Richard Vaughan

A Proposed Classification for the Spectrum of Vanishing Gastroschisis

Infants born with gastroschisis in association with intestinal atresia are well described. We are the proposing the classification of vanishing gastroschisis. In this series of six cases, at one end of the spectrum is an infant having gastroschisis with a much narrower defect on the right side of umbilicus. The ischemic bowel loops were connected to bowel inside the abdomen by a fibrous band compressing the exposed bowel mesentery. On the other end of spectrum, an infant having extensive bowel atresia and complete closure of abdominal wall defect (gastroschisis) detected on antenatal ultrasound. These cases should raise awareness of this devastating complication in prenatal management of gastroschisis.

Hepatic artery pseudoaneurysm: Delayed presentation after bicycle accident

A13-year-old boy presented to the emergency department with hematemesis. Nine days earlier, the patient was involved in a bicycle accident during which the handlebars struck against his right lower rib cage. He was seen at an outside facility complaining only of rib pain. Plain films were negative for traumatic abnormality. He was discharged home from the emergency room on that day. On posttrauma day 6, he returned to the outside facility complaining of hematemesis. An nasogastric tube returned heme-positive fluid, but no gross blood. Abdominal computed tomography (CT) showed hepatic laceration and a hematoma. He was sent home with instructions to rest. On posttrauma day 9, he experienced another episode of hematemesis, now with visible clots. He was referred to our facility for evaluation. He denied other complaints including melena. Abdomen was soft and mildly tender in the right upper quadrant. Rectal examination had no gross blood but hemepositive stool. He was not jaundiced. Medical history was significant for ADHD and OCD. He was taking Paxil-CR, Trazodone, and Focalin. There was no significant family or social history. CBC demonstrated anemia with Hgb/Hct of 10/28, total and conjugated bilirubin of 6 and 3.6, respectively, AST 252, and ALT 316. A CT of the abdomen was done (Fig. 1). CT revealed a grade III liver laceration and blood clots within the gallbladder lumen and mild intrahepatic biliary duct dilatation. On the basis of these findings, the working diagnosis was hemobilia secondary to trauma. He was admitted to the pediatric trauma service. He was made nothing by mouth and placed on maintenance fluids. The next day he was taken to interventional radiology for evaluation of hepatic blood supply. Hepatic arteriogram revealed a pseudoaneurysm of the superior branch of the right hepatic artery and no active extravasation (Fig. 2). Two coils were placed in the pseudoaneurysm. He was taken to the PICU for monitoring. Daily laboratory values showed gradual improvement of his liver function tests. On posttrauma day 13, he was discharged. Follow-up CT demonstrated unchanged embolization coils and a resolving hematoma. Hepatic artery pseudoaneurysm is a rare but serious condition. It can be seen after acute surgical injury, chronic damage, or most commonly after trauma. The presentation of symptoms may vary. They can present clinically silent or as massive hemorrhage. As in this case, signs of upper or lower gastrointestinal bleeding can be present. The majority of pseudoaneurysms are extrahepatic and occur in the right hepatic artery. The diagnosis is most commonly made with contrast-enhanced CT; however, this was not the case with our patient.1 Maintaining a high index of suspicion is important if a focally enhancing region of high attenuation is noted within a vascular structure.2 The sensitivity for CT has been found to be only 67%. Selective angiography is 100% sensitive.3 It has been suggested that if the initial CT suggests a hepatic pseudoaneurysm, then the patient should be taken for angiography.1 This allows for confirmation of the injury, and intervention can begin immediately. As a result, most of these cases are treated nonoperatively. If not treated, there is potential for delayed rupture and subsequent hemorrhage. Other possible complications include enteric fistula formation and infection or abscess formation.2

Idiopathic non-hypertrophic pyloric stenosis in an infant successfully treated via endoscopic approach

Non-peptic, non-hypertrophic pyloric stenosis has rarely been reported in pediatric literature. Endoscopic pyloric balloon dilation has been shown to be a safe procedure in treating gastric outlet obstruction in older children and adults. Partial gastric outlet obstruction (GOO) was diagnosed in an infant by history and confirmed by an upper gastrointestinal series (UGI). Abdominal ultrasonography and computed tomography scan excluded idiopathic hypertrophic pyloric stenosis, abdominal tumors, gastrointestinal and hepato-biliary-pancreatic anomalies. Endoscopic findings showed a pinhole-sized pylorus and did not indicate peptic ulcer disease, Helicobacter pylori infection, antral web, or evidence of allergic and inflammatory bowel diseases. Three sessions of a step-wise endoscopic pyloric balloon dilation were conducted under general anesthesia and a fluoroscopy at two week intervals using catheter balloons (Boston Scientific Microvasive(®), MA, USA) of increasing diameters. Repeat UGI after the first session revealed normal gastrointestinal transit and no intestinal obstruction. The patient tolerated solid food without any gastrointestinal symptoms since the first session. The endoscope was able to be passed through the pylorus after the last session. Although the etiology of GOO in this infant is unclear (proposed mechanisms are herein discussed), endoscopic pyloric balloon dilation was a safe procedure for treating this young infant with non-peptic, non-hypertrophic pyloric stenosis and should be considered as an initial approach before pyloroplasty in such presentations.

Posterior Flank Approach to the Peritoneum for Ventriculoperitoneal Shunting

We report a technique for insertion of a distal shunt catheter into the peritoneal cavity through a posterior incision. This has been helpful in patients with impaired access anteriorly. It may also be useful in elective situations. We have performed this procedure on 3 infants where the traditional anterior approaches would have been difficult due to subcutaneous scar tissue, ostomy sites and anterior intra-abdominal adhesions. This technique was safe and provided easy access to the peritoneal cavity. Complications would be similar to those for the standard ventriculoperitoneal shunts including bowel perforation, damage to the kidney or liver, infection and occasionally posterior cervical webbing that may cause pain locally or pull the head into an extended position.

Feasibility of Cryopreserved Conduits for Complex Vascular Reconstruction in the Pediatric Population: The Case of a 3-Year-Old With Femoral Vessels Transections:

This report presents an unusual case of traumatic iliofemoral vessel transection in a 3-year-old patient successfully reconstructed using a cryopreserved greater saphenous conduit. Five years after injury, the patient continues to do well with normal ambulation. An arterial duplex demonstrated graft patency free of aneurysmal dilatation. These encouraging results suggest that the natural history of cryopreserved conduits may differ in the pediatric population and cryopreserved conduits could be used for complex vascular reconstructions.