Hypoplasia of Extrahepatic Biliary Tree and Intrahepatic Cholangiolopathy in Cystic Fibrosis Imperfectly Mimic Biliary Atresia in 4 Infants With Cystic Fibrosis and Kasai Portoenterostomy.

Four male infants with cystic fibrosis and prolonged neonatal jaundice underwent Kasai procedure to relieve biliary obstruction due to apparent biliary atresia. The excised remnants had viscid mucus accumulation in hypoplastic gallbladders and distended peribiliary glands. Main hepatic ducts were narrow and/or malformed. Microscopic differences between the gallbladder and extrahepatic bile ducts in cystic fibrosis and sporadic biliary atresia were unequivocal, despite some histologic overlap; no erosive or fibro-obliterative lesions typical of biliary atresia were seen. Common in liver, biopsies were small duct cholangiopathy with intense focal cholangiolitis and massive accumulation of ceroid pigment within damaged cholangiocytes, and in portal macrophages, portal fibrosis, and unequivocal features of large duct obstruction were inconspicuous compared with biliary atresia. Plugs of bile in small ducts tended to be pale and strongly periodic acid-Schiff-reactive in cystic fibrosis. Distinguishing the liver lesion from that of biliary atresia is challenging but possible. Liver biopsies from 2 additional infants with cystic fibrosis and prolonged jaundice that spontaneously resolved showed a similar small duct cholangiopathy. Small gallbladders and extrahepatic ducts challenge surgical judgment as findings in liver biopsies challenge the pathologist. The decision to perform a Kasai procedure is reasonable when mimicry of biliary atresia is grossly complete. We hypothesize that a disorder of bile volume/flow during development and/or early infancy linked to the CFTR mutation alone or in combination with the stresses of neonatal intensive care causes destructive cholangiolitis and intrahepatic reduction of bile flow with secondary hypoplasia of extrahepatic biliary structures.

Spontaneous onset of labor, not route of delivery, is associated with prolonged length of stay in babies with gastroschisis.

BACKGROUND/PURPOSE: We studied obstetric delivery practices for fetal gastroschisis and correlated this with neonatal outcomes. Our objectives were to identify changes in delivery practices over time and to determine if these changes resulted in improved neonatal outcomes. METHODS: After IRB approval, maternal and neonatal records from 219 gastroschisis births between 1990 and 2008 were reviewed. Obstetrical data and neonatal data were collected. Univariate comparisons were made between maternal delivery variables and neonatal outcomes. Significant and clinically relevant obstetrical variables were combined for multivariate linear regression modeling. RESULTS: The practice of elective cesarean delivery (ELCS) shifted to spontaneous vaginal delivery (sVD) over time (p <0.001). Babies born by sVD had longer hospitalization than those born by ELCS (median 36.0 vs 21.6days, p <0.05). Gestational age (GA) and birth weight were similar between groups. Babies born by induced VD (iVD) had short hospitalization (median 22.5days). A linear regression model demonstrated that spontaneous onset of labor (SOL) and GA were independently related to LOS. CONCLUSIONS: Over nearly two decades, delivery of gastroschisis babies shifted from ELCS to sVD, a practice associated with a significantly longer LOS. Regression models suggest that shorter LOS could be achieved if elective delivery modes are utilized prior to SOL.

Association of high ventilator pressures with the development of chronic pulmonary hypertension in congenital diaphragmatic hernia patients requiring ECMO.

PURPOSE: Congenital diaphragmatic hernia (CDH) patients requiring extracorporeal membrane oxygenation (ECMO) were examined to determine, if aspects of their complex ventilatory management were associated with the development of chronic pulmonary hypertension (cPH). METHODS: CDH patients requiring ECMO from 1992 to 2007 were retrospectively reviewed. cPH was defined as pulmonary hypertension at 3 months of age. Demographic and clinical variables including peak ventilatory pressures (PVP) and mean airway pressures (MAP) were tabulated. RESULTS: 10/31 (32 %) patients developed cPH. Gestational age, birth weight, inborn status, CDH side and liver position were not different between cPH and non-cPH patients. Pre-ECMO, both groups required statistically similar ventilatory support, though there was a trend toward higher oxygenation index and higher PVP for cPH patients. While ECMO duration was similar between groups, cPH patients required significantly higher PVP (30.0 vs. 25.0 cmH(2)O, p = 0.01) and MAP (11.5 vs. 9.0 cmH(2)O, p = 0.02) for ECMO decannulation. Post-ECMO, maximum PVP (50.0 vs. 26.0 cmH(2)O, p < 0.001), MAP (18.1 vs. 12.0, p = 0.001), HFV requirement (90 vs. 10 %, p < 0.001), and ventilator time (35.7 vs. 20 days, p < 0.001) increased significantly for cPH patients. CONCLUSION: Not until after ECMO decannulation do we see clinical differences separating patients who ultimately develop cPH. Although the degree of pulmonary hypoplasia may ultimately dictate ECMO decannulation criteria, perhaps greater physiologic optimization before decannulation could decrease the incidence of cPH.

Method to our madness: an 18-year retrospective analysis on gastroschisis closure.

BACKGROUND: The advent of preformed silos has facilitated routine bedside placement often without any attempt of intestinal reduction. It is unclear whether a strategy of routine silo (RS) placement with delayed fascial repair is beneficial over attempted primary repair (aPR) and silo placement only for those patients who cannot be reduced. We retrospectively compared clinical outcomes of neonates having aPR to those having RS placement to determine the impact of routine silo use and silo duration on gastroschisis care. METHODS: Neonatal records from patients with gastroschisis at a single children's hospital between 1990 and 2008 were reviewed. Demographic and outcome data were recorded and subjected to statistical analyses. Documentation of attempted intestinal reduction was used as a surrogate marker for aPR. The remaining patients were placed in the RS group. RESULTS: Two hundred forty-eight neonates with gastroschisis were identified. Thirteen were excluded for congenital or clinical issues which precluded aPR. Of the remaining 235 patients, neonates with RS had significantly more ventilator days (6.2 vs 4.4; P = .0011), more time of total parenteral nutrition (36.5 vs. 28.5; P = .0018), longer length of stay (LOS, 46.5 vs. 40.5; P = .0011), and greater hospital charges ($216,000 vs $172,000; P < .0001) than patients who had aPR. There was no significant difference observed in complications or survival. Linear regression modeling demonstrated that time to closure was significantly related to LOS as an independent variable. Each day to closure was associated with 2.2 extra days of hospitalization and approximately $9557 in hospital charges. CONCLUSION: Although limited by retrospective biases, this study demonstrates that time to closure is the most significant variable related to LOS in gastroschisis. This relationship is intuitive since longer time to closure is probably determined by the severity of gastroschisis. The method of closure, by primary repair or silo, is of secondary importance. Conversely, unnecessarily increasing the time to closure may increase the LOS. The speed of reduction, whether through primary repair or by silo, should be guided by physiologic principles.

Laparoscopic distal pancreatectomy in children: four cases and review of the literature.

INTRODUCTION: Laparoscopy has been utilized for children with pancreatic masses and blunt transection. In this article, we report our technique and experience. OPERATIVE TECHNIQUE: With supine positioning, an umbilical trocar and three 5-mm trocars are positioned across the epigastrium. The gastrocolic ligament is opened completely and the stomach is retracted superiorly. A vessel-sealing electrosurgical device is used to dissect along the inferior margin of the pancreas. Dissection proceeds proximally or distally, depending on the location of the pathology. The proximal pancreatic duct is oversewn or stapled closed. The distal pancreas is mobilized from the splenic vessels. If the vessels cannot be mobilized from the pancreas, they are divided and a laparoscopic splenectomy is performed. The specimen is removed through the umbilical trocar by using a retrieval bag. Drains are placed prior to closure. CASE EXPERIENCE: We report 4 cases: 2 with pseudopapillary tumors and 2 with traumatic injuries. One patient was male; the mean age was 13.0 +/- 1.4 years. Two spleens were removed due to pathology. Mean operative time was 256 +/- 46.6 minutes, with no open conversion or mortality. Patients initiated oral intake 2.0 +/- 1.4 days postoperatively. Hospital stay was 6.2 +/- 3.9 days. One patient required 15 days of total parenteral nutrition to resolve a pancreatic fistula. CONCLUSIONS: Laparoscopic pancreatic resection in children is feasible, safe, and leads to rapid recovery without significant morbidity. The spleen can often be spared, minimizing the risk of overwhelming postsplenectomy sepsis. This initial experience should encourage wider use of laparoscopy for pancreatic resection in children.

Blunt posterior tracheal laceration and esophageal injury in a child.

Blunt force trauma to the neck can result in the unusual injury pattern of laceration of the posterior tracheal wall in combination with esophageal injury. We present the report of a 10-year-old child who had blunt cervical trauma because of a bicycle accident and subsequently presented with profound subcutaneous emphysema. This case was addressed with operative management with a good result. The essential management principles for this rare constellation of injuries include a high index of suspicion, early control of the airway, endoscopic and radiographic diagnosis, and use of a buttressing strap muscle flap in the event of operative management to prevent delayed complications, including leak and tracheoesophageal fistula.

Severe sepsis attributable to community-associated methicillin-resistant Staphylococcus aureus: an emerging fatal problem.

We observed a number of cases of sepsis from bacteremia in children from community-associated methicillin-resistant Staphylococcus aureus (MRSA), which led us to study its patterns of infection and outcome. A retrospective review identifying children admitted to our institution with blood culture-proven community-associated MRSA sepsis over a 2-year period was performed. The inclusion criteria were younger than 19 years old, two or more blood cultures for MRSA within 48 hours of admission, evidence of systemic inflammatory response syndrome, and no prior hospital admissions within 6 months. Eight patients were included; seven required mechanical ventilation. Vasopressors were required in seven patients. Four patients required extracorporeal membrane oxygenation. Four patients had culture-proven septic arthritis or thrombophlebitis and three of these patients developed bilateral necrotizing pneumonia. Bilateral necrotizing pneumonia was identified in the other four patients, but the primary source of infection was never identified. The overall intact neurologic survival was 50 per cent. Children with severe community-associated MRSA sepsis can rapidly progress to cardiorespiratory failure. Mortality appears to be high, and children may benefit from a search of their soft tissues and joints to identify the source of infection to prevent embolic dissemination.

Increasing use of extracorporeal life support in methicillin-resistant Staphylococcus aureus sepsis in children.

BACKGROUND: Pediatric cases of fulminant community-associated methicillin-resistant Staphylococcus aureus (MRSA) infections requiring extracorporeal life support (ECLS) have been reported, but the frequency of ECLS use for severe presentations of staphylococcal disease is unknown. OBJECTIVE: To describe the frequency and characteristics of children with MRSA infections requiring ECLS using local and international databases. METHODS: The reasons for use of ECLS in children 0-18 yrs of age were determined in both the Vanderbilt Children's Hospital medical record system and the Extracorporeal Life Support Organization database during the years 1994-2005. Demographic characteristics, ventilatory management, and measurements of cardiopulmonary status in subjects undergoing ECLS with a pre-ECLS diagnosis of infection with Staphylococcus aureus and MRSA were included. RESULTS: Three subjects with MRSA sepsis requiring ECLS were identified at Vanderbilt since 2000. Before that time, no cases due to MRSA were reported. The three subjects were previously healthy adolescents with severe necrotizing pneumonia associated with skin/soft-tissue infection and two died. A total of 45 patients requiring ECLS for MRSA infection were identified in the International Extracorporeal Life Support Organization database, with nearly half reported in the past 2 yrs (20 of 45 patients). The median age was 2.4 yrs (interquartile range, 0.36-14 yrs), with peaks noted in infancy and adolescence. In Extracorporeal Life Support Organization subjects with MRSA, survival to discharge was highest in infants and young children aged 1-4 yrs (65% and 71%, respectively) and lowest in the age ranges of 5-9 yrs and 13-18 yrs (0% and 31%, respectively). There were no statistically significant differences in pre-ECLS ventilatory settings, cardiopulmonary status, or frequency of complications between survivors and nonsurvivors. CONCLUSIONS: The use of ECLS for MRSA infection seems to be increasing both locally and internationally. High mortality rates, particularly in older patients, are concerning and highlight the increasing problem with this pathogen.

Hydrostatic stretch-induced growth facilitating primary anastomosis in long-gap esophageal atresia.

We present a novel simple technique for stimulating distal esophageal pouch growth in a patient with isolated, long-gap esophageal atresia. In this case report, distal esophageal growth was achieved through daily intermittent pressurization via a surgically placed indwelling balloon catheter. The patient ultimately underwent successful, uncomplicated, tension-free, primary repair and remains asymptomatic.

Neonatal thoracoscopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome.

BACKGROUND/PURPOSE: Complications of open conversion, hypercarbia, and intestinal injury have plagued minimally invasive approaches to congenital diaphragmatic hernia (CDH) repair in neonates. To safely begin using minimally invasive techniques for neonatal CDH repair, we formulated preoperative selection criteria and operative techniques that would enhance chances for successful thoracoscopic primary diaphragm repair and uncomplicated outcome. METHODS: During the period from January 2003 to October 2004, neonates were selected for thoracoscopic CDH repair using anatomic and physiologic criteria. Anatomically, all patients were required to have stomach in the abdomen by radiography. Physiologically, all patients were required to be on minimal ventilator support with preoperative ventilator peak inspiratory pressures in the low 20s mm Hg. No patient could have clinical evidence of pulmonary hypertension at the time of surgery. Thoracoscopic CDH repair was performed using 3 trocars (3 and 5 mm). The hernia contents were reduced into the abdomen using 5-mm Hg insufflation, and the diaphragms were repaired primarily using interrupted 3-0 Ethibond simple sutures (Ethicon, Inc, Piscataway, NJ). Posterolateral diaphragm stitches were passed around the posterolateral ribs and tied extracorporeally. RESULTS: Thirty neonates with CDH were admitted to Children's Hospital Boston and Vanderbilt Children's Hospital during the study period. Eight patients (27%) met selection criteria and 7 underwent thoracoscopic CDH repair. Primary diaphragmatic repair was successfully accomplished thoracoscopically in all neonates without perioperative complication. Preoperative anatomic criteria correlated accurately with intact esophageal hiatus and primary diaphragm repair. Physiologically, each patient tolerated intrathoracic insufflation and CDH repair without clinical pulmonary hypertension or blood pressure lability. Three patients had intraoperative respiratory acidosis that was reversed with ventilator changes. Operative times averaged 152 minutes and ranged from 212 to 106 minutes. Postoperative mechanical ventilation ranged from 0 to 7 days, and the length of hospitalization ranged from 5 to 32 days. Longest follow-up has been 17 months. One patient required reoperation for recurrent CDH at 10 months after repair, but there have been no other long-term complications. CONCLUSIONS: Neonatal thoracoscopic CDH repair is safe in selected patients who have good preoperative pulmonary function and anatomy amenable to primary diaphragmatic repair. A wider range of neonates may be acceptable for thoracoscopic CDH repair with increasing surgical experience.