Increasing severity of pectus excavatum is associated with reduced pulmonary function.

OBJECTIVE: To determine whether pulmonary function decreases as a function of severity of pectus excavatum, and whether reduced function is restrictive or obstructive in nature in a large multicenter study. STUDY DESIGN: We evaluated preoperative spirometry data in 310 patients and lung volumes in 218 patients aged 6 to 21 years at 11 North American centers. We modeled the impact of the severity of deformity (based on the Haller index) on pulmonary function. RESULTS: The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), forced expiratory flow from 25% exhalation to 75% exhalation, and total lung capacity findings increased with increasing Haller index score. Less than 2% of patients demonstrated an obstructive pattern (FEV(1)/FVC <67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV(1) <80% predicted; FEV(1)/FVC >80%). Patients with a Haller index of 7 are >4 times more likely to have an FVC of ≤80% than those with a Haller index of 4, and are also 4 times more likely to exhibit a restrictive pulmonary pattern. CONCLUSIONS: Among patients presenting for surgical repair of pectus excavatum, those with more severe deformities have a much higher likelihood of decreased pulmonary function with a restrictive pulmonary pattern.

Pectus deformities: a review of open surgery in the modern era.

Pectus deformities represent a spectrum of relatively common congenital chest malformations. The adoption of less invasive techniques has renewed interest in surgical repair by both patients and clinicians. The aim of this review is to identify current management, outcomes, and controversy in the treatment of pectus excavatum and pectus carinatum.

Prospective multicenter study of surgical correction of pectus excavatum: design, perioperative complications, pain, and baseline pulmonary function facilitated by internet-based data collection.

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.

Multicenter study of pectus excavatum, final report: complications, static/exercise pulmonary function, and anatomic outcomes.

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.

Surgical repair of pectus excavatum markedly improves body image and perceived ability for physical activity: multicenter study.

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.

Ethics and the pediatric surgeon.

PURPOSE: Care of infants and children with life-impairing or life-threatening congenital and acquired disorders often raises ethical concerns for pediatric surgeons. The purpose of this survey was to determine the level of interest in clinical ethics and how respondents would manage ethical dilemmas within several clinical case scenarios. METHODS: A 12-item validated questionnaire developed by the Ethics and Advocacy Committee was provided for the American Pediatric Surgical Association (APSA; www.eapsa.org) members on the organizational website. General categories of questions included informed consent, patient privacy, and what constitutes research. RESULTS: The survey was completed by 235 of the 825 APSA members; a response rate of 28.4%. The majority (62%) were in academic practice, 22% had additional education or an advanced degree in ethics, and 11% were members of a hospital ethics committee. There was a clear majority response for seven questions. Topics generating the most controversy included the impact of consent by minors, decision making in the neurologically devastated child, what constitutes research in pediatric surgery, the use of interpreters for consent, and patient privacy. Respondents chose a well-referenced manuscript as the preferred modality for ethics education of the APSA members. CONCLUSION: Pediatric surgeons have a general interest in clinical ethics as it relates to the care of their patients. An important mission of the Ethics and Advocacy Committee can be to provide education that gives guidance and knowledge to the members of APSA on timely topics in surgical ethics.

The optimal timing of intestinal transplantation for children with intestinal failure: a Markov analysis.

OBJECTIVE: Identify an optimal approach to the timing of intestinal transplantation for children dependent on total parenteral nutrition (PN). SUMMARY BACKGROUND DATA: Children with short bowel syndrome are frequently dependent on PN for growth and development. Intestinal transplantation is often considered after PN-related complications occur, but optimal timing of transplantation is controversial. METHODS: A Markov analytic model was used to determine life expectancy (LY) and quality-adjusted life years on a theoretical cohort of 4-year-old subjects for two treatment strategies: (1) standard care consisting of PN and referral to transplantation according to accepted guidelines and (2) early listing for isolated small intestine transplantation. RESULTS: Early listing for intestinal transplantation was associated with 0.27 additional life years (13.16 vs. 12.89) and 0.76 additional quality-adjusted life years (10.51 vs. 9.75) as compared with current standard care. The unadjusted analysis was sensitive to the development of PN-associated liver disease, at a threshold of approximately 11% per year, and its related probability of dying at a threshold of 80% 2-year mortality. Early listing for transplantation was the dominant strategy until the probability of late bowel rejection reached 35% per year. CONCLUSIONS: Children with short bowel syndrome dependent on PN should be considered for intestinal transplantation earlier than what is current practice.

Sirolimus for pediatric liver transplant recipients with post-transplant lymphoproliferative disease and hepatoblastoma.

Sirolimus is a promising immune suppressive agent, with the potential to reduce calcineurin inhibitor associated nephrotoxicity, halt progression of chronic rejection and prevent tumor proliferation. The aim of this study was to review the experience using sirolimus in pediatric liver transplant recipients at a single center. Database and medical charts of all pediatric liver transplant recipients receiving sirolimus at the Hospital for Sick Children in Toronto were reviewed. Eight patients received sirolimus between October, 2000 and September, 2002. Indications for using sirolimus were post-transplant lymphoproliferative disease (PTLD) (n = 6) and hepatoblastoma (n = 2). Two patients with PTLD concurrently had renal impairment and chronic rejection. Sirolimus dosages ranged between 1.5 and 5 mg once daily. Median duration of follow-up was 17 months. Persistently elevated liver transaminase levels in the two children with chronic rejection decreased during sirolimus therapy. Recurrence of PTLD occurred in one patient. Two patients were diagnosed with acute cellular rejection after transition to maintenance sirolimus monotherapy. Resolution of adverse effects including mouth sores (n = 3), leg swelling (n = 2) and hyperlipidemia (n = 3) occurred either spontaneously or with dose reduction. Sirolimus was discontinued in four patients because of persisting bone marrow suppression, interstitial pneumonitis, life-threatening sepsis and refractory diarrhea. Children with PTLD or hepatoblastoma may benefit from immune suppression with sirolimus after liver transplantation. Further multi-center, prospective, randomized controlled trials will be instrumental to further the knowledge of long-term efficacy, safety and tolerability of sirolimus for selected children following liver transplantation.

ABO-incompatible liver transplantation with no immunological graft losses using total plasma exchange, splenectomy, and quadruple immunosuppression: evidence for accommodation.

ABO-incompatible liver transplants (LTX) have been associated with a high risk of antibody-mediated rejection, poor patient and graft survival, and a high risk of vascular thrombosis and ischemic bile duct complications. We used pretransplantation and posttransplantation double-volume total plasma exchange (TPE), splenectomy, and quadruple immunosuppression (cyclophosphamide or mycophenolate mofetil, prednisone, cyclosporine or tacrolimus, and OKT3 induction) in 14 patients receiving ABO-incompatible LTX between June 1992 and February 2001: A(1) to O (seven), B to O (two), B to A (two), A to B (one), AB to A (one), and AB to O (one). Actuarial 1- and 5-year patient and graft survival rates are 71.4% and 61.2 % and 71.4% and 61.2%, respectively, with a mean follow-up of 62.9 +/- 39.4 months. Ten acute cellular rejections occurred, and the mean time to the first episode was 62 +/- 33 days. All were steroid sensitive. No antibody-mediated rejection or vascular thromboses occurred. Pretransplantation pre-TPE immunoglobulin (Ig) G mean isohemagglutinin titers were 262 +/- 326, compared with pretransplantation post-TPE titers of 65 +/- 103 (P =.04). Eight of nine patients with measurable titers before and after TPE achieved a reduction in titers. The mean number of posttransplantation TPE was 5.5 +/- 4.1 (range, 0 to 12), and the last TPE was on postoperative day 9.4 +/- 5.3. IgG isohemagglutinin titers 2 weeks posttransplantation had increased to 153 +/- 309 (P =.03 compared with pretransplantation pre-TPE IgG). ABO-incompatible liver transplantations can be performed with acceptable patient and graft survival rates with a low risk of antibody-mediated rejection with a combination of TPE, splenectomy, and quadruple immunosuppression. Recovery of isohemagglutinin antibody levels without humoral rejection suggests that accommodation may be the protective mechanism preventing late antibody-mediated rejection.