Adam J. Kaye

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.

Effect of Kasai procedure on hepatic outcome in Alagille syndrome.

Objectives: Alagille syndrome (AGS) frequently presents with neonatal jaundice and can mimic other causes of high γ-glutamyl transpeptidase (GGT) cholestasis, most notably biliary atresia. As a result infants with AGS may undergo intraoperative cholangiogram and even Kasai procedure. The aim of the study was to assess the hepatic outcomes of children with AGS who underwent the Kasai procedure. Patients and Methods: A retrospective review of the AGS clinical database at the Childrens Hospital of Philadelphia was performed to identify clinically defined patients with AGS who underwent a Kasai. A cohort of Alagille control subjects was selected with equivalent symptoms of neonatal jaundice and matched for age and presence of cardiac anomaly. JAGGED1-mutation analysis was performed on available samples. Clinical courses were reviewed. Fisher exact and t tests were used for analysis. Results: Of the 430 patients with AGS, 19 underwent a Kasai procedure (K). The control cohort (C) consisted of 36 patients. Total bilirubin measured between 6 and 10 weeks of age in each cohort was equivalent (K: 9.6 mg/dL, C: 8.7 mg/dL); GGT levels were higher in the control group (K:493.4 U/L, C:574.4 U/L). Of note, the Kasai cohort had a significantly larger number of liver transplants (K: 9 [47.3%], C: 5 [13.9%], P = 0.01) and sustained higher mortality (K: 6 [31.6%], C: 1 [2.8%], P = 0.005). There was no genotype-phenotype correlation between the mutations identified and patients who underwent Kasai. Conclusions: These data suggest that the Kasai procedure, although appropriate for children with biliary atresia, does not benefit children with AGS and actually appears to worsen outcome. The current data suggest that the Kasai is not a marker for underlying severe liver disease, but the procedure itself may have a detrimental effect on outcome. An appropriate medical evaluation and particular consideration of AGS is essential before surgical referral in infants with high GGT cholestasis.

Esophageal Perforation in Children: A Review of One Institution's Experience

BACKGROUND The current approach to esophageal perforation treatment in children has shifted towards conservative management. However, the consensus of what constitutes conservative management is unclear, with various therapies and protocols described, including the need for various decompression and drainage procedures. Our institution utilizes conservative management with minimal intervention guided by the patients clinical course. The purpose of this study is to report our management and add to the growing evidence for conservative management of esophageal perforation in children. METHODS We performed a retrospective chart review of all patients with an ICD-9 diagnosis of esophageal perforation from January 1995 to July 2009. Patients with postoperative anastomotic leaks with drains in place were excluded, although patients with anastomotic leaks that were not controlled by drains were included. Data collected included patient demographics, etiology, diagnosis, treatment, complications, and outcome. RESULTS Eight patients were identified who met inclusion criteria. Mean age was 28 mo (1 d-10 y), and the average time from causative event to diagnosis was 1.4 d (0-2 d). The etiology for esophageal perforation included esophagoscopy with dilation (n = 4), button battery ingestion (n = 1), coin ingestion (n = 1), nasogastric tube placement (n = 1), and leak after stricture resection (n = 1). All the patients were treated conservatively without primary surgery or thoracic drainage, and the mean time to perforation healing was 10.2 d (1-24 d). The average length of antibiotic therapy was 10 d (0-26 d). Enteral nutrition was utilized in five patients, and total parenteral nutrition (TPN) was utilized in five patients. No patient developed a new-onset esophageal stricture. CONCLUSION Conservative management, guided by the patients clinical course, with antibiotics and nutritional support is a safe and effective treatment for esophageal perforations in children.

Use of laparoscopic cholecystectomy for biliary dyskinesia in the child.

BACKGROUND Biliary dyskinesia (BD) is a consideration as a cause of chronic abdominal pain in the pediatric population. We sought to correlate the results of cholecystokinin-diisopropyl iminodiacetic acid (CCK-DISIDA) scanning, the basis for diagnosis of BD, with outcome after laparoscopic cholecystectomy. METHODS A retrospective review was performed of all patients who underwent a laparoscopic cholecystectomy from May 2000 through March 2007. The diagnosis of BD was based on CCK-DISIDA scan demonstrating a gallbladder ejection fraction (GBEF) of less than 35% and/or reproduction of pain on CCK administration or no filling of the gall bladder with a normal ultrasound examination. Hospital, General Surgery office, and Gastroenterology Office charts were reviewed for demographic and management data points. We used chi(2) and Mann-Whitney tests for statistical analysis. RESULTS For the period of review, 430 patients underwent a laparoscopic cholecystectomy including 75 patients with a preoperative diagnosis of BD. The mean age of the BD population was 14 (range, 9-19) years. Female to male ratio was 2.4:1. The mean body mass index was 24.4 kg/m(2). On average, patients had abdominal symptoms for 15.5 (range, 0.25-72) months. Each patient underwent nearly 2.5 studies (computed tomography, ultrasound, esophagogastroduodenoscopy, or upper gastrointestinal series) before diagnosis by CCK-DISIDA. The mean GBEF was 17.4%. When commented on (n = 41), pain on CCK administration was noted in 25 (61%) patients. Pathology revealed chronic cholecystitis in 44%. After laparoscopic cholecystectomy, 58 (77.33%) patients reported resolution of their abdominal pain (mean follow-up 4 months). Of the 17 patients without improvement, 7 were later diagnosed with other underlying pathology (Crohns, hiatal hernia, cyclic vomiting). There was no difference in GBEF, age, histopathology, or sex between the two groups. There were no complications. CONCLUSION Laparoscopic cholecystectomy is a safe and effective treatment for the majority of children diagnosed with BD. Although CCK-DISIDA was used to identify biliary dysfunction, it did not correlate with outcome.

Complex vascular reconstruction of abdominal aorta and its branches in the pediatric population

BACKGROUND Subdiaphragmatic aortic diseases in children are rare and form a heterogeneous group. The pediatric patient presents unique challenges because of their size, concerns about proper timing and conduit for repair, and anticipating expected growth. METHODS We performed a retrospective review of operations involving the abdominal aorta and called branches in children between January 2003 and April 2007, focusing on the details of preoperative evaluation, operative technique, and outcomes. The pertinent literature is reviewed. RESULTS Twenty-two children (age, 2 days to 17 years) were included. Mean follow-up was 28 months. Aneurysms were seen in 5 children; the remainder had stenotic disease. Aneurysms were typically asymptomatic and diagnosed incidentally, whereas stenotic lesions most commonly presented with hypertension (HTN). Fourteen complex vascular repairs were performed. All of the children with aneurysms underwent prompt surgery. The children with stenoses had operations for poorly controlled HTN, claudication, and/or mesenteric ischemia. Most patients with stenotic disease were treated medically for HTN and were followed closely while awaiting optimal size and availability of autogenous conduit for reconstruction. Cryopreserved allograft was used in 3 of the aneurysm operations. Dacron grafts were used to repair 5 aortic stenotic lesions. Renal and mesenteric revascularizations were performed with saphenous vein grafts. Pediatric, general, and transplant surgeons and nephrologic and cardiologic teams were integral to evaluation and management. No major operative complications occurred. CONCLUSION Proper management of pediatric aortic vascular disease requires a multidisciplinary approach. It is best to use autologous grafts whenever possible. Children with stenotic disease should be treated medically for hypertension until they are large enough for an autologous graft reconstruction. Children with aneurysmal disease are at risk for embolism and thrombosis and therefore usually treated immediately using artificial graft material, if necessary.

Mild Traumatic Brain Injury in the Pediatric Population: The Role of the Pediatrician in Routine Follow-Up

BACKGROUND Traumatic brain injury is common in children. Fortunately, most patients suffer mild traumatic brain injury (MTBI). Appropriate guidelines for follow-up care are not well established. We sought to determine practice experience and preferences of general pediatricians related to follow-up care of MTBI. METHODS Members of the American Academy of Pediatrics Council of Community Pediatrics and general pediatricians in the Pennsylvania Chapter of the American Academy of Pediatrics participated in a web-based survey regarding practice setting, level of comfort caring for patients with MTBI, and referral patterns for such patients. RESULTS A total of 298 pediatricians responded. An urban or suburban practice setting was reported by 83.3% with a wide distribution in practice experience (0-10 years 40.5%, 11-20 years 24.5%, >21 years 35%). Most respondents (54.5%) had cared for at least 2 to 5 patients with MTBI in the past 6 months but only 8% had seen >10 patients. Fifty-nine percent had not participated in continuing medical education activities related to MTBI and 62.2% did not use neurocognitive tests. The majority (89%) thought that they were the appropriate provider for follow-up; this declined to 61.2% for patients with loss of consciousness and only 5.4% if patients had persistent symptoms. Neurologists (75%) were the consultant of choice for referral. Increased practice experience was associated with an increased comfort in determining return to play status. CONCLUSION In this survey, pediatricians thought that they were the most appropriate clinicians to follow-up patients with MTBI. However, most accepted this responsibility without the benefit of specific continuing medical education or using neurocognitive tests. Ensuring the availability of appropriate resources for pediatricians to care for these patients is important.

Sternal wound reconstruction: management in different cardiac populations.

Poststernotomy mediastinitis is a feared complication for patients undergoing cardiac surgery associated with high rates of morbidity and mortality. Approximately 15% of patients will ultimately be readmitted for a recurrent sternal wound infection. The objective of this study is to review a large single surgeon experience with sternal wound patients managed with a variety of soft tissue flaps to assess mitigating factors, involved organisms, and treatment protocols as related to specific cardiac populations. Records for 136 sternal reconstruction patients treated from January 2000 to July 2007 were evaluated. Patients underwent a variety of cardiac surgeries including coronary artery bypass grafting (CABG), valve replacement, aortic reconstruction, heart transplantation, lung transplantation, and combinations of these procedures. A total of 39.2% of patients developed a sternal wound during the same admission as their cardiac surgery, at an average of 16.1 days. This rate was only 6% for CABG-only patients and rose to nearly 50% in heart transplant and CABG + valve patients. A total of 78.6% of heart transplant patients with a sternal wound had a history of ventricular assist device and 41% of all patients had at least 1 previous sternotomy. Thirteen patients (9.6%) had 1 or more recurrent infections requiring surgery; 50% occurring in transplant patients, most of whom had diabetes and/or renal insufficiency. The most common presenting symptom was drainage (n = 75, 55.6%) or wound dehiscence (n = 22, 16.3%). Twenty-five different organisms were identified; 26 patients (18.5%) had multiple organisms. Staphylococcus species were most common. Plastic surgery intervention occurred on average 109.2 days after cardiac surgery. CABG and CABG + valve patients most frequently received right pectoralis muscle turnover flaps or left pectoralis muscle advancement flaps. Ten heart transplant patients (37.0%) underwent omental flaps. The 30-day perioperative mortality rate was 13 patients (9.6%).

Improving Radiologist-IT Staff Communications and Collaboration Through a Shadowing Project.

It is important for radiology practices to have efficient and skillful IT staff to provide support for issues that arise during patient care. However, an anecdotal barrier exists between radiologists and IT staff that can hinder the delivery of this care, either by lengthening the time required to solve problems with the IT infrastructure or not bringing critical issues to the IT staff’s attention. We first created a survey of the radiologists and IT staff in our department to investigate this barrier, and found that there was, at baseline, a significant difference in how one group viewed the other, and, at times, there were irrational expectations from either party of what their counterparts were responsible for or capable of. We then instituted a shadowing project, whereby radiologists shadowed IT staff and vice versa, in order to give each group a better understanding of the others’ daily workflows. We gave the participants a post-intervention survey to assess their experiences, which were markedly positive. In one case, an IT issue regarding pre-fetching of cardiovascular studies was, in fact, solved during the shadowing session.

Umbilical Diorders and Anomalies

An abnormal umbilicus represents one of the most frequent indications for pediatric surgical consultation. In the newborn, the majority of umbilical abnormalities are related to the embryologic development and function of the umbilicus. The formation of the umbilical region of the abdominal wall begins in the fourth week of gestation. The embryo attaches to the primitive placenta by a stalk that will eventually house the umbilical vessels and the allantois, which together form the umbilical cord. The vitelline duct also briefly runs within this stalk connecting the yolk sac as it divides into intracoelomic and extracoelomic portions by the in-folding embryo. The intracoelomic portion forms the alimentary canal and the remaining extracoelomic yolk sac feeds the embryo through the vitelline duct. This duct normally closes between the fifth and seventh week of gestation as the placenta begins to provide nutritional support to the embryo.