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Background: A multicenter study of pectus excavatum was described previously. This report presents our final results., Study Design: Patients treated surgically at 11 centers were followed prospectively. Each underwent a preoperative evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, complications, and perioperative pain. One year after treatment, patients underwent repeat chest CT scan, pulmonary function tests, and body image survey. A subset of 50 underwent exercise pulmonary function testing., Results: Of 327 patients, 284 underwent Nuss procedure and 43 underwent open procedure without mortality. Of 182 patients with complete follow-up (56%), 18% had late complications, similarly distributed, including substernal bar displacement in 7% and wound infection in 2%. Mean initial CT scan index of 4.4 improved to 3.0 post operation (severe >3.2, normal = 2.5). Computed tomography index improved at the deepest point (xiphoid) and also upper and middle sternum. Pulmonary function tests improved (forced vital capacity from 88% to 93%, forced expiratory volume in 1 second from 87% to 90%, and total lung capacity from 94% to 100% of predicted (p < 0.001 for each). VO2 max during peak exercise increased by 10.1% (p = 0.015) and O2 pulse by 19% (p = 0.007) in 20 subjects who completed both pre- and postoperative exercise tests., Conclusions: There is significant improvement in lung function at rest and in VO2 max and O2 pulse after surgical correction of pectus excavatum, with CT index >3.2. Operative correction significantly reduces CT index and markedly improves the shape of the entire chest, and can be performed safely in a variety of centers., (Copyright © 2013 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: This study evaluated changes in both physical and psychosocial quality of life reported by the parent and child after surgical repair of pectus excavatum., Methods: As part of a multicenter study of pectus excavatum, a previously validated tool called the Pectus Excavatum Evaluation Questionnaire was administered by the research coordinator, via telephone, to parents and patients (8-21 years of age) before and 1 year after surgery. Eleven North American children's hospitals participated. From 2001 to 2006, 264 patients and 291 parents completed the initial questionnaire, and 247 patients and 274 parents completed the postoperative questionnaire. Responses used a Likert-type scale of 1 to 4, reflecting the extent or frequency of a particular experience, with higher values conveying less-desirable experience., Results: Preoperative psychosocial functioning was unrelated to objective pectus excavatum severity (computed tomographic index). Patients and their parents reported significant positive postoperative changes. Improvements occurred in both physical and psychosocial functioning, including less social self-consciousness and a more-favorable body image. For children, the body image component improved from 2.30+/-0.62 (mean+/-SD) to 1.40+/-0.42 after surgery and the physical difficulties component improved from 2.11+/-0.82 to 1.37+/-0.44. For the parent questionnaire, the child's emotional difficulties improved from 1.81+/-0.70 to 1.24+/-0.36, social self-consciousness improved from 2.86+/-1.03 to 1.33+/-0.68, and physical difficulties improved from 2.14+/-0.75 to 1.32+/-0.39. Ninety-seven percent of patients thought that surgery improved how their chest looked., Conclusions: Surgical repair of pectus excavatum can significantly improve the body image difficulties and limitations on physical activity experienced by patients. These results should prompt physicians to consider the physiologic and psychological implications of pectus excavatum just as they would any other physical deformity known to have such consequences.

Background: Given widespread adoption of the Nuss procedure, prospective multicenter study of management of pectus excavatum by both the open and Nuss procedures was thought desirable. Although surgical repair has been performed for more than 50 years, there are no prospective multicenter studies of its management., Study Design: This observational study followed pectus excavatum patients treated surgically at 11 centers in North America, according to the method of choice of the patient and surgeon. Before operation, all underwent evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, hospital complications, and perioperative pain. One year after completion of treatment, patients will repeat the preoperative evaluations. This article addresses early results only., Results: Of 416 patients screened, 327 were enrolled; 284 underwent the Nuss procedure and 43 had the open procedure. Median preoperative CT index was 4.4. Pulmonary function testing before operation showed mean forced vital capacity of 90% of predicted values; forced expiratory volume in 1 second (FEV(1)), 89% of predicted; and forced expiratory flow during the middle half of the forced vital capacity (FEF(25% to 75%)), 85% of predicted. Early postcorrection results showed that operations were performed without mortality and with minimal morbidity at 30 days postoperatively. Median hospital stay was 4 days. Postoperative pain was a median of 3 on a scale of 10 at time of discharge; the worst pain experienced was the same as was expected by the patients (median 8), and by 30 days after correction or operation, the median pain score was 1. Because of disproportionate enrollment and similar early complication rates, statistical comparison between operation types was limited., Conclusions: Anatomically severe pectus excavatum is associated with abnormal pulmonary function. Initial operative correction performed at a variety of centers can be completed safely. Perioperative pain is successfully managed by current techniques.

The occurrence of ectopic gastric mucosa in Meckel's diverticula and duplications of the intestinal tract is a source of serious morbidity from bleeding and perforation in children. Intraoperative localization of the ectopic mucosa is aided by its occurrence in the duplicated intestinal segment or Meckel's diverticulum. However, its identification in the nonduplicated intestine poses problems for the surgeon faced with removal of the affected segment. The authors present a case of ectopic gastric mucosa in a nonduplicated jejunal segment and discuss the use of technetium 99m pertechnetate scanning in its localization., (Copyright 2001 by W.B. Saunders Company.)

Both pigmentation and otic defects of Waardenburg Syndrome and Hirschsprung's disease have a common origin in neural crest cells and were described in 1951 and 1887, respectively. The clinical manifestations of both in the same patient were described in 1981 in 12 infants so afflicted. The authors present such a case of long segment aganglionosis in a 15-day-old Marshallese girl with Waardenburg-Shah syndrome and discuss diagnosis, treatment, and prognosis.

Purpose: The aim of this study was to examine the efficacy of the antirefluxing, mucosal-flap valve (AMFV) for biliary drainage relative to technical feasibility, surgical complications, and incidence of ascending cholangitis (AC)., Methods: Twenty-seven infants requiring biliary tract reconstruction underwent valve construction. Twenty biliary atresia (BA) patients received the Kasai procedure, and 7 choledochal cyst (CC) infants had cystectomy and hepatoenterostomy. A retrospective review of all patients was performed including radiographic evaluation of the current valve function in 10 patients., Results: Construction was successful in all cases, and no morbidity was incurred by incorporation of the valve. Of 7 CC patients, there have been no known episodes of AC with mean follow-up of 4.4+/-4.2 years. Of 20 BA patients, there have been 5 deaths (25%), 7 liver transplants (35%), 2 (10%) lost to follow-up, and 6 (30%) survivors. Nine BA patients (45%) have had AC, with patients in all 4 outcome categories represented. Ten patients (5 CC and 5 BA) have been evaluated with barium small bowel radiographs, with no reflux to the liver hilum in all cases., Conclusions: The AMFV has caused no morbidity and continues to prevent reflux to the liver hilum. Despite functioning as designed, it does not appear to influence the occurrence of AC. Because CC patients had no AC, we feel that infection is related to the underlying atresia rather than to reflux.

In five dogs, we have created an anti-refluxing Roux en-Y jejunal anastomosis by removing the antimesenteric half of the seromusculature from five cm of the biliary limb proximal to the anastomosis prior to coating the gastric and biliary limbs. In the presence of increasing intraluminal pressure in the gastric and vertical limbs of the Y, the wall of the biliary limb collapses and occludes the lumen, acting as a nonrefluxing one-way valve. All three limbs of the Y were then exteriorized as enterostomies. Saline was instilled into either the gastric or distal limb with the other clamped. There was never reflux from the biliary limb despite intraluminal pressures of 200 mm Hg. We have used the anti-refluxing valve in seven infants. Three are alive at 17, 7, and 2 months postoperatively, one of which had a brief episode of fever without leukocytosis or increasing jaundice. Three have died of unresolved obstruction and one has been lost to follow-up.