Evaluation of a three-session biliary dilation protocol following transplant-related biliary stricture in pediatric patients.

To evaluate whether a serial biliary dilation protocol improves outcomes and decreases total biliary drainage time for biliary strictures following pediatric liver transplantation. From 2006 to 2016, 213 orthotopic deceased and living related liver transplants were performed in 199 patients with a median patient age of 3.1 years at a single pediatric hospital. Patients with biliary strictures were managed by IR or surgically by the transplant team. Patients managed by IR were divided into two groups. The first group was managed with a standardized three-session protocol consisting of dilation every two weeks for three dilations. The second group was managed clinically with varying number and interval of dilations as determined by a multidisciplinary team. The location of biliary stricture, duration of drainage, number of balloon dilations, balloon diameter, time interval between dilations, and success of percutaneous treatment were recorded. Thirty-four patients developed biliary strictures. Thirty-one patients were managed with percutaneous intervention. Three strictures could not be crossed and were converted to operative management. Ten patients were managed in the three-session protocol, and 18 patients were managed in the clinically treated group. There was no significant difference in clinical success rates between groups, 80% and 61%, respectively. The three-session protocol group trended toward a lower total biliary drain indwell time (median 49 days) compared with the clinically treated group (median 89 days), P = .089. Our study suggests that a three-session dilation protocol following transplant-related biliary stricture may decrease total biliary drainage time for some patients.

Partial Splenic Embolization Is a Safe and Effective Alternative in the Management of Portal Hypertension in Children.

OBJECTIVE: There are multiple approaches to manage the clinical complications of portal hypertension (PHTN) to treat/prevent spontaneous hemorrhage by mitigating thrombocytopenia. No single approach is ideal for all patients given the heterogeneity of this population. Our goal was to determine whether partial splenic embolization (PSE) was safe and effective in the pediatric population. METHODS: This is a retrospective review of our single-center experience for all patients ages 0 to 21 who underwent PSE between January 2010 and August 2017. The embolized splenic volume targeted was 60% to 70%. RESULTS: Twenty-six patients underwent PSE due to thrombocytopenia and/or recurrent variceal bleeding. Patients ranged in age from 18 months to 20 years (mean 13.1 years). The median platelet count before PSE was 53.0 (×10/L). The platelet count improved after PSE with values >100,000 in 21 patients (80.8%). Children with prior esophageal varices showed improvement after PSE with only 9 (34.6%) requiring further endoscopic therapy. After PSE, patients developed transient abdominal pain, distention, fever, and perisplenic fluid collections. Serious complications such as splenic abscess, splenic rupture, bleeding, pancreatic infarction, opportunistic infection, or death were not observed. One patient experienced thrombotic complications after PSE and was later diagnosed with myelodysplastic syndrome. CONCLUSIONS: PSE is a safe and effective alternative in the management of pediatric PHTN in select populations. PSE may be a favorable alternative to splenectomy and portal systemic shunting because it preserves functional spleen mass and avoids postprocedure accelerated liver disease or encephalopathy.

Abnormal renal parenchyma accumulation of 123I MIBG in a child with renal artery stenosis.

Hypertension is rare in the pediatric population, however renal artery stenosis (RAS) is an important cause that can be difficult to diagnose. Doppler ultrasound is the first line imaging modality, with computed tomography and magnetic resonance imaging as adjunct modalities, all with variable sensitivity for RAS. The gold standard for evaluation of RAS is invasive selective catheter angiography. We present a unique multimodality case of abnormal radiotracer uptake in the renal parenchyma on 123-Iodine metaiodobenzylguanidine (123I MIBG) scan in a patient with unilateral RAS. RAS is a potential cause of a false positive MIBG scan, and proper recognition may lead to accurate diagnosis.

Image-guided esophageal anastomosis in esophageal atresia.

Infants with tracheoesophageal fistulas may present with complex cardiac disease that may obviate or disrupt a safe operative repair. Here we present a case of an infant who developed cardiac instability during esophageal atresia repair, precluding formal anastomosis after approximation of the distal and proximal esophageal segments. Postoperatively, anastomosis of the esophagus was achieved using an image-guided technique with subsequent dilation. This approach may provide an alternative approach for establishment of esophageal continuity in patients who are high-risk operative candidates.

Embolotherapy of an arterioportal fistula.

We present a complex case of a splanchnic arterioportal vein fistula in a patient who presented with weight loss, abdominal pain, diarrhea, and pancreatitis. We report successful use of the Guglielmi Detachable Coil (GDC) and N-butyl cyanoacrylate glue for the therapeutic embolization of the fistula between the superior mesenteric artery, the common hepatic artery, and the portal vein. On the day following the procedure, the patient reported total remission of the abdominal pain and diarrhea. These results were maintained at 3 months follow-up.