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OBJECTIVE We report the largest clinical experience to date of surgically treated patients with blue rubber bleb nevus syndrome (BRBNS). SUMMARY BACKGROUND DATA BRBNS is a rare congenital disorder presenting with multifocal venous malformations of the skin, soft tissues, and gastrointestinal (GI) tract. Patients with BRBNS develop anemia from chronic GI(More)
PURPOSE The purpose of this study was to determine the outcomes among three different surgical approaches for performing an aortopexy to treat severe tracheomalacia (STM). METHODS A retrospective review was performed for all patients who underwent an aortopexy by pediatric surgeons at a single institution during 1997-2012. Data collected included details(More)
PURPOSE Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves(More)
We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy(More)
PURPOSE The Foker process (FP) uses tension-induced growth for primary esophageal reconstruction in patients with long gap esophageal atresia (LGEA). It has been less well described in LGEA patients who have undergone prior esophageal reconstruction attempts. METHODS All cases of LGEA treated at our institution from January 2005 to April 2014 were(More)
BACKGROUND/PURPOSE Sclerotherapy for vascular malformations is often limited by luminal recanalization. This study examined whether an injectable tissue-engineered construct could prevent this complication in a rabbit model of venous sclerotherapy. METHODS Ethanol sclerotherapy of a temporarily occluded jugular vein segment was performed in 46 rabbits,(More)
PURPOSE Tracheobronchomalacia (TBM) is associated with esophageal atresia, tracheoesophageal fistulas, and congenital heart disease. TBM results in chronic cough, poor mucous clearance, and recurrent pneumonias. Apparent life-threatening events or recurrent pneumonias may require surgery. TBM is commonly treated with an aortopexy, which indirectly elevates(More)
The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We(More)
Surgical resections of massive sacrococcygeal teratomas (SCTs) carry significant risk due to baseline hemodynamic instability and the potential for significant hemorrhage. In this case report, a fetus with sacrococcygeal teratoma developed high-output cardiac instability at 32 weeks' gestation. After urgent cesarian delivery, a damage-control operation(More)
Placement of gastrostomy tubes in infants and children has become increasingly commonplace. A historical emphasis on use of open gastrostomy has been replaced by less invasive methods of placement, including percutaneous endoscopic gastrostomy and laparoscopically assisted gastrostomy procedures. Various complications, ranging from minor to the more severe,(More)