Outcome of congenital diaphragmatic hernia repair depending on patch type.

INTRODUCTION: Patch repair of a congenital diaphragmatic hernia is associated with a much higher rate of recurrence than when primary repair is feasible. The biosynthetic options for the repair materials continue to expand. We therefore reviewed our experience to benchmark complication rates as we progress with the use of new materials. METHODS: A retrospective review was conducted of all patients who underwent repair of congenital diaphragmatic hernia from January 1994 to May 2009. RESULTS: Of the 155 patients included in the study, 101 patients had primary closure and 54 received a diaphragmatic patch. The rates of recurrence, Small Bowel Obstruction (SBO), and subsequent abdominal operation were all significantly higher in the group of patients requiring patch repair. There were 3 types of patch repairs: 37 patients received a SIS patch, 12 had a nonabsorbable patch, and 5 received an AlloDerm patch. The incidence of SBO in patients with a nonabsorbable mesh was 17% and was associated with a 50% recurrence rate and 67% re-recurrence rate. SIS was associated with 19% incidence of SBO, a recurrence rate of 22% and a 50% re-recurrence rate, whereas AlloDerm had a 40% incidence of SBO, 40% recurrence rate, and 100% re-recurrence rate. DISCUSSION: As we move towards the next generation of materials, these data do not justify the continued comparison with nonabsorbable patches. We do not have enough comparative data to define a superior biosynthetic material, but we plan to use our data on SIS to benchmark our experience with future generation materials.

A review of venovenous and venoarterial extracorporeal membrane oxygenation in neonates and children.

The use of extracorporeal membrane oxygenation (ECMO) has increased since its inception. As this modality gained wider acceptance, its application in a variety of disease states has increased. The initial use of ECMO required cannulation of both the carotid artery and internal jugular vein (VA ECMO). Ligation of the carotid artery and concern regarding potential long-term sequelae prompted the development of the single cannula venous only (VV ECMO) technique. Various reports in the literature have compared VV ECMO and VA ECMO. We present a review of the literature with regard to both physiology and clinical application.

The progressive development of pyloric stenosis: a role for repeat ultrasound.

INTRODUCTION: Patients presenting in the first 3 months of life with nonbilious emesis are commonly studied by ultrasound. A negative study effectively rules out pyloric stenosis. However, the development of pyloric stenosis is a progressive and dynamic process. The rate of hypertrophy to the point of meeting diagnostic criteria is unknown and there is no data published in the literature regarding the role of repeat ultrasound in patients with persistent symptoms. During a prospective, randomized trial we identified 3 patients with negative ultrasounds who subsequently were diagnosed and treated for pyloric stenosis. We present this series as an illuminating depiction of the development of muscle hypertrophy in patients with pyloric stenosis. METHODS: Patients with pyloric stenosis and repeat ultrasound were identified from our prospective, randomized trial. All patients had sonographic pyloric measurements obtained at our institution. Data included patient age upon presentation, ultrasound-defined pyloric parameters, operation, and outcome. RESULTS: Three patients were identified with a negative ultrasound with a pyloric thickness ranging from 0.8 mm to 2.5 mm. Subsequent thickness on repeat ultrasound ranged from 3.5 to 6.2 mm. The rate of hypertrophy ranged from 0.17 mm/day to 0.5 mm/day. CONCLUSIONS: A negative pyloric sonogram may be due to the fact that the patient is in the very initial stages of development of pyloric stenosis. Caregivers should counsel parents to return if symptoms persist and there should be a low threshold for repeat ultrasound in these patients.

Experience with laparoscopic adrenalectomy in pediatric patients.

BACKGROUND: Laparoscopic adrenalectomy (LA) is now being recognized as the standard in the management of benign adrenal pathology in adult patients. Few reports have described the use of this technique in pediatric patients. This study combines experience from 2 institutions with lateral transperitoneal LA in children to analyze our results and the clinical and biochemical response to laparoscopic adrenalectomy in patients with hormonally active adrenal tumors. METHODS: A bi-institutional retrospective review of all patients undergoing LA between January 1997 and January 2001 was performed. Clinical and biochemical data were obtained during routine follow-up. RESULTS: Seventeen laparoscopic adrenalectomies were performed during this period. The average operating time was 120 minutes, mean estimated blood loss was 25 mL, the mean size of the adrenal lesion was 4.8 cm, and the mean length of hospitalization was 35 hours. Resolution of clinical and biochemical parameters of adrenal hyperfunction was accomplished in all patients with adrenocortical hyperplasia and pheochromocytoma in postoperative follow-up. CONCLUSIONS: Laparoscopic adrenalectomy can be performed safely and effectively with a short hospital stay, minimal blood loss, and excellent functional outcome in this age group. The authors believe laparoscopic adrenalectomy is an excellent approach for the management of benign pediatric adrenal pathology.

Minimally invasive bar repair for 'redo' correction of pectus excavatum.

BACKGROUND/PURPOSE: A small percentage of patients who have undergone traditional, "Ravitch-type" pectus excavatum repair present with unsatisfactory results and require a second procedure for correction. Reoperative open surgery for pectus excavatum has been associated with extensive dissection and substantial blood loss. The minimally invasive (MIS) bar repair for the correction of pectus excavatum has been gaining acceptance. This study evaluates the authors results with patients who have undergone the MIS bar repair for redo correction of their pectus excavatum. METHODS: A retrospective chart review of all patients undergoing MIS bar repair between December 1997 and August 2001 was performed. Information about demographics, deformity, operative course, complications, and early outcome was recorded. RESULTS: Ninety-two patients underwent MIS repair during this period. Ten patients had redo MIS bar repair for unsatisfactory prior open correction. Operating time was 52 minutes for standard patients and 70 minutes for the redo patients (P <.001). Blood loss and postoperative hospitalization were similar between groups. CONCLUSION: The minimally invasive pectus repair can be performed safely with minimal blood loss and short operating time in patients who have undergone prior unsatisfactory open repair of pectus excavatum and can be an alternative approach to reoperative open repair in these patients.

Omphalocele in three generations with autosomal dominant transmission.

We report a family with nine subjects over three generations affected with an omphalocele requiring surgical intervention within the first few days of life. Because of the vertical transmission and male to male inheritance in our family, we conclude that an autosomal dominant gene caused the omphalocele in the affected family members. The paternal great grandfather of the proband was not clinically affected but produced two children with omphaloceles with different spouses.

Physiological aspects of pig-to-primate renal xenotransplantation.

BACKGROUND: Few data exist on the physiological aspects of pig-to-primate renal xenotransplantation. METHODS: Use of organs transgenic for human decay accelerating factor has allowed assessment of the metabolic and hormonal functions of these xenografts. RESULTS: Porcine renal xenografts largely maintain plasma electrolyte homeostasis. An increase in proteinuria was detected that may result from graft injury. In contrast to allotransplantation a severe anaemia developed requiring recipient treatment with exogenous human erythropoietin. CONCLUSIONS: Our experience provides qualified encouragement for the likely physiological compatibility of pig and primate species, but identifies areas where a xenograft may not match the performance of an allograft.

Life supporting function for over one month of a transgenic porcine heart in a baboon.

BACKGROUND: Inhibition of hyperacute rejection (HAR) and sustained graft survival have been demonstrated in a pig-to-primate model of heterotopic cardiac xenotransplantation using pigs transgenic for human Decay Accelerating Factor (hDAF). Building on this work, an orthotopic model has been developed. This case records 39-day cardiac xenograft function in a life-supporting capacity with clinically applicable immunosuppression. METHODS: Using a heart from an hDAF transgenic pig, an orthotopic cardiac transplant was performed on an adult baboon. The immunosuppressive regimen consisted of induction with a short course of cyclophosphamide, followed by maintenance therapy with cyclosporine A, mycophenolate mofetil and a tapering course of corticosteroids. Post-operative monitoring included daily anti-pig hemolytic antibody titer surveillance and endomyocardial biopsy. RESULTS: The animal survived 39 days and was active and energetic throughout its postoperative course, remaining free of signs of cardiopulmonary failure. Endomyocardial biopsy performed on post-operative Day 36 revealed only patches of sub-endocardial fibrosis with no signs of active rejection. The baboon succumbed to an acute cardiopulmonary decompensation immediately following administration of medication via oral gavage. Post-mortem histopathology demonstrated well-preserved myocardial architecture with small foci of mild humoral rejection. CONCLUSIONS: This case documents the longest survival recorded to date of a discordant orthotopic cardiac xenograft and illustrates that the hDAF transgene combined with a clinically acceptable maintenance immunosuppressive regimen enables sustained, life-supporting function of porcine cardiac xenografts in non-human primates. The inhibition of hyperacute rejection and the subsequent control of humoral and cellular rejection for over 1 month demonstrated in this experiment represent significant progress in the development of a viable strategy for clinical xenotransplantation.

Undescended testicle associated with spigelian hernia.

The authors encountered a patient with a spigelian hernia (SH) and undescended testicle (UDT), making the fourth reported case with this combination. This patient provides further evidence that congenital SH predisposes neonates to UDT.