Pramod S. Puligandla

Effect of hospital case volume on outcome in congenital diaphragmatic hernia: the experience of the Canadian Pediatric Surgery Network

PURPOSE Despite advances in neonatal care of congenital diaphragmatic hernia (CDH), a significant variation exists in the mortality rates reported by individual centers. Center experience (reflected by case volume) may contribute to this variation in outcome. The aim of the study was to determine whether CDH mortality is affected by hospital case volume. METHODS The CDH cases were abstracted from a disease-specific, 16-hospital, national network. Thirteen hospitals participated in this study. Anonymized hospitals were categorized as either high (>6 cases) or low-volume (<or=6 cases) centers (HVC, n = 6; LVC, n = 7) according to the median case number per center. Risk-adjusted (Score for Neonatal Acute Physiology, version II [SNAP-II] score) mortality rates were compared between HVC and LVC. RESULTS One hundred twenty-one CDH cases were identified. Overall in-hospital survival was 81%. No significant difference in SNAP-II score was observed between HVC and LVC. Of 97 (15%) infants treated in 6 HVC, 15 (15%) died compared to 8 (33%) of 24 in 7 LVC (P < .05). CONCLUSION Hospital case volume may be partially responsible for mortality rate variation in CDH. This result requires careful analysis, as case volume may merely be a surrogate for other predictive variables.

Use of continuous positive airway pressure (CPAP) in acute viral bronchiolitis: A systematic review

Continuous positive airway pressure (CPAP), used either alone or associated with heliox (CPAP‐He), has become a popular therapeutic option for bronchiolitis. This systematic review assesses the impact of CPAP on endotracheal intubation, carbon dioxide pressure (PCO2) and respiratory distress in patients with bronchiolitis.

Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network

BACKGROUND Perinatal management of congenital diaphragmatic hernia (CDH) and gastroschisis (GS) remains nonstandardized and institution specific. This analysis describes practice and outcome variation across a national network. METHODS A national, prospective, disease-specific database for CDH and GS was evaluated over 4 years. Centers were evaluated individually and defined as low (low-volume center [LVC]) or high (high-volume center [HVC]) volume based on case mean. RESULTS Congenital diaphragmatic hernia. Two hundred fifteen liveborn cases were studied (mean, 14.3 cases/center) across 15 centers (8 LVCs and 7 HVCs). Significant interinstitutional practice variation was noted in rates of termination (0%-40%) and cesarean delivery (0%-61%). Centers demonstrated marked variation in ventilation strategies, vasodilator and paralytic use, timing of surgery, and rates of primary closure. Overall survival was 81.4% (LVC, 76.9%; HVC, 82.4%; P = .43). Gastroschisis. Four hundred sixteen cases were investigated (mean, 26 cases/center; range, 6-72) across 16 centers (10 LVCs and 6 HVCs). Cesarean delivery rates varied widely between centers (0%-86%) as did timing of closure (early vs delayed, 1%-100%). There was no difference in length of stay, days on total parenteral nutrition, and overall survival (94.3% vs 97.2%; P = .17) between LVCs and HVCs. CONCLUSIONS The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis.

Outcome of laparoscopic appendectomy for perforated appendicitis in children.

BACKGROUND The aim of this study was to evaluate the outcome of laparoscopic (LA) vs open appendectomy (OA) in patients with perforated appendicitis in our center. METHODS Retrospective review from July 2002 to April 2007 (institutional review board-approved), evaluating 281 patients with perforated appendicitis based on surgical approach. We compared demographics, mean operative time, length of stay (LOS), infectious complications, and follow-up in patients with OA (n = 213) and LA (n = 68). RESULTS Laparoscopic appendectomy patients were significantly older (12 vs 9.4 years), heavier (51.8 vs 36.6 kg) and more frequently girls (47.8% vs 34.3%). Mean operative time was longer in LA (72.6 vs 50.2 minutes). Median LOS was 5 days in LA and 6 days in OA. Few patients in each group required a drainage procedure for a persistent abscess (LA 4.4%, OA 4.7%; P = 1.000). Laparoscopic appendectomy patients had fewer wound infections (1.5% vs 9.5%; P = .034), and less follow-up visits were needed (>2 clinic visits 4.5% vs 16.4%; P = .013). CONCLUSION Laparoscopic appendectomy has a shorter median LOS, a trend toward less postoperative infectious complications, and fewer clinic visits than OA, which makes it a safe and effective procedure for patients with perforated appendicitis.

Congenital lung malformations: informing best practice.

The management of congenital lung malformations is controversial both in the prenatal and postnatal periods. This article attempts to inform best practice by reviewing the level of evidence with regard to prenatal diagnosis, prognosis, and management and postnatal management, including imaging, surgical indication, surgical approach, and risk of malignancy. We present a series of clinically relevant statements along those topics and analyze the evidence for each. In the end, we make a plea for an adequate description of the lesions, both before and after birth, which will allow future comparisons between management options and the initiation of prospective registries.

A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection

BACKGROUND/PURPOSE The timing and need of resection of asymptomatic congenital lung lesions are controversial. The morbidity of such surgery needs to be considered in the decision analysis. We analyzed the contemporary outcome of infants and neonates undergoing lung resection. METHODS With institutional review board approval, all patients 12 months or younger undergoing lung resection between 1995 and 2004 in 2 hospitals were reviewed. Demographic data, indications for surgery, operative procedure, complications, use of regional anesthesia, length of stay (LOS), and follow-up were assessed. RESULTS Forty-five patients (28 male, 17 female) with a median age of 4 months (2 days-12 months) were evaluated. Congenital lesions (42) were the most frequent indication for surgery. Twenty-two (48.9%) patients had cardiorespiratory symptoms or infection preoperatively. Lobectomy was the most common operation (40/45). Three patients had intraoperative difficulty (bleeding, hypotension, desaturation). Significant postoperative complications occurred in 7 patients: prolonged air leak or chest tube drainage (4), anemia or bleeding (2), respiratory distress requiring reintubation (1). Fewer complications occurred in asymptomatic vs symptomatic patients (1/23 vs 6/22). Of 12 patients, 7 (58%) requiring 24 hours of ventilation or longer were 3 months or younger. Increasing age did significantly influence the chance of successful extubation (P = .01; odds ratio, 1.5; 95% confidence interval, 1.0-2.0), as did the use of epidural anesthesia (P < .001). Median LOS was 6 days (2-89 days). Asymptomatic patients had shorter LOS (median, 4 days; range, 2-20 days; P = .024) vs symptomatic patients (median, 8 days; range, 4-89 days). The only death occurred from underlying heart disease. Mean follow-up at 35 months (12-132 months) revealed no subjective reduction in cardiopulmonary function. CONCLUSIONS Lung resection is safe and well tolerated in infancy. Surgery should be scheduled before the development of symptoms but likely after 3 months of age to improve the chances of postoperative extubation. The use of regional anesthesia may facilitate this.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

OBJECTIVE Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Congenital lung lesions.

Confusion, controversy, and uncertainty are all terms applicable to the diagnosis and management of congenital lung lesions both prenatally and postnatally. This review examines the current status of fetal diagnosis and treatment of these lesions; reviews the various classifications, including congenital cystic adenomatoid malformation/congenital pulmonary airway malformation, sequestrations, variants and hybrid lesions; discusses the risk of malignant transformation or misdiagnosis with pleuropulmonary blastoma; presents the arguments in favor and against resection of asymptomatic lesions, the timing of such resection, and the long-term pulmonary function after resection; and reviews the experience with thoracoscopic resection of congenital lung lesions.

A contemporary evaluation of pulmonary function in children undergoing lung resection in infancy

BACKGROUND/PURPOSE The management of asymptomatic congenital lung lesions is controversial. Some centers recommend resection in infancy, and others prefer observation. Our objective was to evaluate the pulmonary function of children who underwent lung resection at 12 months or younger. We hypothesized that these children would not have a significant reduction in pulmonary function when compared with norms for age. METHODS All patients at 2 tertiary-care childrens hospitals who underwent lung resection at 12 months or younger and are currently older than 5 years were identified and prospectively recruited. Pulmonary function testing was standardized in all patients. RESULTS Fourteen children were tested prospectively, whereas results were available for another 5 children. Four children were excluded for inability to perform pulmonary function testing (n = 2) or for preexisting pulmonary hypoplasia/syndrome (n = 2). Pulmonary function testing values were considered normal if they were more than 80% of predicted. Forced vital capacity was normal in 14 (93%) of 15 children, and forced expiratory volume in 1 second was normal in 13 (86%) of 15 children. Diffusion capacity and respiratory muscle strength were normal in all children tested. CONCLUSIONS Most children undergoing lung resection in infancy will have normal pulmonary function tests, supporting our philosophy of early, elective resection of congenital lung lesions.

The pitfalls of endotracheal intubation beyond the fistula in babies with type C esophageal atresia

The intraoperative management of a neonate with esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) is a true anesthetic challenge. Pediatric anesthesia textbooks recommend a distal tracheal intubation beyond the fistula and spontaneous ventilation, if possible, until surgical control of the fistula is achieved to minimize gastric distention. A full-term neonate with Trisomy 21 presented with an EATEF and was transferred to the operating theater for repair after appropriate evaluation. After induction of anesthesia, a size 3.0 endotracheal tube was inserted orally with confirmation of its position by good air entry and chest movement bilaterally. After positioning for thoracotomy, the patient desaturated and became bradycardic with abdominal distention. Despite reintubation, gastric needle decompression, and bilateral pleural aspiration to exclude pneumothorax, cardiopulmonary resuscitation was unsuccessful and the child died. Autopsy revealed the endotracheal tube in the trachea with its distal end passing through a large distal TEF. Preoperative bronchoscopy may help the team to assess the size and location of the distal TEF and plan for the best anesthetic strategy. It may also be useful to confirm tube location after endotracheal intubation and intraoperatively in the event of cardiorespiratory instability.