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Objectives: To test the hypothesis that physician errors (failure to diagnose appendicitis at initial evaluation) correlate with adverse outcome. The authors also postulated that physician errors would correlate with delays in surgery, delays in surgery would correlate with adverse outcomes, and physician errors would occur on patients with atypical presentations. Methods: This was a retrospective two-arm observational cohort study at 12 acute care hospitals: 1) consecutive patients who had an appendectomy for appendicitis and 2) consecutive emergency department abdominal pain patients. Outcome measures were adverse events (perforation, abscess) and physician diagnostic performance (false-positive decisions, false-negative decisions). Results: The appendectomy arm of the study included 1,026 patients with 110 (10.5%) false-positive decisions (range by hospital 4.7% to 19.5%). Of the 916 patients with appendicitis, 170 (18.6%) false-negative decisions were made (range by hospital 10.6% to 27.8%). Patients who had false-negative decisions had increased risks of perforation (r = 0.59, p = 0.058) and of abscess formation (r = 0.81, p = 0.002). For admitted patients, when the inhospital delay before surgery was >20 hours, the risk of perforation was increased [2.9 odds ratio (OR) 95% CI = 1.8 to 4.8]. The amount of delay from initial physician evaluation until surgery varied with physician diagnostic performance: 7.0 hours (95% CI = 6.7 to 7.4) if the initial physician made the diagnosis, 72.4 hours (95% CI = 51.2 to 93.7) if the initial office physician missed the diagnosis, and 63.1 hours (95% CI = 47.9 to 78.4) if the initial emergency physician missed the diagnosis. Patients whose diagnosis was initially missed by the physician had fewer signs and symptoms of appendicitis than patients whose diagnosis was made initially [appendicitis score 2.0 (95% CI = 1.6 to 2.3) vs 6.5 (95% CI = 6.4 to 6.7)]. Older patients (>41 years old) had more false-negative decisions and a higher risk of perforation or abscess (3.5 OR 95% CI = 2.4 to 5.1). False-positive decisions were made for patients who had signs and symptoms similar to those of appendicitis patients [appendicitis score 5.7 (95% CI = 5.2 to 6.1) vs 6.5 (95% CI = 6.4 to 6.7)]. Female patients had an increased risk of false-positive surgery (2.3 OR 95% CI = 1.5 to 3.4). The abdominal pain arm of the study included 1,118 consecutive patients submitted by eight hospitals, with 44 patients having appendicitis. Hospitals with observation units compared with hospitals without observation units had a higher “rule out appendicitis” evaluation rate [33.7% (95% CI = 27 to 38) vs 24.7% (95% CI = 23 to 27)] and a similar hospital admission rate (27.6% vs 24.7%, p = NS). There was a lower miss-diagnosis rate (15.1% vs 19.4%, p = NS power 0.02), lower perforation rate (19.0% vs 20.6%, p = NS power 0.05), and lower abscess rate (5.6% vs 6.9%, p = NS power 0.06), but these did not reach statistical significance. Conclusions: Errors in physician diagnostic decisions correlated with patient clinical findings, i.e., the missed diagnoses were on appendicitis patients with few clinical findings and unnecessary surgeries were on non-appendicitis patients with clinical findings similar to those of patients with appendicitis. Adverse events (perforation, abscess formation) correlated with physician false-negative decisions.

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.

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.

Background As pyramidal programs in general surgery were eliminated in recent decades, nondesignated preliminary surgery (NDPS) positions were introduced to fill vacant positions. Graduating medical students can pursue NDPS positions with the goal of obtaining categorical positions in either general surgery residencies or other fields altogether. The fate of residents who complete 2 years as NDPS residents remains ill defined. Methods From 1997 to 2007, data concerning NDPS residents were prospectively collected from 2 general surgery training programs. Trainees were followed by prospectively gathered data, Internet identification, and internal records of hospital privileging. Results One hundred ten graduating medical students initiated postgraduate training as NDPS residents. Seventy-four (67%) were men, and 98 (89%) were international medical graduates. Among all 110 subjects, 95 (86%) were hired as postgraduate year 1 NDPS residents, and 15 (14%) were hired as postgraduate year 2 NDPS residents. Fifteen (14%) left postgraduate medical education. Fifty-two NDPS residents (47%) pursued nonsurgical specialties after their internships. Forty-three (39%) eventually matriculated as categorical general surgical residents. Of these, 20 (47%) became categorical residents in their initial training programs. Nearly all NDPS residents who proceed to categorical positions obtained board certification. Conclusions More than one third of NDPS residents successfully obtained categorical general surgery positions. Only a small fraction (14%) failed to continue in postgraduate medical education. NDPS positions allow most candidates successful career paths. The persistent rate of attrition among categorical surgical residents allows NDPS residents to join categorical training programs and become eligible for board certification in general surgery.

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.

Background Attrition of general surgery residents is of continued concern in graduate medical education. It results in loss of morale and resources and often leaves programs scrambling to find replacement residents. The aim of this study was to evaluate the incidence of attrition of categoric general surgery residents as well as the fate of those who left the general surgery training program among a defined cohort of categoric general surgery residents in a university hospital residency training program. Methods We retrospectively reviewed the files of all general surgery residents at the Yale University School of Medicine–Yale New Haven Hospital Surgery Program who began as categoric interns from July 1, 1986 to June 30, 2006. Ninety-nine residents were identified. Attrition of residents was divided into withdrawals (changed specialty or left graduate medical education), transfers (transferred to a different program in general surgery), and dismissals (dismissed from the program). Results Among the 99 residents who began as categoric interns from 1986 to 2006, 66 of 99 (67%) were men. Thirty of 99 (30%) failed to complete the general surgery training program. Of these, 21 of 30 (70%) withdrew, 5 of 30 (17%) transferred, and 4 of 30 (13%) were dismissed. Attrition occurred before entering the third clinical year in 23 of 30 (77%). Two of 30 (7%) left graduate medical education. Thirteen of 21 (62%) who withdrew entered primary care or another nonsurgical specialty, whereas 7 of 21 (38%) matriculated into a surgical subspecialty. The attrition rate was 40% (12 of 30) since the academic year 2000. The overall annual attrition rate for the past 20 years was 6.7%. Comments Attrition in our general surgery training remains low. Most who leave remain in graduate medical education and transfer to a different specialty. The overwhelming majority leave before beginning their third clinical year. Although our 6.7% annual attrition rate remains favorable (national attrition rate in general surgery 5.8%), we must continue to analyze the root causes and solutions.

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.

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.

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.

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.

A 4-week-old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants with a recognized cause of intrahepatic cholestasis is necessary.

The diagnosis of wandering spleen is usually made at the time of operation owing to symptoms caused by torsion of the spleen. Splenectomy has been performed in almost all of the reported cases. A 6-year-old girl presented with intermittent abdominal pain and a mobile abdominal mass. The diagnosis of wandering spleen was confirmed by computerized tomography. Elective splenopexy was performed by securing the spleen in an extraperitoneal pocket.

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)

Rates of infections and catheter complications in children with right atrial catheters (RACs) receiving chemotherapy were compared with those without RACs in a retrospective cohort study. One hundred sixty-five children presenting to Yale-New Haven Hospital with a malignancy diagnosed between January 1, 1981 and December 31, 1985 were followed through June 30, 1986 for the number of treatment-related complications resulting in hospitalization. The time on therapy totaled 80,089 person-days that were divided into 510 periods of observation defined by the presence or absence of a RAC, disease status (remission, partial remission, or relapse), intensity of chemotherapy (using a 0 to 4 scale), and use of radiation therapy. Children with RACs experienced markedly higher rates of sepsis (14-fold higher, P less than .0001) and catheter-site infection or other catheter complications requiring hospitalization (both greater than 30-fold higher, P less than .0001). These effects persisted after controlling for other risk factors, ie, disease status, chemotherapy intensity, and age. Children with RACs also had higher rates of fever and neutropenia and other infections requiring hospitalization that were more strongly influenced by the other risk factors. The association of RACs with sepsis, fever and neutropenia, and days hospitalized for treatment-related complications was assessed using multivariable analysis methods to control for disease status, intensity of chemotherapy, radiation therapy, type of cancer, age, and sex. The presence of a RAC (rate ratio [RR], 6.39; P less than .0001) and the disease status (RR, 2.45; P = .0004) were the only factors that predicted sepsis. Fever and neutropenia rates were most influenced by chemotherapy intensity (RR, 2.31; P = .0001) and age (RR, 0.94; P = .013 per year of age) and less by the presence of a RAC (RR, 1.56; P = .053). Controlling for other risk factors, children with RACs experienced a 6.4-times higher rate of sepsis and spent an average of 15.4 more days per year in the hospital for treatment-related complications.

• 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)

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.

Use of muscle flap obliteration for bronchopleural fistulas appears to be indicated with (1) failure of a previous thoracoplasty, (2) anticipated failure of a thoracoplasty alone, and (3) the need to obviate a formal debilitating thoracoplasty. With the use of well-vascularized muscle flaps to fully obliterate the densely scarred cavities associated with persistent bronchopleural fistulas, we may hope to see improved healing in the bronchial stump and, in cases of residual infection, better resistance of the flap to necrosis, as well as improved delivery of chemotherapeutic agents to the local tissues. These factors may confer improved cure rates for bronchopleural fistulas similar to those seen in lower extremity salvage surgery for osteomyelitis following the introduction of vascularized pedicle and free muscle flaps. In this article we have described the versatility of the island pedicle latissimus dorsi muscle flap for closure of recalcitrant bronchopleural fistulas and associated empyema cavities. Utilizing either the dominant thoracodorsal or the minor paraspinal pedicle(s), it can reach any intrathoracic cavity by means of the appropriate thoracotomy incision.

Metabolic complications of TPN in infants and children are numerous and frequent. Properly designed nutrient solutions, careful monitoring, knowledge of potential problems, and appropriate management are essential to avoid serious complications. Hepatic dysfunction is most likely to prevent achievement of adequate nutrient intake. Better delineation of trace metal, essential fatty acid, and vitamin deficiencies is necessary, particularly in patients who require long-term TPN.

From 1978 to 1987, 350 infants and children had silicone central catheters placed for long-term venous access. Real time echocardiography showed a catheter tip thrombus in 16 patients, including 12 premature infants and four children. Catheters had been in place for eight to 560 days. Thirteen patients were receiving total parenteral nutrition (TPN) at the time thrombus was identified, and one patient had received TPN 2 weeks previously. Indications for echocardiography included sepsis (7), respiratory insufficiency (6), evaluation of heart disease (3), and catheter malfunction (1); several patients had multiple indications. Treatment was individualized and included atriotomy (2), systemic heparin and thrombolytic agents with or without catheter removal (8), catheter removal only (3), and observation (3). Four of eight patients treated with thrombolysis had complete clot lysis and four had partial dissolution. Two patients managed expectantly had resolution of the thrombus. None of the patients suffered further complications or died as a result of the thrombus, but ten of the 16 died 1 to 12 weeks later from their underlying disease. Forty-seven asymptomatic patients were studied by echocardiography to assess the incidence of unsuspected right atrial thrombus. Their catheters had been in place for a mean of 200 days and only 11 had been used for TPN. None of these patients had identifiable thrombus at the catheter tip. The incidence of catheter tip thrombus, which is higher than previously suspected, is related to prematurity, TPN, and continuous catheter use, but not duration of catheterization. The incidence is low in asymptomatic patients. Treatment regimen must be individualized and this series reflects a trend toward more conservative management.

Congenital abdominal wall defects are among the more common anomalies encountered by pediatric surgeons. The author's experience with omphalocele and gastrochisis is presented.

Summary Giant cystic lymphangioma is a rare lesion that presents major therapeutic challenges. Four infants presented in the first weeks of life with diffuse cysts in the neck. The tongue was involved in three, the mediastinum in one, and the pharynx and supraglottic larynx in two. These lesions are characterized by hundreds of cysts that infiltrate in and around muscles, nerves, and vessels. Complete excision is not possible without damaging the normal structures. All four infants initially had partial removal with unroofing and drainage of the remaining cysts and temporary tracheostomy to protect the airway. One infant died from a thrombosed sagittal sinus. She had severe venous congestion of the head and neck postoperatively which may have been caused by an overzealous attempt to remove all of the cysts. The other three have required nine additional operations for removal of recurrent neck cysts (one operation), removal of mediastinal cysts (one operation), partial glossectomy (three operations), and laser excision of lymphangiomas of the pharynx and supraglottic larynx (four operations). Two patients have required partial glossectomy which should be performed early to protect the airway, to allow normal speech development, and to prevent malocclusion and prognathism from constant pressure of the tongue against the teeth. Removal of wedges of tissue from each side and from the center of the dorsum of the tongue reduces bulk with minimal risk to the lingual arteries and nerves. Recurrent tongue enlargement is common and can be treated by repeated partial glossectomy. Lymphangiomatosis of the pharynx and supraglottic larynx is difficult to treat. The carbon dioxide laser allows very precise excision of individual cysts with minimal drainage to adjacent mucosa, but in our limited experience it has not prevented recurrence. Despite the extensive nature of these lesions, the impossibility of complete excision, and the need for multiple operations, the long-term results in the three surviving patients have been satisfactory. Asymmetry of the neck and face due to lymphedema tends to improve with time. All three children have an acceptable appearance and normal speech for their ages.

Nutritional support, whether enteral or parenteral, is an important part of the treatment of IBD. Inadequate oral intake, malabsorption, and increased gastrointestinal losses all contribute to malnutrition. Weight loss, cachexia, abnormal body composition, and multiple micronutrient deficiencies are common. Acute repletion of body weight and correction of specific nutrient deficiencies improve the patients' sense of well-being and decrease morbidity, especially in the perioperative period. If a short period of bowel rest (10 to 14 days) is part of the medical therapy of acute exacerbations of IBD, TPN should be administered to prevent further nutritional deficiencies. Chronic undernutrition, and growth failure in children, usually are best treated by intensive enteral supplementation. Prolonged bowel rest and TPN (4 to 6 weeks) have not been shown to improve outcome but may be appropriate in carefully selected patients. Long-term home TPN may be necessary for patients who have short gut syndrome. The mainstay of treatment for IBD is medical therapy including corticosteroids. Timely and appropriate surgery is equally important and should not be considered a last resort. Careful nutritional management is essential but is adjunctive rather than primary therapy.

Fifty-seven cases of apple peel jejunal atresia have been reported in the English literature. Patients with this anomaly have a high incidence of prematurity (70%), malrotation (54%), short gut syndrome (74%), multiple atresias (15%), complications (63%), and mortality (54%). Mortality has decreased from 63% to 47% since 1970. We report three new cases of apple peel jejunal atresia, including two from one family. Radiographic evidence of high small bowel obstruction and a malrotated microcolon on preoperative roentgenogram with barium enema should suggest this diagnosis. Five families, including ours, have been reported in which more than one child was affected, and it has been suggested that this disorder is transmitted by an autosomal recessive gene. However, the occurrence of conventional intestinal atresia in other siblings, the association with multiple atresias, and discordance in a set of apparently monozygotic twins indicate that there may be a more complex spectrum of genetic transmission. Subsequent siblings are at increased risk for apple peel atresia or related malformations. Prenatal ultrasound can facilitate early diagnosis and treatment.

Elective cesarean section (CS) following prenatal diagnosis of gastroschisis has been advocated to decrease morbidity and mortality. To examine this hypothesis, we reviewed the records of 28 consecutive patients with gastroschisis treated between 1975 and 1987. Fourteen infants were delivered vaginally (V) and fourteen by CS, of which seven were elective, five were for fetal distress, and two were for breech presentation. Prenatal diagnosis was made in nine infants in the CS group and none in the vaginal group. The two groups were comparable in gestational age (V = 37.6 weeks, CS = 35.8 weeks, P = .05), birth weight (V = 2,508 g, CS = 2,444 g, P = NS), and five-minute Apgar score (V = 7.8, CS = 6.8, P = NS). Outcome was similar as measured by hospital mortality (V = 0/14, CS = 1/14, P = NS), complications (V = 4/14, CS = 5/14, P = NS), days to enteral feeding (V = 14, CS = 19, P = NS), and days in the hospital (V = 27, CS = 34, P = NS). The only complication related to mode of delivery was preventable; an infant delivered vaginally had avulsion of a short segment of mesentery requiring bowel resection. Infants born by CS were slightly more likely to have primary closure (5/14) than babies delivered vaginally (3/14, P = NS), but this may reflect independent trends in the last 5 years. Elective CS following prenatal diagnosis in seven patients did not improve outcome; primary closure was achieved in only one infant, and three had a complication. Since these data show no significant difference in morbidity and mortality between vaginal and CS delivery, we suggest that CS should not be recommended simply because a prenatal diagnosis of gastroschisis is made.

To determine the relative accuracy of the various radiologic signs of Hirschsprung disease (HD), we retrospectively reviewed both radiographs obtained after a barium enema and the medical records of 62 children who had surgery to prove or exclude the diagnosis of HD. The visualization of a rectosigmoid transition zone was highly predictive of HD, but nonvisualization did not rule out HD. A false positive transition zone at the splenic flexure was seen in four neonates who had small left colon syndrome rather than HD. Retention of barium seen on radiographs obtained 24 hours after a barium enema was not a specific sign, but it was the only sign of HD in seven neonates, including two who had total colonic aganglionosis. Anal manipulation prior to the barium enema examination did not affect the diagnostic value of that procedure. We conclude that the single most reliable radiographic sign of HD is the presence of a rectosigmoid transition zone. Statistically, the use of three radiographic features combined (rectosigmoid transition zone, retention of barium, and stool mixed with barium) correlated better with the presence or absence of HD than did any of these features alone. A comparison of 24 and 48 hour postevacuation radiographs may help to differentiate HD from meconium plug syndrome.

We describe a case of a full-term infant with severe respiratory failure and pulmonary insufficiency caused by an anomaly consisting of a single, unilobar lung arising from the trachea and situated in the middle mediastinum. Plain film, echocardiogram, and surgical aspects will be described and correlated with the post mortem findings and embryologic considerations. We were unable to find a similar case reported in the literature and conclude that this rare lesion represent an intermediate form between total and unilateral pulmonary agenesis.

Laparoscopy was performed at operation in 32 patients between 7 and 63 months old (average age 31 months), who had 33 nonpalpable testes. Of the testes 7 (21.2 per cent) were in the inguinal region or just proximal to the internal ring, 5 (15.2 per cent) were in a high intra-abdominal position and 21 (63.6 per cent) were absent. In every instance of a nonpalpable gonad in which a testis was found to be absent at operation the vas and gonadal vessels were observed laparoscopically to exit the inguinal ring or to end blindly just proximal to the internal inguinal ring. A hernia or patent processus vaginalis was not observed in these 21 cases. In every case of an absent testis the contralateral testis was located normally in the scrotum. Furthermore, when a hernia was observed laparoscopically a testis was found on the ipsilateral side at the level of the internal ring or more distally.

Hydrostatic reduction of ileocolic intussusception by barium enema is uniformly regarded as optimal management, with surgical treatment reserved for occasions when this is not successful and for other selected circumstances.1 In an attempt to maximize the success rate of hydrostatic reduction, we examined the effect of analgesic premedication on both the outcome for the barium study and the operative findings. MATERIALS AND METHODS The case records and radiographs of 76 children with ileocolic intussusception treated at the Yale-New Haven Hospital between Jan 1, 1974, and Dec 31, 1984, were reviewed for patient age, sex, seasonal incidence, presenting signs and symptoms, and results of hydrostatic reduction, as well as surgical treatment with operative findings, morbidity, and mortality.

Splenic pseudocysts have traditionally required splenectomy because of the risks imposed by partial splenectomy or excision of the cyst lining. During the past 2 years, a 6-year-old boy and a 9-year-old girl presenting with vague upper abdominal discomfort, palpable splenomegaly, and a large unilocular sonolucent cyst within the spleen, were treated by partial splenic decapsulation with preservation of the hilar blood supply. This procedure involves (1) mobilizing the spleen by dividing the renal, colic, and diaphragmatic attachments; (2) decompressing the liquefied cyst contents through a thoracostomy trochar; (3) excising the outer splenic capsule and gaining hemostasis of the splenic wall with a running interlocked silk suture; and (4) providing external tube drainage of the left upper quadrant. During the follow-up period of 26 and 12 months, splenic size has returned to normal. Serial nuclear scan and ultrasound show a small residual crescent-shaped deformity of the functioning splenic remnant. We conclude that partial splenic decapsulation for splenic pseudocyst is simpler and safer than other preservation procedures attempted, and carries no increased risk of recurrence from leaving a portion of the pseudocyst wall.

Transient hepatic secretory obstruction manifested primarily by chemical evidence of cholestasis with a conjugated bilirubin above 2.0 mg/100 ml occurred in eight of 19 neonates and infants receiving total parenteral nutrition. The incidence of cholestasis was greater in the premature than full-term infant. Prospective determinations of conjugated bilirubin and 5' nucleotidase are essential to detecting cholestasis before jaundice becomes obvious. These tests are more sensitive than serum alkaline phosphatase which normally rises after birth and during periods of accelerated osteoblastic activity. Preliminary data indicate that the 5' nucleotidase is the most sensitive indicator of secretory obstruction and may become elevated in patients with a normal direct bilirubin. The etiology of hepatic cholestasis during total parenteral nutrition is unknown but is presumed to be caused by interference with hepatocellular enzymes controlling bile secretion; immaturity of these enzyme systems increases the risk of secretory obstruction.

A new born presented in congestive heart failure on the second day of life. Cardiac catheterization was normal, but aortic injection showed a congenital umbilical artery to umbilical vein arteriovenous malformation. Three similar cases have been reported. All four patients were successfully treated by complete excision of the malformation.

• Renal and pulmonary Aspergillus granulomas in two 6-year-old boys with chronic granulomatous disease were treated by nephrectomy and lobectomy as well as intravenous amphotericin B and granulocyte transfusion. After lobectomy two smaller pulmonary aspergillomas resolved with medical therapy. The second child's condition improved after nephrectomy, but he died with disseminated Aspergillus infection. This experience suggests that surgical excision of localized Aspergillus granulomas in conjunction with specific antimicrobial therapy provides temporary clinical improvement and may prevent further blood-borne dissemination of the fungus. ( Arch Surg 113:640-642, 1978)

Insidious leg pain and weakness followed inguinal hernia repair. Despite the temporal relationship of the surgery and the symptoms, the diagnosis proved elusive, in part, because of the rarity of the complication.

The foreshortened, strictured esophagus resulting from chronic gastroesophageal reflux presents a therapeutic challenge. The fundic patch procedure, combined with fundoplication, may be uniquely suited to this problem since the fundoplication is effective even in the thorax. The first child reported to have an intrathoracic fundoplication continues to have a good result seven years later. Two other children have had effective treatment by the combined Thal-Nissen procedure. Further clinical trials with this operation in children appear to be warranted.

We have investigated the role of the urinary 3-methylhistidine (3MH) excretion, a measure of protein catabolism, in the evaluation of the metabolic state of premature infants. Two-hundred and twenty-two 24 hr urine collections and 3MH/Cr ratio determinations (expressed as mumoles of 3MH per mg creatinine) were carried out in 36 infants (average gestational age 32.7 +/- 0.7 wk, weight 1640 +/- 120 grams) and the relationship between the 3MH/Cr ratios and the metabolic and clinical state has been investigated. Five or more 3MH/Cr measurements were carried out on each of 19 infants and serial determinations on four of those babies are presented. The urinary 3MH/Cr ratio of healthy infants with adequate caloric intake and normal growth curve was .148 +/- .039 (S.D.) mumol/mg, about 35% higher than the 3MH/Cr ratio in healthy adults. As long as the premature infants were healthy the degree of prematurity had no effect on the 3MH/Cr ratio. The relationship between 3MH/Cr ratio and nitrogen balance was highly significant (p less than .001). 3MH/Cr ratio also correlates very well with the metabolic status of the infants: in the group with normal 3MH/Cr ratios less than or equal to .175 (.148 + 1 S.D., n = 90) there were four clinically stressed infants (4.4% false negative rate) while in the group with elevated 3MH/Cr ratios greater than .225 (.148 + 2 S.D.; n = 79) there were only three clinically well infants (3.8% false positive rate). In comparing the clinical status and 3MH/Cr ratios, we found that in the group of infants who could not be clearly defined as clinically well or stressed (n = 108) the 3MH/Cr ratio was more useful than clinical judgment in the prediction of metabolic status. It can be concluded that 3MH/Cr ratio is a potentially useful clinical tool which describes with high accuracy the clinical and metabolic status of premature infants. This conclusion is further supported by the data of serial 3MH/Cr determinations.

Summary Porcine skin grafts and human amniotic membranes have been used as biologicaldressings for the treatment of gastroschisis or omphalocele in 16 newborn infants. Eleven infants with gastroschisis were managed initially by the Silastic pouch technique but delayed healing resulted in separation of the Silastic sheeting from the wound margins and required use of biologic dressings to achieve complete closure. Biologic dressings were used in five patients with intact omphaloceles for all or part of their course. Average time to achieve complete closure was 55 days. Three patients died of associated anomalies. One patient developed an enteric fistula. Biologic dressings appear to be useful adjuncts in the management of some patients with gastroschisis or omphalocele.

The nutritional and metabolic complications following intestinal resection and ileostomy for necrotizing enterocolitis (NEC) in low birth weight preterm infants often necessitate repeated prolonged hospitalization for salt and water imbalance and reliance on total parentaral nutrition (TPN). The traditional concerns about anesthetic and anastomotic complications delays the restoration of intestinal continuity until the infant has attained a weight of about 5 kg, but recent nutritional balance studies in our unit have shown a combination of nutrient and mineral malabsorption in neonates with ileostomies. Beginning 4 years ago, a prospective study of early closure of the ileostomy was undertaken in infants weighing as low as 2 kg to examine the effect on surgical morbidity, infant growth, and gastrointestinal function using the preclosure infant as his/her own control. Ten infants with birthweights ranging from 670 to 2,000 g developed NEC requiring ileostomy at age three days to 11 weeks. In addition to partial ileal resection, the cecum was resected in 10 patients, ascending colin in 7, transverse in 4, descending colon in 1 patient. Postoperative treatment, including short-term TPN and elemental diet, preceded closure of ileostomy at a mean age of 18 weeks (range 5 to 36 weeks). Mean weight at time of closure was 3,052 +/- 994 g. There were no short-term complications of early closure in this series, nor was there any incidence of anastomotic dysfunction, colon stricture, or recurrent NEC.(ABSTRACT TRUNCATED AT 250 WORDS)

From 1979 through 1981, 64 premature infants who weighed less than 1,500 g underwent 68 operations and 52 survived (81 percent). Twenty-six of 31 infants who weighed less than 1,000 g (84 percent) and 26 of 33 infants who weighed between 1,000 and 1,500 g survived (84 and 79 percent, respectively). The most common operation was ligation of a patent ductus arteriosus in 53 infants, of whom 43 survived (81 percent). Ten infants were operated on for necrotizing enterocolitis and 7 survived (70%). Four of five infants (80 percent) survived other major operations. Factors which influence survival include appropriate timing of operation, preoperative and postoperative mechanical ventilation, and parenteral nutrition. Intraoperative management includes short-duration anesthesia, continuous monitoring of vital signs, and judicious use of small amounts of amnestic and neuromuscular blocking agents combined with low fractional inspired oxygen concentration to reduce the risk of oxygen toxicity. Utilizing these principles, the survival rate compared favorably with the survival rate of all premature infants, which establishes that major surgery can be undertaken with only moderate risk in the infant with very low birth weight.

Twenty-two children with severe active inflammatory bowel disease were treated with total parenteral nutrition and bowel rest for 2 to 4 weeks in an attempt to induce remission, decrease steroid dependence, stimulate growth and avoid surgical resection. Of 14 patients with Crohn's disease, 9 had complete remission, 4 improved and 1 was unchanged. Eight patients had recurrence within 3 months and three within 1 year, and two are in remission. Eight patients have required operation within 22 months. Eight children had severe exacerbations of ulcerative colitis (more than 10 bloody stools a day). Four patients did not improve and required total colectomy. Four patients had resolution of symptoms, but two of these had recurrent symptoms at 10 and 11 months. Total parenteral nutrition and bowel rest induce temporary remission or significant improvement of Crohn's disease in most children. The remission is short-lived, however, and most patients have recurrence and require operation within 1 year. A few children have a satisfactory long-term result, but there are no criteria to predict a good response. If an operation is indicated, it should not be deferred in the hope that total parenteral nutrition might provide lasting benefit, although a temporary delay of operation may be of great value in selected patients. Total parenteral nutrition is less effective in ulcerative colitis than in Crohn's disease. Most children who have acute exacerbation of disease and do not respond to bowel rest and intravenous steroids within 1 week should have colectomy. Total parenteral nutrition may be helpful in selected patients, particularly if time is needed to prepare the child for operation, but it does not alter the long-term course of the disease.

It is necessary to consider catheter-related events when a previously stable patient has an acute change in cardiopulmonary status, regardless of the duration of catheter use; and to recognize that hydrothorax from vein perforation may be bilateral, necessitating bilateral drainage procedures.

Received October 27,1981;accepted after revision December 30,1981.‘Department ofDiagnostic Radiology, YaleUniversity School ofMedicine andYale-New Haven Hospital, 333Cedar St.,NewHaven, CT06510. Addressreprint requests toR.C.Ablow.2Department ofPediatrics, YaleUniversity School ofMedicine andYale-New Haven Hospital, NewHaven, CT06510.‘Department ofSurgery, YaleUniversity School ofMedicine andYale-New Haven Hospital, NewHaven, CT06510.742AJR138:742-743, April19820361-803X/82/1 384-0742 $00.00 ©American Roentgen RaySociety