Cathy E. Shin

Laparoscopic Choledochal Cyst Excision: Lessons Learned in Our Experience

BACKGROUND Choledochal cyst (CDC) is a rare biliary disorder. Surgical treatment consists of CDC excision and biliary-enteric reconstruction. Recently, some institutions have reported successful CDC excision by using minimally invasive techniques. In this study, we report our experience with the laparoscopic management of CDC, with a focus on key operative maneuvers that enhance the likelihood of successful excision. METHODS Following institutional review board approval, we performed a retrospective review of patients who underwent the laparoscopic excision of CDC and Roux-en-Y hepaticojejunostomy. Between October 2003 and November 2007, we performed laparoscopic CDC excision in 9 patients (8 female and 1 male). Median age was 4 years (range, 8 months to 16 years). There were 7 type I and 2 type IV cysts, according to Todanis classification. Average cyst size was 4.4 cm (range, 1.3-8.5). The procedures were performed by utilizing four or five trochars. RESULTS Six of 9 children presented with preoperative pancreatitis, 1 with abdominal pain, 1 with jaundice, and 1 was found incidentally. Three patients required the conversion to laparotomy due to dense adhesions, secondary to pancreatitis. Six patients underwent successful laparoscopic procedures, 5 had complete cyst excisions, and 1 underwent a proximal excision with distal mucosectomy. Of the 3 patients who required conversion, 2 underwent complete excisions; the other underwent a proximal excision, distal mucosectomy. There were no intraoperative complications. One patient had a postoperative bile leak that required an open hepaticojejunostomy revision. Eight patients had an uneventful recovery. Oral feedings were resumed within an average of 3.4 days (range, 2-9). Average time to discharge was 6.1 days (range, 5-12). Average follow-up time was 18 months (range, 4-48). No further laboratory abnormalities were detected in any of the patients. CONCLUSIONS Laparoscopic resection of CDC and Roux-en-Y hepaticojejunostomy in children is an excellent treatment option. Preoperative pancreatitis may cause increased technical difficulty, necessitating a conversion. Proximal excision with distal mucosectomy

Contemporary management of lipoblastoma

PURPOSE Lipoblastoma is a rare, benign, adipose tissue tumor. We report the largest single institution experience managing these uncommon neoplasms. METHODS We retrospectively reviewed 32 cases of lipoblastoma entered in the pathology database at our institution between January 1991 and August 2005. We conducted a comprehensive literature review of lipoblastoma and summarized the results of the largest series published. RESULTS Most patients presented with an enlarging, palpable, firm, nontender, mobile mass. The male-to-female ratio was 1.9:1. The anatomical distribution was trunk (n = 12), extremity (n = 12), groin (n = 5), and neck (n = 3). Average age at resection was 2.8 years (range, 2.6 months to 12 years). Thirty-one cases were completely excised, although 1 patient underwent staged partial excision to preserve nerve function. Chromosomal analysis performed in selected patients revealed characteristic aberrations in chromosome 8. Complications included keloid formation (n = 3), wound infection/dehiscence (n = 2), wound seroma (n = 1), and transient brachial plexus neurapraxia (n = 1). Average follow-up was 7.4 months (range, 1 day to 6.5 years); 2 patients were lost to follow-up. There were no recurrences. CONCLUSIONS A staged approach with meticulous sparing of the neurovascular bundle provides excellent functional outcome for patients with large tumors. Nonmutilating surgical excision is the treatment of choice.

Thoracoscopic Repair of Neonatal Diaphragmatic Hernia

INTRODUCTION The use of minimally invasive surgery (MIS) in the neonatal population is increasing. Thoracoscopic intervention for congenital diaphragmatic hernia (CDH) is no exception. In this report, we describe our initial experience with thoracoscopic repair of left-sided diaphragmatic defects in neonates. MATERIALS AND METHODS We performed retrospective chart reviews on all neonates who underwent thoracoscopic repair of CDH between November 2004 and January 2008. Neonates that underwent thoracoscopic repair were physiologically stable with resolved pulmonary hypertension and minimal to moderate ventilatory support. They had no associated cardiac anomalies. RESULTS We identified 15 neonates with CDH who underwent thoracoscopic repair during the study period. Ten neonates underwent primary repair of the diaphragmatic defect. Five neonates with large defects required closure with a synthetic patch, which was placed thoracoscopically. The average operating room time was 134 minutes. There were no instances of intraoperative respiratory or cardiac instability. Three patients had a recurrence. One recurrence was seen after thoracoscopic patch repair. Two recurrences occurred following primary repair of left diaphragmatic hernias. There were no deaths. Follow-up has been 4-40 months. CONCLUSIONS Neonatal MIS for CDH should be limited to stable patients. The ideal candidate is the newborn without associated anomalies, not requiring extracorporeal membrane oxygenation, on minimal ventilatory support, and without evidence of pulmonary hypertension. It is technically possible to perform thoracoscopic repair with a patch.

Laparoscopic Versus Open Treatment of Congenital Duodenal Obstruction: Multicenter Short-Term Outcomes Analysis

BACKGROUND Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair. PATIENTS AND METHODS Medical records for all cases of congenital duodenal obstruction from 2005 to 2011 at three academic teaching hospitals were retrospectively reviewed. Patients were excluded from the analysis if they had confounding surgical diseases, did not have duodenoduodenostomy during the first hospital admission, had the repair performed before transfer from a referring hospital, or weighed less than 1.7  kg at the time of surgery. Analysis was performed as intention to treat, with laparoscopic converted to open cases included in the laparoscopic group. RESULTS Sixty-four cases were included in the analysis (44 open, 20 laparoscopic). Baseline characteristics were similar between the two groups with the exception that the open group, on average, underwent repair later than the laparoscopic group (6 days versus 4 days, respectively). Seven laparoscopic cases were converted to an open procedure (35%), most commonly for difficulty in exposing the decompressed distal duodenum. Laparoscopic repair did take significantly longer than open repair (145 minutes versus 96 minutes, respectively), but clinical outcomes were similar. Complications were rare and were similar between methods of repair. Two patients in the laparoscopic group required subsequent open revision. CONCLUSIONS Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.

State of the practice for pediatric surgery—career satisfaction and concerns. A report from the American Pediatric Surgical Association Task Force on Family Issues

BACKGROUND There has been increasing interest and concern raised in the surgical literature regarding changes in the culture of surgical training and practice, and the impact these changes may have on surgeon stress and the appeal of a career in surgery. We surveyed pediatric surgeons and their partners to collect information on career satisfaction and work-family balance. METHODS The American Pediatric Surgical Association Task Force on Family Issues developed separate survey instruments for both pediatric surgeons and their partners that requested demographic data and information regarding the impact of surgical training and practice on the surgeons opportunity to be involved with his/her family. RESULTS We found that 96% of pediatric surgeons were satisfied with their career choice. Of concern was the lack of balance, with little time available for family, noted by both pediatric surgeons and their partners. CONCLUSION The issues of work-family balance and its impact on surgeon stress and burnout should be addressed in both pediatric surgery training and practice. The American Pediatric Surgical Association is positioned to play a leading role in this effort.

Inversion Herniotomy: A Laparoscopic Technique for Female Inguinal Hernia Repair

PURPOSE Laparoscopic inversion herniotomy (LIH) is a method of inguinal hernia repair for female patients. In this article, we report our technique and outcomes for this procedure. We propose that LIH is a valid alternative to conventional open herniorraphy. METHODS Following institutional review board approval, we reviewed the charts of patients who underwent LIH or open inguinal hernia repair (OIH) from 2004 to 2007. LIH was performed with three instrument sites and no groin incision. The diagnosis, operative time, and complications were reviewed. Follow-up ranged from 5 months to 4 years. RESULTS We performed 79 LIH repairs (15 bilateral, 23 left, and 26 right) in 63 female children. We also performed 76 contemporaneous OIH repairs in girls. The age range was 1 month to 18 years (median, 3.8 years). Operative times for both the unilateral and bilateral LIH repairs were significantly shorter than those for OIH (P = 0.001). There were 2 recurrences after laparoscopic inguinal hernia repair, both within 5 months following repair early in our experience, compared to no hernia recurrences with OIH. The first recurrence was repaired conventionally via an open technique. The second case was repaired laparoscopically. CONCLUSIONS LIH is an effective method for the repair of pediatric indirect inguinal hernia in female patients. The procedure allows the evaluation of the asymptomatic contralateral side without the necessity of a second long inguinal incision.

Treatment of Congenital Pulmonary Airway Malformation Induced Hydrops Fetalis via Percutaneous Sclerotherapy

Large type II and III congenital pulmonary airway malformations (CPAMs) can cause pulmonary hypoplasia, non-immune hydrops fetalis and fetal demise. Fetal intervention is indicated if hydrops fetalis develops. In this report, we describe three cases of type II and III CPAMs complicated by hydrops and treated with percutaneous sclerotherapy by ethanolamine injection into the tumor. All 3 cases demonstrated reduction in size of the CPAM and resolution of the hydrops with subsequent delivery at term. We believe that fetal percutaneous sclerotherapy can be used as a minimally invasive palliative strategy to treat CPAM-induced hydrops fetalis. Further studies are needed to delineate the risks of this novel technique.

Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients.

Background: Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting central control of breathing. Thus, patients require lifelong assisted ventilation. Diaphragm pacing (DP) may permit decannulation in those who are ventilator dependent only during sleep. Objective: The purpose of this study is to determine if patients with CCHS can be successfully ventilated by DP without tracheostomy. Methods: We reviewed the records of 18 CCHS patients (mean age 19.5 ± 10.1 years; 44% female) who were ventilated by DP only during sleep. Results: Prior to diaphragm pacer implantation surgery, 14 CCHS patients had been using home portable positive pressure ventilation (PPV) via tracheostomy, 1 had been on PPV via endotracheal tube, and 3 had been using noninvasive PPV (NPPV). Of the patients with tracheostomy prior to DP (n = 15), 11 (73%) were decannulated and ventilated successfully by DP without tracheostomy. Of all the patients reviewed (n = 18), 13 (72%) were successfully ventilated by DP without tracheostomy. Obesity prevented successful DP without tracheostomy in 1 patient, and upper airway obstruction prevented success in another patient. Snoring and/or obstructive apneas were present in some patients, but they were improved by diaphragm pacer changes, adenotonsillectomy, and/or use of nasal steroids. Conclusions: DP without tracheostomy can be successfully achieved in patients with CCHS. Snoring and obstructive apneas, when present, can be managed by diaphragm pacer changes and medical therapies. Obesity can pose a challenge to successful DP.

Thoracoscopic placement of phrenic nerve pacers for diaphragm pacing in congenital central hypoventilation syndrome

PURPOSE Congenital central hypoventilation syndrome (CCHS), or Ondines curse, is a rare disorder affecting central respiratory drive. Patients with this disorder fail to ventilate adequately and require lifelong ventilatory support. Diaphragm pacing is a form of ventilatory support which can improve mobility and/or remove the tracheostomy from CCHS patients. Little is known about complications and long-term outcomes of this procedure. METHODS A single-center retrospective review was performed of CCHS patients undergoing placement of phrenic nerve electrodes for diaphragm pacing between 2000 and 2012. Data abstracted from the medical record included operation duration, ventilation method, number of trocars required, and postoperative and pacing outcomes. RESULTS Charts of eighteen patients were reviewed. Mean surgical time was 3.3±0.7 hours. In all cases except one, three trocars were utilized for each hemithorax, with no conversions to open procedures. Five patients (27.8%) experienced postoperative complications. The mean ICU stay was 4.3±0.5 days, and the mean hospital stay is 5.7±0.3days. Eleven patients (61.1%) achieved their daily goal pacing times within the follow-up period. CONCLUSIONS Thoracoscopic placement of phrenic nerve electrodes for diaphragmatic pacing is a safe and effective treatment modality for CCHS. Observed complications were temporary, and the majority of patients were able to achieve pacing goals.

Pediatric Patients with Intravascular Devices: Polymicrobial Bloodstream Infections and Risk Factors

A retrospective study was conducted, including 61 patients with long-term intravascular devices (IVDs) admitted to the Childrens Hospital Los Angeles with diverse underlying diseases, different types of catheters, and culture-proven catheter-related bloodstream infections (BSIs). Within these patients, 125 catheter-related BSIs occurred, and the incidence of monomicrobial and polymicrobial BSIs was evaluated. Risk factors for polymicrobial BSIs were determined. Forty-two BSIs contained more than one pathogen. These polymicrobial BSIs were observed more often in younger patients (<4.1 years versus ≥4.1 years) and less in patients using venous implanted ports. No other associations were found between the occurrences of polymicrobial BSIs and underlying diseases, other types of catheters, host defense status, parenteral nutrition, recurrences, or catheter removal. Patients with long-term IVDs at a younger age have a higher risk of developing a polymicrobial BSI. Future prospective studies should address the issue of polymicrobial infection in IVDs in more detail.