate the presumed antireflux role of the ileocecal valve with retrosternal ileocolic interposition. This operation was done in eight patients with esophageal atresia (six) and lye stricture (two) from 19 to 50 months of age between 1983 and 1992. There were no operative deaths. The duration of follow-up ranged from 4 to 115 months. Barium swallow obtained in all patients showed unobstructed esophagoileocolic transit without reflux. Two patients with esophageal atresia had localized proximal anastomotic leaks, which healed spontaneously without stricture. In the two patients with lye ingestion ileoesophageal strictures developed that necessitated revision. None of the patients had postoperative respiratory complications or symptomatic gastroesophageal reflux. All eight children have had their gastrostomy tubes removed, are eating a regular diet, and are growing well. In conclusion, the retrosternal ileocolic conduit provides an excellent substitute esophagus in selected pediatric patients, with potential advantages over delayed primary anastomosis or the straight colon or gastric tube interposition because of the antireflux role of the ileocecal valve.

A total of 116 cases of intestinal atresia or stenosis were encountered at the Yale-New Haven Hospital between 1970 and 1990. Sites involved were the duodenum (n = 61; 53%), jejunum or ileum (n = 47; 46%), and colon (n = 8; 7%). All but two patients underwent operative correction, for an overall survival rate of 92%. Challenging problems were the management of apple-peel atresia (five patients), multiple intestinal atresia with short-gut syndrome (eight patients), and proximal jejunal atresia with megaduodenum requiring imbrication duodenoplasty (four patients). Major assets in the improved outlook for intestinal atresia are prenatal diagnosis, regionalization of neonatal care, improved recognition of associated conditions, innovative surgical methods, and uncomplicated long-term total parenteral nutrition.

There is a significant incidence of inguinal hernia in premature infants and the optimal timing of repair is controversial. A high rate of postoperative respiratory complications has been reported in this group. In this study, the records of 47 premature infants (mean gestational age, 30.3 weeks) who underwent herniorrhaphy while still in the neonatal intensive care unit were reviewed in an effort to define those conditions that are independent risk factors for complications. Forty-three percent of infants had complications, including postoperative assisted ventilation (34%), episodes of apnea and/or bradycardia (23%), emesis and cyanosis with first feeding (6%), and requirement for postoperative reintubation (4%). Although low gestational age and postconceptual age at operation, low birth weight for gestational age, and preoperative ventilatory assistance were significantly associated with postoperative complications, only a history of respiratory distress syndrome/bronchopulmonary dysplasia (odds ratio 2.3), a history of patent ductus arteriosus (odds ratio 2.5), and low absolute weight at operation (odds ratio 3.5 for 1,000-g decrease) were independent risk factors for postoperative complication. Despite previous reports citing postconceptual age as the factor having the greatest impact on postoperative complications, these results indicate that a history of respiratory dysfunction and size at operation may be more important predictors of postoperative respiratory dysfunction in preterm infants.

Congenital diaphragmatic hernia (CDH) is associated with multiple congenital anomalies affecting several organ systems, including the gastrointestinal system. Pyloric stenosis and bands are known and previously reported etiologies of gastric outlet obstruction in infants with CDH. We report the first case of gastric antrum hypertrophy causing gastric outlet obstruction in an infant with CDH.

We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood.

End-to-side anastomosis (ES) and ligation of the tracheoesophageal fistula (TEF) has been the procedure of choice for esophageal atresia at our institution since 1967. This report summarizes our operative and long-term results with the ES operation in 68 babies, including 33 in Waterston group A (50%), 23 in group B (35%), and 12 in group C (15%). An additional 10 patients had a primary end-to-end (EE) anastomosis, while 14 others required either staged EE repair or an esophageal replacement procedure. Overall survival rate with ES was 93% including two deaths attributed to major anastomotic leaks and sepsis, and three others in group C from cardiac anomalies. Six (9%) of those having ES anastomosis developed a recurrent TEF between 40 days and 21 months of age, necessitating reoperation. Predisposing factors to recurrent TEF were surgical inexperience (three cases; first operation for each surgeon), forceful vomiting secondary to gastroesophageal reflux (GER) in two, and drug overdose in one. Anastomotic leak occurred in seven (10%) following end-to-side repair and was implicated in two deaths. Three patients developed minor anastomotic stricture requiring less than three dilatations, while one with a tight stricture needed as many as five dilatations over the first 14 months of life. Mild dysphagia and respiratory symptoms were uniformly observed during the first year, but only five patients (7%), including the two with recurrent TEF, required fundoplication for persistent GER. All patients were eating table foods after 1 year of age, while 10 (15%) have required periodic endoscopic removal of solid food lodged at the radiographically unobstructed anastomosis. Eight patients in Waterston groups B and C remain below the fifth percentile in weight. It is concluded that the principal advantages of ES over EE repair are a lesser incidence of anastomotic leak, stricture, and GER with a similar incidence of recurrent TEF usually reported for EE repair. The risk of recurrent TEF is minimized with greater operator experience and prompt correction of severe GER by fundoplication. ES repair remains the favored operation for esophageal atresia at this institution.

The value of performing Nissen fundoplication in neurologically impaired children is a controversial issue. To evaluate the benefit of fundoplication in these children, hospital records were reviewed for 77 children who underwent fundoplication for gastroesophageal reflux (GER). Fifty-two children were neurologically impaired; 25 children had no neurological impairment. Impaired children had significantly fewer hospital admissions (1.8 v 0.7; P less than .005) and total days of hospitalization (36 v 14; P less than .005) during the first postoperative 6 months, compared with the immediate preoperative 6-month period. Normal children had fewer hospital admissions and days postoperatively, but the difference was not significant. Impaired children with preoperative failure to thrive (FTT had significantly increased average monthly weight gain over the first 6-month period postoperatively, compared with preoperative growth rate (3.0% v 0.9% of total body weight; P less than .05). Average monthly weight gain at 1 and 2 years postoperatively was not significantly different from preoperative values for impaired children. Growth rate of normal children with FTT did not change significantly postoperatively. Symptomatic relief was comparable in the normal and impaired children. Perioperative mortality was 0% in the normal children and 6% in the impaired children. This study demonstrates that Nissen fundoplication in neurologically impaired children with GER can be performed safely, reduces the frequency of hospitalization, and improves short-term weight gain.

Juvenile polyps of the colon and rectum traditionally have been viewed as being benign inflammatory or harmartomatous lesions without potential for malignant change. The authors report a case of adenocarcinoma developing in a patient with sporadic juvenile polyposis. Juvenile polyposis was diagnosed in the patient at age 4 years. He underwent subtotal colectomy at age 6 years. At age 12, he underwent a proctectomy and a Swenson pull-through because of adenomatous changes in the rectal stump. At age 19 surveillance endoscopy revealed invasive cancer in a juvenile polyp.

Primary epithelial tumors of the pancreas are extremely uncommon in children, and among these, acinar cell carcinoma (ACC) is the most rare. Here we describe our recent observations in the case of a 10-year-old boy with one of these exceptional examples. The histologic diagnosis of ACC was supported by both immunohistochemistry and electron microscopy. Despite its rarity, ACC should be kept in the differential diagnosis of pediatric pancreatic exocrine tumors. We also provide a comparison with an example of solid pseudopapillary tumor, another relatively infrequent epithelial tumor of the pancreas in the young. We review the relevant literature addressing the clinical and pathologic features of ACC and its distinction from other pancreatic neoplasms.

Purpose Intestinal rotation abnormalities and complex congenital heart disease associated with heterotaxia coexist. Despite the risk for midgut volvulus, performing a Ladd procedure for asymptomatic malrotation with heterotaxia remains to be controversial because the presumed risk for postoperative complications is thought to exceed the benefits of the operation. The purpose of this study was to review the incidence of complications after a Ladd procedure in asymptomatic patients with heterotaxia to guide recommendations for this patient population. Methods The medical records of all patients with heterotaxia who underwent a Ladd procedure for asymptomatic malrotation between 1984 and 2004 were reviewed. Type of cardiac disease, postoperative complications, and survival were recorded. Results Twenty-two patients (9 boys and 13 girls) with heterotaxia underwent an elective Ladd procedure after their medical stabilization or surgical correction or palliation of their cardiac anomaly. Of these patients, 19 were younger than 1 month at the time of the operation. The remaining 3 patients underwent the operation when they were between 2 and 5 months old. Three of the 22 patients (14%) developed postoperative intestinal obstruction: lysis of adhesions was performed in 1 patient; another patient required a staged bowel resection for a closed loop obstruction; and yet another patient had recurrent midgut volvulus 4 years after an incomplete initial Ladd procedure. All patients survived the initial and secondary procedures. Four deaths, all more than 1 month after the surgery, occurred as sequelae of the underlying cardiac anomaly. Length of follow-up ranged from 1 to 17 years. Conclusions We report on a 14% risk of postoperative bowel obstruction after an elective Ladd procedure, as compared with a small but significant incidence of midgut volvulus in patients with malrotation in the setting of complex congenital heart disease. Our results support the conclusion that an elective Ladd procedure at a time of relative cardiac stability for selected patients with heterotaxia has an acceptably low morbidity and should be considered to prevent midgut volvulus.

Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 18-weeks-gestation. Findings included enlarged lungs, inverted diaphragms, dilated trachea distal to the obstruction, and ascites. At 35 weeks' gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed, allowing bronchoscopic evaluation of the airway and placement of a tracheostomy. The postnatal course was marked by gradual recovery of diaphragmatic function with corresponding weaning from conventional ventilation to positive airway pressure. The sustained improvement in diaphragmatic and pulmonary function after the EXIT procedure for laryngeal atresia is one of the first reported for this entity and provides encouragement for future attempts.

Treatment of small postoperative interloop abscesses (ILA) can be challenging. In children, these collections have usually been drained surgically at a second operation. This article describes three children with small postoperative ILAs who were treated by percutaneous needle aspiration and parenteral antibiotics, with good outcomes. The advantages of this technique and its utilization in the management of children with ILAs are discussed.

Objective. Children with a question of occult head injury are routinely hospitalized despite having both normal central nervous system (CNS) and computed tomographic (CT) scan examinations. We determined the incidence of significant CNS morbidity after occult head injury to determine whether or not hospital admission was necessary in children after minimal head trauma. Methods. We reviewed the records of children admitted to a level I trauma center with a question of closed head injury, an initial Glasgow Coma Scale equal to 15, a normal neurologic exam, and a normal head CT scan. Children with associated injuries requiring admission were excluded. The endpoints were deterioration in CNS exam, new CT findings, and the need for a prolonged hospital stay. Results. Sixty-two patients were studied with a mean age of 7 years (range, 1 month to 15 years), and 65% were male. The primary mechanisms of injury were fall (45%) and vehicular crash (23%). The mean injury severity score was 4 ± 2. The mean length of stay was 1.2 days (range, 1 to 3 days). Prolonged hospitalization occurred in 9 patients (15%). No child developed significant CNS sequelae warranting hospital admission. Total charges for these hospitalizations were $177 874. Conclusions. Children undergoing emergency department work-up of occult head injury, who have a normal CNS exam and a normal head CT scan, do not seem to be at risk for significant CNS sequelae. These patients can be discharged home with parental supervision and avoid unnecessary and costly hospitalization.

Purposes of this study were: 1) to compare mortality and postoperative morbidities (intra-abdominal abscess, wound dehiscence, and intestinal stricture) in extremely low birth weight (ELBW) infants who underwent initial laparotomy or drainage for necrotizing enterocolitis (NEC) or isolated intestinal perforation (IP); 2) to determine the ability to distinguish NEC from IP preoperatively and the importance of this distinction on outcome measures; and 3) to evaluate the association between extent of intestinal disease determined at operation and outcome measures.ELBW infants who undergo operation for NEC or IP have a postoperative, in-hospital mortality rate of approximately 50%. Whether to perform laparotomy or drainage initially is controversial. Also unknown is the importance of distinguishing NEC from IP and the current ability to make this distinction based on objective data available prior to operation.A prospective, multicenter cohort study of 156 ELBW infants at 16 neonatal intensive care units (NICU) within the NICHD Neonatal Research Network.Among the 156 enrolled infants, 80 underwent initial peritoneal drainage and 76 initial laparotomy. Mortality rate was 49% (76 of 156). Ninety-six patients had a preoperative diagnosis of NEC and 60 had presumed IP. There was a high level of agreement between the presumed preoperative diagnosis and intraoperative diagnosis in patients undergoing initial laparotomy (kappa = 0.85). The relative risk for death with a preoperative diagnosis of NEC (versus IP) was 1.4 (95% confidence interval, 0.99-2.1, P = 0.052). The overall incidence of postoperative intestinal stricture was 10.3%, wound dehiscence 4.4%, and intra-abdominal abscess 5.8%, and did not significantly differ between groups undergoing initial laparotomy versus initial drainage.Survival to hospital discharge after operation for NEC or IP in ELBW neonates remains poor (51%). Patients with a preoperative diagnosis of NEC have a relative risk for death of 1.4 compared with those with a preoperative diagnosis of IP. A distinction can be made preoperatively between NEC and IP based on abdominal radiographic findings and the patient's age at operation. Future randomized trials that compare laparotomy versus drainage would likely benefit from stratification of treatment assignment based on preoperative diagnosis.

Hypothesis End-to-side repair (ES) with ligation of the tracheoesophageal fistula (TEF) reduces the risks of stricture and gastroesophageal reflux disease requiring operation compared with the end-to-end repair of esophageal atresia and distal TEF. Design Case series with institutional and historical control subjects. Setting Referral children's hospital. Patients One hundred thirty-four infants diagnosed as having esophageal atresia and distal TEF between June 30, 1968, and July 1, 2003. Interventions Ninety-six infants having ES and 38 having end-to-end repair. Main Outcome Measures Patients were studied for overall survival, surgical complications, and well-being during the first year of life. Results Survival was 95% vs 90% (patients undergoing ES vs end-to-end repair). Complications included anastomotic leak, 8% vs 13%; recurrent TEF, 7% vs 3%, with only 1 recurrence in the last 28 patients having ES; anastomotic stricture (requiring dilatation), 5% vs 13%; gastroesophageal reflux disease requiring operation, 6% vs 18%; and esophageal dysmotility, which was present following nearly all ES and end-to-end procedures. Tracheomalacia-related respiratory symptoms following ES decreased from 50% to 11% at 1 year of age. Age-appropriate diet following ES was achieved in 93% by 1 year; 5% experienced occasional dysphagia or choking episodes. Conclusions The ES operation is accompanied by a reduced rate of stricture and gastroesophageal reflux disease requiring operation compared with end-to-end repair. Earlier concerns regarding an unacceptable risk of recurrent TEF were not substantiated.

fetus in fetu (FIF) is a rare anomaly of fetal development in which a pedunculated, fetiform mass develops inside a more mature fetus, 1,2 Minimal criteria for diagnosis include the presence of an axial skeleton or a fetus with metameric organization, skin coverage, encapsulation, and a 2-vessel cord. 3 In most cases that meet diagnostic criteria, the FIF is poorly developed and may be difficult to distinguish from a teratoma. The etiology of this condition is unknown. Although the diagnosis of an FIF has traditionally been made in infancy or childhood, there are an increasing number of diagnoses being made prenatally on the basis of sonography. 1,4-14 This report describes the prenatal diagnosis of an FIF with an extraordinarily high degree of differentiation using two-dimensional (2D) and three-dimensional (3D) sonographic imaging.

The superiority of computed tomography (CT) for detection of lung nodules has been documented and attempts have been made to distinguish benign from malignant lesions in adults. We attempted to characterize lung nodules in 12 children with solid malignant tumors (aged 8 months to 17 years) in an effort to differentiate benign from metastatic disease. All scans were performed at 10-mm contiguous intervals on a GE 9800 CT scanner. The scans were retrospectively viewed by two pediatric radiologists independently and without knowledge of the pathological findings. All biopsies were done via open thoracotomy. The CT findings were correlated with pathology results. Twelve children had 13 nodules biopsied. Six of these showed malignancy, two showed inflammatory changes, and two had a reactive subpleural lymph node. In three children, no abnormality was found and a biopsy was not obtained. One child had a metastatic nodule in one lung, and a simultaneous inflammatory nodule in the other. The radiologists agreed with each other on the CT interpretation in 11 of 13 surgically explored areas. They correctly predicted malignancy in four cases and correctly excluded it in two cases. However, they were simultaneously incorrect in five instances. Our conclusion is that, contrary to reports in adults, a tiny nodule may be either benign or malignant. Malignancy cannot be separated from benign disease by CT established criteria.

Air rifles, or BB guns, are generally thought of as childhood toys. Although most injuries are not serious, life-threatening events have been reported. Within a 1-year period, 3 boys presented after BB gun shots to the chest, all requiring surgical intervention for penetrating injuries to the heart. A 15-year-old underwent window pericardiotomy for hemopericardium with thrombus 24 hours after admission. Another, 5 years of age, underwent emergent exclusion of the cardiac apex for a traumatic ventricular septal defect. The third, 8 years old, had a right ventricular injury requiring an urgent subxiphoid pericardial window for tamponade. All recovered uneventfully. Increased public awareness, adult supervision, safety training, and appropriate legislation are needed to decrease the risks of these potentially lethal weapons.

A case of acquired diaphragmatic paralysis in an extremely low birth weight infant complicated by respiratory failure, recurrent atelectasis, and pneumonia is described. Diaphragmatic plication led to a rapid improvement in pulmonary function and allowed for discontinuation of mechanical ventilation in less than 1 week. Therapeutic options for acquired diaphragmatic paralysis, including the rationale for early operative intervention, in this patient population are discussed.

Lower pole splenectomy with preservation of residual splenic function was successfully performed in a 3-year-old boy with a symptomatic splenic hamartoma. Following resection, several of the patient's constitutional symptoms resolved. This is the first reported case of a splenic hamartoma treated by partial splenectomy.

Purpose : The presence of squamous epithelium in the cyst wall of children with large splenic cysts has generally been thought to require complete resection of the cyst lining to prevent recurrence. The risks of major bleeding or possible total splenectomy necessitates a simplified procedure to preserve splenic function without cyst recurrence. Methods : Six patients treated for splenic cyst over a 10-year period underwent partial splenic decapsulation retaining the hilar portion of the spleen. The patient group, including five girls, ranged in age from 6 to 18 years (mean, 13 years). Presenting findings were splenomegaly (n = 4), progressive cyst enlargement (n = 4), left upper quadrant pain (n = 2), ultrasound for unrelated condition (n = 2), and prior history of trauma (n = 1). Cyst location varied from peripheral to deep cortical and ranged in size from 5 to 20 cm containing from 200 to 1,700 mL of sterile straw-colored to dark brown-colored liquid. Results : Palpable splenomegaly resolved in all patients with progressive decrease in size of the splenic remnant, and return of vascularity to normal was confirmed by Doppler ultrasound. Each patient remains asymptomatic without recurrence of the splenic cyst from 1 to 10 years after surgery. Squamous epithelial lining cells found in sheets and clusters were seen within the cyst lining of all patients. Immunohistochemistry of the paraffin-embedded tissue showed that these cells expressed keratin, epithelial membrane antigen (EMA), carcino-embryonic antigen (CEA), but were negative for BerEP4 (conventionally positive in cells of epithelial origin). Conclusion : The authors conclude that splenic decapsulation is an effective treatment for splenic epithelial cysts, which both preserves splenic function and prevents recurrence despite retention of hilar cyst lining. Epithelial metaplasia of the mesothelial-mesodermal undifferentiated cells from exposure to an unidentified irritant may explain the histological presence of squamous epithelium within the cyst wall.

The twentieth century begins the modern era of pediatric surgery, a time of innovation and vastly improved operative survival. Until then, many correctable congenital anomalies had been described in case reports only as medical curiosities, seemingly having no chance for surgical cure, but the challenge was available to those wishing to accept. The first texts devoted to surgical diseases of infancy and childhood were descriptive, emphasizing visible and palpable lesions, tuberculosis, musculoskeletal deformity, and major trauma, but also recognized that "surgical diseases of children by their number, their variety, and their character must, we think, be studied in a special manner and demand a special chapter in surgical books." 4 The construction of Children's Hospitals during the nineteenth century identified the need to provide special facilities for children. Surgical antisepsis was practiced and general anesthesia used. Physicians such as Hirschsprung recognized a wide spectrum of treatable surgical conditions that would soon become the domain of pediatric surgeons. Indeed, at that moment in time at the very beginning of the twentieth century, Roentgen discovered the x-ray, Ladd received his MD degree at Harvard, the first pediatric appendectomies for appendicitis were successfully performed, and Fredet and Ramstedt recognized that pyloroplasty was not the preferred treatment for hypertrophic pyloric stenosis. Modern pediatric surgery had passed from embryo to fetus to newborn.

Objective: To identify risk factors for midgut volvulus (MGV) and to seek clues to early diagnosis of MGV in children with malrotation. Design: Retrospective patient series. Setting: Academic medical center. Patients: Sixty-eight consecutive children who had a Ladd operation performed between January 1970 and December 1991. Excluded were three patients whose records were unavailable and patients who had a Ladd operation during the course of repair of an abdominal wall defect or congenital diaphragmatic hernia. Results: Forty of 68 patients had MGV at operation. There was an inverse correlation between age at onset of symptoms and the probability of MGV: 85% (29/34) of patients less than 1 month of age had MGV compared with 43% (10/23) of older children. Patients who had symptoms for less than 4 days were more likely to have MGV (88%; 30/34) than patients who had more chronic symptoms (43%; 10/23). Bilious (green) vomiting was more highly associated with MGV (80%; 35/44) than nonbilious vomiting (38%; 3/8) or pain (50%; 6/12). Roentgenograms of the upper gastrointestinal tract were very accurate for the diagnosis of malrotation but frequently failed to identify MGV (sensitivity, 54%; 13/24). Despite the high proportion of MGV, only three patients had gangrenous bowel. Of these three patients, one died and two have short-gut syndrome. Conclusion: Neonates with a short history of bilious vomiting are most likely to have MGV-complicating malrotation, but older children who have chronic intermittent symptoms are also at risk. Since there is no way to predict which patients will develop catastrophic bowel necrosis, early diagnosis and operation are necessary to prevent mortality and short-gut syndrome. (Arch Pediatr Adolesc Med. 1994;148:43-46)

Permanent bile flow following hepatoportoenterostomy for biliary atresia is presumed to follow mucosal healing of the biliary tract to the intestinal epithelium, but the morphology of the anastomosis and the histology of the neo-biliary duct is not known. Experimental portoenterostomies were constructed in five normal 10-kg mini-pigs at approximately 3 months of age to study the healing of the anastomosis. The extrahepatic bile duct was resected in continuity with an en-bloc 1.5 × 0.3-cm segment of liver at the porta hepatis and biliary drainage was achieved with a Roux-en-Y jejunal limb. The bile duct remnant at the porta hepatis measured 3 mm in diameter. HIDA scan performed at 2 months showed prompt excretion of the radioisotope and normal function of the jejunal limb. Animals were killed at 2 weeks, 1 month, and 3 months following operation and the intact hepatobiliary anastomosis examined by routine histology and histochemical staining for acid and neutral mucins. At 2 weeks the biliary epithelial ducts were hyperplastic and showed evidence of cellular regeneration and cholangitis. By 1 month the biliary and intestinal epithelia were in close approximation. At 3 months the biliary-intestinal epithelium was healed, forming a funnel-like autoanastomosis with normal proximal biliary epithelium and distal intestinal epithelium. An intervening zone of hybrid biliary-intestinal epithelium showed basal crypts with characteristics of biliary epithelium, while the superficial glandular epithelium resembled intestinal villi. Mucin histochemistry of the hybrid epithelial lining revealed predominantly neutral mucins in the basal region of the mucosa characteristic of biliary mucosa. The luminal epithelium revealed a high acid mucin content, including goblet cells. A microvillus brush border, rich in neutral mucin, was also present apically on the luminal most cells. We conclude that: (1) hepatoportoenterostomy in normal mini-pigs is a reproducible model to study healing of the anastomosis; (2) a continuous, intact hepatobiliary-intestinal mucosa is present within 3 months following portoenterostomy; and (3) the neo-epithelium has a hybrid biliary-intestinal appearance. This study may have application to understanding the cause of cholangitis following portoenterostomy for biliary atresia.

The case presented is of an infant who developed a radial artery pseudoaneurysm following arterial puncture and was subsequently diagnosed with hemophilia. A discussion of radial artery pseudoaneurysms follows.

Surgery of Infants and Children , edited by Oldham et al, is a major text that emphasizes the "scientific principles and practice" of pediatric surgery while presenting the most available information on the diagnosis and treatment of the wide spectrum of clinical conditions encountered in this age group. The principal editors have been joined by 149 contributing authors "substantially younger than tradition would suggest for a text of this sort." Their work encompasses 1834 pages, 18 major sections, and 104 chapters. The editors have done exceptionally well in maintaining balance and consistency in style and language to make the book very readable. The text is a reference work "with a unique and contemporary view of pediatric surgery," one of the goals announced in the preface. The first section is devoted to the basic considerations required to understand the pathophysiology of surgical disease, while other major sections cover trauma, oncology

• Uncertainties remain about the frequency and need for diagnostic imaging following recovery from splenic injury with nonoperative management. To gain further understanding, the final appearance of the splenic roentgenographic image was evaluated in 20 consecutive children (mean age, 10.1 years) undergoing serial studies up to 70 weeks following injury. A total of 65 technetium 99m sulfur colloid scans, including 45 follow-up studies, were obtained and evaluated. By 20 weeks following injury, six patients (30%) were normal, four (20%) demonstrated minimal residual effects, and 10 (50%) had significantly improved, leaving some persistent abnormality. None of the patients in the last group showed any clinical problem. No distinctions could be made by comparing the severity of the initial injury with a persisting imaging defect. We conclude that clinical considerations alone should determine whether any follow-up imaging be performed in children recovering from splenic injury. ( AJDC . 1992;146:198-200)

Two newborn, low-birth-weight infants with traumatic perforation of the pharynx and an additional 18 previously reported cases are presented. Injury produced by pharyngeal suction catheters and nasogastric and endotracheal tubes is generally unrecognized until the baby develops signs of esophageal obstruction or radiographic evidence of pharyngeal perforation, usually in the posterior mediastinum. The catheter may enter the pericardial space. Conservative management with intravenous antibiotics and a feeding gastrostomy is favored, except in instances that require mediastinal decompression. The key to prevention is the use of soft-tipped suction catheters and nasogastric tubes and careful visualization of the cords during endotracheal intubation. The use of metal stylets to direct the endotracheal tube is condemned. Pediatrics, 56:1019-1022, 1977, PHARYNX, PERFORATION, SUCTION CATHETER, NASOGASTRIC TUBE, ENDOTRACHEAL TUBE, METAL STYLET.

Metabolic alkalosis is regarded as the "classical" electrolyte abnormality occurring with hypertrophic pyloric stenosis (HPS) but recent experience suggests that atypical electrolyte findings frequently occur and delay establishing the correct diagnosis. The records of 65 infants with HPS treated by pyloromyotomy during the past 4 years were reviewed to determine the serum electrolytes at the initial presentation. The four study groups formed included 8 (12.3%) patients in group A with serum bicarbonate (HCO3) below 18 mEq/L (mean 15.7 +/- 0.5 mEq/L); 19 (29%) in group B with HCO3 between 18 and 25 (22.9 +/- 0.3); 22 (33.8%) in group C with HCO3 between 25 and 30 (27.0 +/- 0.3) and 16 (24.6%) in group D with HCO3 over 30 (34.0 +/- 0.9). Established values for normal HCO3 in neonates is 20.1 +/- 2.5 (mean +/- SD). The mean values in group D for HCO3, potassium (4.0 +/- 0.18 mEq/L), and chloride (88.75 +/- 2.15 mEq/L) were each significantly different (p less than 0.001) from determinations of similar electrolytes in other groups. The duration of vomiting in group D of 10.5 +/- 1.3 days is almost double the time (p less than 0.001) in group A, and was associated with more severe dehydration, predominantly acid urine (pH less than 6), and ketonuria as compared to other groups. No significant difference in other demographic characteristics including the age at presentation, the gestational age, sex distribution, or types of formula used was observed. The results of the study emphasize that serum electrolytes in early HPS may be normal, that HCO3 is significantly lower than established normals for older children, and that the effects of hydrogen-ion loss elevating the serum HCO3 precedes alterations in other serum electrolytes.

A case is reported of a twenty-two-month-old child who has survived for seven years without recurrence after multimodal therapy of a rhabdomyosarcoma of the prostate. The patient was treated with preoperative irradiation, radical surgery, and intraoperative and postoperative actinomycin D.

A prospective plan for the nonoperative treatment of obstructing intramural duodenal hematoma was developed to (1) promptly establish the diagnosis and rule out transmural leaks by obtaining a contrast roentgenogram on admission and to subsequently study evolution of the obstructing intramural duodenal hematoma with sequential roentgenograms at 5 to 7 day intervals; (2) identify associated pancreatic injury with ultrasonography and serum amylase and lipase determinations; and (3) to determine effectiveness of nasogastric suction and total parenteral nutrition. Twelve children, who ranged in age from 2 to 15 years, with obstructing intramural duodenal hematoma following blunt injury were admitted over the past 9 years. The two youngest were battered children and two others had subsequently diagnosed clotting disorders (idiopathic thrombocytopenic purpura and von Willebrand's disease). Significant resolution of the obstruction allowed resumption of oral intake by the end of the first week of treatment in eight patients, whereas the remaining four required 13, 14, 22, and 38 days of nasogastric suction and total parenteral nutrition. Residual deformity seen on roentgenograms did not interfere with achieving adequate oral nutrition. Excellent results in this series substantiate the conclusion that a management plan that assesses the evolution of an obstructing intramural duodenal hematoma and provides adequate nutrition is a successful alternative to surgical treatment. The presence of underlying hematologic disorders and child abuse must be suspected.

Four children (ages 3-9 years) with intermittent abdominal pain and vomiting are reported. Upper gastrointestinal examinations revealed a sharply angulated to-and-fro course (Z configuration) of the distal duodenum and proximal jejunum, rather than the usual smooth duodenojejunal loop at the ligament of Treitz. At operation this configuration was associated with broad peritoneal bands extending across the involved small intestine without an accompanying volvulus at this level. The Z sign is diagnostic of incomplete rotation, even in the absence of duodenal obstruction and with a proximal jejunum that may appear to be properly placed.

Advances in the pathophysiology, diagnosis, and treatment of congenital malformations, trauma, tumors, and innovations in organ transplantation form the cornerstones of progress in pediatric surgery during the past year. Significant new contributions presented at the past year's meeting of pediatric surgical organizations, including the 56th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, are summarized, and selected references from the pediatric and surgical literature are presented. PRENATAL DIAGNOSIS/FETAL SURGERY In the area of fetal surgery, Manning et al1 summarized the experience with 74 cases of catheter decompression for fetal hydronephrosis and hydrocephalus reported to the International Fetal Surgery Register. The center reported an overall survival rate of 41% for urinary obstruction with a procedure-related death rate of 4.6%. The results of ventriculoamniotic shunt procedure for obstructive hydrocephalus were even less encouraging with a procedure-related mortality of 10%; moreover, more than 50% of survivors had serious neurologic handicaps. Sauer et al2 studied the relative importance of an abdominal mass and oligohydramnios causing pulmonary hypoplasia in fetal rabbits. Animals having bladder outlet obstruction developed severe pulmonary hypoplasia, whereas other subjects having a synthetic mass inserted into the abdomen of a size equal to that of the obstructed bladder showed minimal signs of pulmonary hypoplasia. Results of the study suggest that prenatal catheter decompression be limited to the fetus with oligohydramnios. The wisdom of planning the mode of delivery for babies with major congenital malformations referred to a maternal high-risk unit for evaluation remains controversial.