Objective: To characterize the natural history and outcome of spontaneous pneumothorax in patients under age 16 years. Design: This is a retrospective review in which all patients admitted to the authors' institution (between December 1980 and July 1993) with a diagnosis of spontaneous pneumothorax were included. Information was obtained from the hospital records. Setting: This study was performed in a university childrens' hospital. patients: All patients under 16 years with a diagnosis of spontaneous pneumothorax were included. Results: Seventeen patients were admit ted and analyzed. Their age range was 2 to 16 years; 65% were male. Only three admissions were for recurrence. The main symptoms included chest pain (100%), shortness of breath (41%), and cough (6%). Chest tube drainage was successful in 65% of the cases; the median time was 4 days. Two patients had a persistent air leak. An underlying cause was recognized in 42% of cases. Seven operations were necessary, and the mean follow-up period is 31 months, with no recurrence reported to date. Conclusion: Spontaneous pneumothorax occurs in the pediatric population, although it is less common than in adults. An underlying pathology is more frequently observed in pediatric patients, but recurrence is less common. Chest tube drainage alone is sufficient treatment for the majority of patients; however, a significant number require some form of surgical intervention. Copyright (C) 1995 by W.B. Saunders Company.

True duplication of the cervical esophagus is rare. An 18-month-old girl presented with a large asymptomatic neck mass, which was found to be an intramural duplication of the esophagus. The evaluation and treatment of this patient is presented as well as a review of the six previously reported cases.

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and pulmonary hypertension. We hypothesize that a surfactant deficiency may in part be contributing to the pathophysiology of CDH. This study documents the functional, quantitative, and qualitative aspects of the surfactant status of the alveolar air-liquid interface and the type II pneumocyte in the fetal lamb CDH model. Ten lamb fetuses (gestational age, 80 days) had a CDH created via a left thoracotomy and then were allowed to continue in utero development until term. Three litter mates and three nonoperated time-dated fetuses served as controls. At term, pressure-volume curves were performed to measure pulmonary compliance and total lung capacity. Alveolar lavage was then performed to measure the quantitative and the qualitative aspects of pulmonary surfactant. Finally, isolation of type II pneumocytes allowed quantification of phospholipid synthesis. When compared with controls (N = 6), the CDH lambs (N = 5) had significantly smaller lungs (P = .009), decreased total lung capacity (P < .001) and compliance (P < .001), reduced total lavaged phospholipids (P = .006), and decreased percent phosphatidylcholine (P = .02). CDH lambs also had increased total lavaged proteins (P = .05) and higher minimum dynamic surface tension (P < .001). A surfactant deficiency may be contributing to the pathophysiology of CDH. Surfactant replacement therapy in premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Exogenous surfactant may also benefit infants with CDH.

Exogenous surfactant therapy (EST) in surfactantdeficient premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Reports have demonstrated that newborns with congenital diaphragmatic hernia (CDH) have lung compliance, pressure-volume curves, and hyaline membrane formation resembling those changes seen in surfactant deficient premature newborns. We hypothesize that EST may also benefit infants with CDH. All high risk cases of prenatally diagnosed CDH at Children's Hospital of Buffalo from November 1988 to February 1991 were prospectively evaluated for EST. In those families who chose to participate, the surfactant preparation, Infasurf (100 mg/kg), was instilled into the newborn's lungs prior to the first breath. The remainder of the perinatal, neonatal, and surgical care was performed in a routine manner. Three high-risk prenatally diagnosed newborns with left CDH were treated with EST. All showed signs of decreased pulmonary compliance, but could still be adequately oxygenated and ventilated. Surgical correction was performed after stabilization and all required patch closures. Two of the three infants suffered no life-threatening episodes of pulmonary hypertension and all survived. These infants had many known indicators for poor outcome in CDH with an expected survival of less than 20%. We believe that EST in these neonates with CDH contributed to their survival with minimum morbidity. These results suggest that surfactant replacement for the high-risk neonate with CDH warrants further consideration and a randomized clinical trial is being planned.

Only one third of the applicants for training in pediatric surgery obtain a position through the pediatric surgery matching program. In order to identify factors that contribute to a successful outcome, we conducted a retrospective survey of all participants in the matching process for positions during the years 1983 to 1991. This survey was designed to identify characteristics associated with success in the match through comparison of successful and unsuccessful applicants. Significant factors associated with a successful match included: a greater incidence of residency-supported research (P = .012) with a greater number of publications (P = .003) and national presentations (P = .014), specifically at the annual meetings of the American Pediatric Surgical Association (P = .05) and the American Academy of Pediatrics (P = .05). In addition, successful candidates had more contact with (P = .004) and letters of recommendation from (P = .015) well-known pediatric surgeons involved in the general surgical residency program. This information should be invaluable to those counseling medical students and residents interested in a career in pediatric surgery.

Multifactorial sex-modified inheritance has been proposed as the model of transmission in familial Hirschsprung's disease (HD). A review of two separate kindreds suggests that aganglionosis may be inherited as an X-linked recessive or an autosomal dominant trait. Chromosomal anomalies and other syndromes, including G6PD deficiency, may occur with familial HD. Recurrence risk counseling for family members depends on accurate pedigree analysis and a comprehensive understanding of the genetic factors involved.

Advanced neuroblastoma treated with standard chemotherapy has a poor prognosis. Combination immunotherapy for murine neuroblastoma with retinyl palmitate, low-dose cyclophosphamide, and interleukin-2 resulted in increased survival, impaired tumor growth, easier surgical resection, and increased class I expression or tumor cells. Preoperative immunotherpay may be useful in treatment of advanced human neuroblastoma.

We report the presence of an extra chromosomal element in a family with Wilms' tumor (WT). This family has three children, two of whom were affected. One son, the proband, had bilateral and one daughter had unilateral WT. The first child, the father, and the mother did not have WT. The son with bilateral WT had a ring chromosome (R) both in the lymphocytes as well as in the kidney tissue. The size of the ring varied considerably from cell to cell. The daughter with unilateral WT had an abnormal clone containing a small chromosomal ring (r) in phytohemagglutinin (PHA)-stimulated and Epstein-Barr virus (EBV)-transformed lymphocytes. The mother had a karyotype similar to that of the daughter with WT. We hypothesize that the proband's ring chromosome could be the amplified form of the r inherited from the mother. Chromosome 11 was cytogenetically normal in all the cells examined of the affected children and the unaffected mother. In situ hybridization with a centromere-specific DNA cocktail indicated dispersed centromeric DNA both in r and R.

To investigate the immunologic consequences of non-operative management of splenic injury, three parameters were studied: survival following pneumococcal sepsis, clearance of blood-borne bacteria, following Hemophilus influenzae challenge, and antibody response to type III pneumococcal capsular polysaccharide. Two hundred twenty-five Sprague-Dawley rats were divided into three groups and subjected either to a splenectomy, a sham operation, or standard blunt trauma. A significant increase in mortality was noted in the splenectomized group as compared with both the traumatized and control groups when challenged with Streptococcus pneumoniae. In both the control and trauma groups, H influenzae cleared significantly within 18 hours. Blood-borne bacteria persisted at the same level for 72 hours in the splenectomized animals. Four and 11 days later, the antibody level in both traumatized and control groups was higher than in the splenectomized subjects (P less than .001). There was no difference in the serum antibody level between the control and trauma groups at four days. However, at 11 days the trauma group showed a significant decrease in the antibody level (P less than .05). It can be concluded that following spontaneously-healing splenic trauma in the rat model, survival, bacterial clearance, and antibody response were all superior to that observed in the splenectomized subjects. In addition, the healed splenic disruption did not impair clearance of blood-borne encapsulated bacteria.

In most medical schools, exposure to pediatric surgery is presented as a subspecialty elective. We have offered it as an integral part of the surgical clerkship for 10 years in the belief that it provides an excellent educational environment. To confirm this concept, the quizzes (Q), final examinations (FE), and grades of students assigned to the pediatric surgical service were prospectively studied. All students (N = 139) in the surgical clerkship entered the study. Thirty-two students were randomly selected and assigned to the surgical service of a major pediatric hospital (P-Surg) for 50% of their clerkship. The other students (N = 107) were assigned to a variety of adult surgical services (G-Surg) and served as the control group. All students attended the same seminars, used the same educational materials, were examined with the same test items, and were evaluated by the same oral examiners. Test items were electronically scored and the database was analyzed on an IBM computer. The statistical analysis was performed using a Student's t test and chi 2 analysis. There was no significant difference in the demonstrated cognitive performance and grades awarded to the two groups of students. We conclude that a pediatric surgical service provides an atmosphere that is educationally comparable to the adult general surgical service.

We report a case of left iliofemoral vein thrombosis with extension to the inferior vena cava associated with giant right hydronephrosis secondary to ureteropelvic junction obstruction. Surgery revealed marked infrarenal vena caval compression and deviation to the left side caused by the dilated right renal pelvis, with resultant kinking of the origin of the left iliac vein. It is postulated that the reduction in blood flow caused by this compression and distortion predisposed this patient to venous thrombosis.

The records of 583 children who were treated for intussusception at the Children's Hospital of Buffalo in the period 1930-1985 were reviewed. Following a change in management in 1970 from operative treatment to hydrostatic reduction of the intussusception by barium enema, two main groups are defined. In earlier years 95% of patients underwent operative reduction whereas in the latter period 92% had barium reduction attempted. The remaining 8% in this group had clinical contraindications for hydrostatic enema attempt. Ten percent had pathological lead points. Recurrent intussusception occurred in 50 cases (8.5%), 66% following barium enema reduction and 33% after surgery. The mortality in the earlier group was 3.9% and 1.3% in the latter group. No deaths occurred in patients treated successfully with barium enema reduction, and there were no deaths in the children with simple uncomplicated intussusception requiring surgery.

Visceral ischemia secondary to increased intraabdominal pressure (IAP) following closure of abdominal wall defects presents a serious postoperative problem. Currently, the method of closure and postoperative management are determined by clinical impressions rather than measurement of IAP. In this study various methods of indirectly measuring IAP were compared in 17 rabbits in which IAP was sequentially increased with an intraabdominal balloon. Vesical and inferior vena caval (IVC) pressures were found to have good statistical correlation with IAP. Other methods tested were gastric, rectal, superior vena caval, femoral and brachial artery, and rectus compartment pressures. All were found to be poor indicators of actual IAP. In nine of the rabbits, radiolabeled microspheres were used to assess cardiac output and visceral blood flow. Renal blood flow was very sensitive to increased IAP with dramatic impairment at IAP above 10 to 15 mmHg. Small intestinal flow was less sensitive and did not become significantly diminished until IAP exceeded 25 to 30 mmHg. Our studies suggest that vesical and IVC pressure monitoring should be used to evaluate IAP in the clinical setting. If IAP is in excess of 10 to 15 mmHg surgical intervention is indicated to prevent the development of renal ischemia.

Before 1969, 58 patients with Wilms' tumors treated at Children's Hospital of Buffalo had an all-stage survival of 57%. From 1970 to 1983, following the National Wilms' Tumor Study protocols, this survival rate has improved to 79% in the last 47 patients. With continued attention toward early diagnosis, accurate and complete staging, refinement of radiation and chemotherapy protocols, and absolute follow-up of all patients, the relapse-free survival rate will continue to improve.

During a five-year period from 1979 to 1985, 100 consecutive children with perforated appendicitis were managed at our institution. These patients were divided into two groups, which were determined, by length of illness and physical findings. Group A consisted of 88 children with signs and symptoms of peritonitis from appendiceal perforation. They were treated with fluid resuscitation, antipyretics, and triple antibiotics (ampicillin, gentamicin, clindamycin), and appendectomy within a few hours of hospitalization. Saline lavage was used. Group B was composed of 12 patients with a periappendiceal mass without generalized peritonitis who had symptoms of 6 to 12 days duration. They were treated nonoperatively with triple antibiotics and underwent interval appendectomy 4 to 6 weeks later. The complication rate for Group A was 6.8%. This included three wound infections (3.4%), one intra-abdominal abscess (1.1%), one patient with pneumonia and ileus (1.1%), and a small bowel obstruction (1.1%). These results are equivalent to the lowest complication rate reported in the literature, in which the treatment included transperitoneal drainage, antibiotic lavage, and parenteral antibiotics Group B patients had a 16.7% complication rate, which included one small bowell obstruction and one recurrent intra-abdominal abscess. Our method of management resulted in the lowest complication rate reported to date in children with perforated appendicitis. Transperitoneal drainage, delayed wound closure, and antibiotic lavage were not used. Subcuticular incisional closure resulted in minimal wound care and excellent cosmetic results. The experience demonstrates that with proper timing of surgery and appropriate use of contemporary antibiotics, the morbidity of perforated appendicitis can be limited and excesses of treatment can be avoided.