Journal of pediatric surgery
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The surgical treatment for patients with progressive familial intrahepatic cholestasis (PFIC) is either liver transplantation (LTX) or partial external biliary diversion (PEBD). Both procedures achieve a good short-term outcome. However, the treatment strategy for these children remains controversial because the long-term outcome after PEBD is unknown. The aim of our study was to assess the long-term outcome and complications after PEBD in our institution. ⋯ Clinical improvement with normalization of serum bile acids within 1 year was associated with an excellent long-term outcome in patients with PEBD. The presence of liver cirrhosis at the time of PEBD indicated an unfavorable outcome. Thus, we recommend primary LTX only in PFIC patients with liver cirrhosis.
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Aneurysms are rare in children. Causes include congenital, traumatic, inflammatory, and infectious etiologies. When and how to best surgically treat arterial aneurysms in a child remain unclear. We present the case of a 3-month-old child with an aneurysm of the left common iliac artery, which was first detected on abdominal ultrasound and was successfully repaired with a cadaveric vein graft.
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The rationale for in utero repair of myelomeningocele (MMC) in the context of pathologic observations, animal models, and outcomes from the initial experience with human fetal MMC repair is presented. This has now culminated in a randomized trial, Management of Myelomeningocele Study, the findings of which are listed. The story is focused on the milestone contributions of members of the Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia on the road to successful fetal surgery for spina bifida. This is now performed in selected patients and presents an additional therapeutic alternative for expectant mothers carrying a fetus with MMC.
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Clinical Trial
A prospective study of safety and satisfaction with same-day discharge after laparoscopic appendectomy for acute appendicitis.
This study examines the safety and patient satisfaction in discharging children undergoing laparoscopic appendectomy (LapAppy) for acute appendicitis on the day of surgery. ⋯ Routine same-day discharge after pediatric LapAppy for acute appendicitis is safe, with good parent satisfaction.
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Venovenous extracorporeal membrane oxygenation (VV-ECMO) has been used as a management strategy for neonates with refractory pulmonary failure. However, VV-ECMO has been limited in neonates secondary to cannula design and patient size. Herein, we describe the use of a bicaval dual-lumen catheter for VV-ECMO in neonates. ⋯ The dual-lumen bicaval catheter can be safely used in neonates with minimal complications and is our preferred method for VV-ECMO support in the neonatal population.