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Data from 505 patients (1976 through 1995) who underwent anterior spinal exposure were retrospectively analyzed. There were 222 boys and 283 girls with a mean age of 14.5 years; 166 had thoracic exposure (T), 300 thoracoabdominal (TA), 44 retroperitoneal (R), and 7 transperitoneal (TP); 17 had repeat exposure (5 had initial exposure elsewhere); 70% had scoliosis, 25% kyphosis, 27% a neuromuscular disorder (NMD) and 6.7% a tumor. Average intensive-care-unit stay was 2.5 days, 6.2 days for NMD (P < .05); average ileus was 3.4 days, 4.1 days for NMD (P < .05); and average length of stay was 15.4 days for all patients, 19.3 days for NMD (P < .05). Mechanical ventilation over 96 hours was required in 31 patients, 66% had an NMD (P < .05). The morbidity rate was 9.8%, 10.1% for NMD; the morbidity rate was zero for tumor and repeat exposures. Mortality was zero. Over half of the vessel injuries (57%) and the urinary tract infections (60%) occurred in NMD patients. Differences between the 1976 through 1985 period and the 1986 through 1995 period were a shorter length of stay and a majority of one-stage combined exposures in the latter period. The authors conclude that anterior exposure of spinal deformities is well tolerated by most pediatric patients, and that this technique is easily adaptable to the resection of retroperitoneal and thoracolumbar tumors.

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Failure of the Nissen fundoplication can be secondary to disruption of the wrap or slippage of the stomach upward within the wrap. A modification of the Nissen fundoplication was devised and implemented between 1982 and 1995 to eliminate these complications. This report describes the modification and the results. Commonly, the Nissen fundoplication uses a single anterior row of sutures securing the wrapped stomach to the esophagus. This modification reinforces the wrap with two additional rows of suture, one right lateral and one left lateral, each further anchoring the wrapped stomach to the esophagus. This retrospective analysis compares the Nissen and modified Nissen fundoplications performed at our institution. Fundoplication operations were considered a failure if they required a reoperation secondary to wrap disruption or stomach slippage. Data were analyzed using the chi 2 method. A total of 948 fundoplication procedures were performed; 326 Nissen and 622 modified Nissen. Follow-up ranged from 0.5 to 13 years (mean, 6 years). Thirteen wrap disruptions and six stomach slippages occurred in the Nissen group (5.8%); 10 wrap failures and no stomach slippages occurred in the modified Nissen group (1.6%), P < .05. The authors conclude that this modification of the Nissen fundoplication significantly reduces wrap disruptions and stomach slippages.

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This is the 17th report of the case of an infant who had ileal atresia associated with Hirschsprung's disease, and the second with ganglion cells distal to the atresia. Experience suggests that Hirschsprung's disease should be suspected in all forms of bowel atresia.

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Familial occurrence of congenital diaphragmatic hernia is rare. This is only the second case of parent-to-child inheritance and the first case of father-to-son inheritance. The available data point toward a multifactorial mode of genetic transmission.

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Central line dislodgment in toddlers continues to be a problem. The deployment of a spandex/nylon bodysuit has resulted in eliminating this problem in two overactive toddlers. We highly recommend using this type of garment in appropriate clinical situations.

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We reviewed 187 cases of documented neonatal necrotizing enterocolitis (NEC) from 1976 to 1988. Of these patients, 111 infants underwent celiotomy for acute surgical complications. The following protocol of operative indications was employed: pneumoperitoneum, localized mass, abdominal wall erythema, portal venous air, and clinical deterioration, singly or in any combination. Clinical deterioration was defined as falling platelet count, rising or falling white blood cell count, left shift in the myeloid series, persistently or progressively low pH, and increasing frequency of apnea or bradycardia. Overall mortality was 15% (28 of 187). For the patients who underwent celiotomy, all had histologic confirmation of NEC. Ninety-five had localized disease, and 16 had diffuse disease. All of the former had resection and diverting enterostomy with 85 (89.5%) surviving; none with diffuse disease survived, P less than 0.0001. Forty-one infants with NEC weighed less than 1,000 g; 25 underwent surgery and 15 (60%) survived. Fifty-one of the 159 surviving neonates (32%) developed intestinal strictures. All neonates with strictures have had resection and successful reconstruction of their gastrointestinal tract. These indications and surgical principles resulted in a high degree of diagnostic accuracy and a low degree of surgical mortality.

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From 1976 to 1986 inclusive, 122 patients were cared for with Hirschsprung's disease. Sixteen of these were treated for total colonic aganglionosis, with or without small bowel involvement. The male to female ratio was 2.2:1. Two children died prior to definitive surgical therapy and two others were transferred following initial therapy. Twelve children underwent Martin's procedure with a 0% mortality rate and an 81.8% morbidity rate. This study would indicate, as do others, that even though the Martin procedure can safely be performed, the long-term results require close scrutiny. A re-evaluation of this procedure and its alternatives is necessary in order to improve long-term results.

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Five critically ill neonates underwent tube mediastinostomy in the neonatal intensive care nursery for tension pneumomediastinum. All of the neonates showed immediate clinical improvement, with a decrease in peak airway pressure and elevation of arterial oxygen pressure levels. There were no complications directly attributable to the procedure. We believe that pneumomediastinum in association with severe hypoxia, metabolic acidosis, and high ventilation pressures indicates clinically significant tension in the mediastinum. This results in a decrease in systemic blood pressure and pulmonary venous return that is not amenable to conservative management. Needle aspiration is inadequate because of the dynamic nature of the air leak. Tube decompression of the mediastinum is the treatment of choice in these circumstances.

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Examination of the records of 378 children with intussusception at our institution revealed that 29 cases were caused by an identifiable intestinal lesion. A Meckel's diverticulum was the causative agent in 21 children, all of whom were under 2 yr of age. A previously undiagnosed ileal lymphosarcoma produced the intussusception in six other children, all between 6 1/2 and 9 yr of age. Our experience indicates that any child over 6 yr of age with the clinical findings of colicky abdominal pain, bloody stools, and a palpable mass plus the radiographic evidence of intussusception must be considered to have ileal lymphosarcoma until proven otherwise. Hydrostatic reduction of the intussusception must be accompanied by extensive small bowel reflux of barium in order to effectively rule out a small intestinal lesion. If this is not accomplished, surgery should be planned with the suspicion that a malignancy may be present. If this suspicion is confirmed by frozen section, the operation procedure should include wide surgical excision of the lesion along with the regional lymph nodes.

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Based on this series of six patients with aganglionosis of the entire colon we conclude: (1) Radiographic findings of a shortened colon of normal caliber or the presence of "jejunalization" of the colon suggest total colonic aganglionosis in patients with a suggestive history. (2) All infants with persistent obstipation, distention, and poor weight gain should have a punch biopsy of the rectum even if the barium enema is normal. (3) The Martin modification of Duhamel's operation gives functional results comparable to those achievable in children with short-segment Hirschsprung's disease. (4) The use of a stapling device to divide the septum between aganglionic colon and pulled-through ileum is less satisfactory than using crushing clamps.

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Serial determinations of the absolute granulocyte and platelet counts were performed in 40 infants with severe neonatal necrotizing enterocolitis. Fourteen of the 38 infants had absolute granulocyte counts less than 1,500 nm3, the mean absolute granulocyte count was significantly lower in the group of infants who died during the acute episode of NEC as compared to that of the infants who survived. Thrity-five of 40 infants had nadir platelet counts less than 150,000/nm3, clinical bleeding occurred in 12 of the thrombocytopenic infants. Fourteen thrombocytopenic infants were evaluated for disseminated intravascular coagulation by additional coagulation studies; six were noted to have laboratory evidence of DIC. We conclude that (1) a low absolute granulocyte count in severe NEC is associated with a poor prognosis and (2) thrombocytopenia is a significant problem in severe NEC and may occur with or without evidence of fulminant intravascular coagulation.

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Forty-five patients, of whom most were children, underwent extensive exposure of the thoracolumbar spine to correct serious orthopedic abnormalities. The spine was exposed through a combined thoracotomy and retroperitoneal approach that gave excellent access with minimal morbidity. The diaphragm was opened circumferentially after the peritoneum had been dissected from its muscular portion. This permitted repair of the diaphragm with no detectable loss of function. Although this approach was developed for exposure of the spine, it can also be utilized to expose the entire aorta, both kidneys and their blood supply, and the retroperitoneal area for possible excision of large tumors.

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

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Over a three-year period, we have operated on 30 infants with necrotizing enterocolitis. Because of the increased experience with this lesion, we have evolved reliable guidelines for both early diagnosis and operative treatment of necrotizing enterocolitis. Initial therapy was nonsurgical in most cases and included gastrointestinal tract decompression, systemic antibiotics, and correction of fluid, electrolyte, and metabolic abnormalities. Absolute indications for surgery and thought to be prima facie evidence for bowel necrosis were (1) cellulitis of the anterior abdominal wall, (2) free intraperitoneal air, (3) a single dilated intestinal segment present on serial roentgenograms, (4) clinical deteioration in the presence of appropriate medical therapy, and (5) persistent abdominal tenderness. Although all 30 patients operated on had necrotic bowel proved at histologic examination, 21 survived (70%). All patients listed as survivors, with the exception of two, have had intestinal tract continuity reestablished and are doing well.

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