R. Cartland Burns

The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival

Objective:The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. Methods:Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). Results:KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. Conclusion:Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

Embryonic mouse blood flow and oxygen correlate with early pancreatic differentiation

The mammalian embryo represents a fundamental paradox in biology. Its location within the uterus, especially early during development when embryonic cardiovascular development and placental blood flow are not well-established, leads to an obligate hypoxic environment. Despite this hypoxia, the embryonic cells are able to undergo remarkable growth, morphogenesis, and differentiation. Recent evidence suggests that embryonic organ differentiation, including pancreatic β-cells, is tightly regulated by oxygen levels. Since a major determinant of oxygen tension in mammalian embryos after implantation is embryonic blood flow, here we used a novel survivable in utero intracardiac injection technique to deliver a vascular tracer to living mouse embryos. Once injected, the embryonic heart could be visualized to continue contracting normally, thereby distributing the tracer specifically only to those regions where embryonic blood was flowing. We found that the embryonic pancreas early in development shows a remarkable paucity of blood flow and that the presence of blood flow correlates with the differentiation state of the developing pancreatic epithelial cells in the region of the blood flow.

Treatment of infantile choriocarcinoma of the liver.

Primary choriocarcinoma of the liver is an extremely rare childhood malignancy frequently associated with clinical instability at initial presentation. It often mimics other benign and malignant childhood liver tumors. Prompt diagnosis and initiation of treatment are necessary to attain a successful outcome. We describe a critically ill infant with metastatic choriocarcinoma whose diagnosis was based on radiographic and tumor marker findings, without an initial biopsy, and her successful management with neo‐adjuvant chemotherapy and delayed surgery. She is currently in continuous remission 24 months from diagnosis. Pediatr Blood Cancer 2007;49:99–102.

Induction of fibroblast growth factor 10 (FGF10) in the ileal crypt epithelium after massive small bowel resection suggests a role for FGF10 in gut adaptation.

We have previously reported that fibroblast growth factor 10 (FGF10) is crucial for the survival and proliferation of progenitor cells during embryonic gastrointestinal development. We sought to characterize the potential role of FGF10 signaling in the adaptive response following small bowel resection. Adult wild‐type and Fgf10LacZ mice underwent 50% small bowel resection (SBR) or sham operation. Tissues were harvested 24 or 48 hr after surgery for histology, immunohistochemistry, and in situ hybridization. After SBR, Fgf10 expression was demonstrated in the epithelium at the base of the crypts. Moreover, there was a statistically significant increase in proliferating cells and goblet cells after SBR. In vitro studies using rat intestinal epithelial crypt (IEC‐6) cells exposed to medium with or without recombinant FGF10 showed increased proliferation and phosphorylation of Raf and AKT with the addition of FGF10. Our results suggest that FGF10 may play a therapeutic role in diseases involving intestinal failure. Developmental Dynamics 238:294–301, 2009.

Laparoscopic Versus Open Distal Pancreatectomy in the Management of Traumatic Pancreatic Disruption

PURPOSE Traumatic pancreatic transection is uncommon. The role of laparoscopy in the setting of this injury has not been well described. PATIENTS AND METHODS Six large-volume pediatric trauma centers contributed patients <18 years of age who underwent a distal pancreatectomy for traumatic pancreatic transection from 2000 to 2010. RESULTS Twenty-one patients without another indication for emergency laparotomy underwent a distal pancreatectomy for Grade III pancreatic injuries, of which 7 underwent laparoscopic distal pancreatectomy. Mean (±SD) age was 8.6±4.7 years, and 67% were male. There was no difference in the presence of other injuries between the two groups (43% in each group). Computed tomography revealed a transected pancreas in 85% of the laparoscopic patients and 75% of the open group (P=1.0). Mean operative time was 218±101 minutes with laparoscopy compared with 195±111 minutes with the open procedure (P=.7). Median duration of hospitalization was 6 days (range, 6-18 days) in the laparoscopic group compared with 11 days (range, 5-26 days) in the open group (P=0.3). Postoperative morbidity was not different between the two groups (57% versus 21% for laparoscopic versus open, P=.2). CONCLUSIONS Laparoscopy is equivalent to open distal pancreatectomy in children with select traumatic pancreatic injuries.

Thoracoscopy in the Management of Chylothorax

The management of chylothorax has undergone a series of changes in the past decade. Current practice includes nonoperative treatment with low-fat dietary manipulation, pleuroperitoneal shunting procedures, and the direct operative approach to the thoracic duct. Medical management should be used initially but should not be continued for extended periods of time. We have felt that after 1 week of medical therapy, the risks of sepsis and malnutrition outweigh the risk of intervention. The shunting procedures have been successful in managing conditions that are associated with widespread lymphatic leak such as postoperative chylothorax with wide mediastinal dissection and lymphangiomatosis. Thoracoscopic procedures are most successful in the management of the discrete thoracic duct injury, including posttraumatic injuries and congenital chylothorax. The thoracoscopic approach to chylothorax appears to be as effective as thoracotomy, while offering the benefits of minimally invasive procedures.

Hemoperitoneum versus bowel perforation in the extremely low birth weight infant

As medical technology continues to advance survival of the extremely low birth weight (ELBW) infant, new diagnostic dilemmas arise for neonatal caregivers. Abdominal discoloration with distention in the extremely premature infant poses one such diagnostic problem. Focal small bowel perforation is the most common cause for abdominal wall discoloration in ELBW infants during the first week of life. 1