Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis.

BACKGROUND/PURPOSE: 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. METHODS: We retrospectively analyzed the characteristics of all patients with PFIC undergoing PEBD in our department from 1994 to 2008. The course of serum bile acids, pruritus, and liver enzymes was assessed in a regular follow-up. RESULTS: Twenty-four patients underwent PEBD. Thirteen patients (54%) improved significantly, with a normalization of serum bile acids (P < .001 vs postoperatively) and lessened pruritus (P < .05 vs preoperatively) at 12 months after PEBD. None of these patients showed progression of cholestasis during a median follow-up of 9.8 years (range, 1.6-14.3 years). Partial external biliary diversion failed to normalize bile acids in 11 patients, of whom 9 required secondary LTX at a 1-year follow-up, with a median interval of 1.9 years (range, 0.5-3.8 years). All 7 patients (100%) with liver cirrhosis at the time of PEBD and 2 of 17 patients without cirrhosis (12%) required secondary LTX (P < .001). CONCLUSIONS: 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.

Penile block is associated with less urinary retention than caudal anesthesia in distal hypospadia repair in children.

BACKGROUND: Caudal anesthesia (CA) is widely recommended due to excellent analgesia in distal hypospadia repairs, but its potential side effect on urinary retention interferes with patient comfort. To objective is to determine the impact of CA versus penile block (PB) on postoperative micturition. METHODS: Sixty distal hypospadia repairs performed over a 48-month period were analyzed concerning the impact of applicated analgesia on postoperative micturition. Inclusion criteria were Mathieu or Snodgrass procedures, use of a non-dribbling urethral stent, CA or PB. Endpoint was first postoperative micturation (<6 h), or treatment of delayed postoperative micturation (>6 h). RESULTS: CA was used in 27 and PB in 33 cases. Compared groups had similar age (mean: 31 months, range 12-68 vs. 28 months, range: 14-145), weight (mean: 13 kg, range 9-18 vs. 15 kg, range 8-59), operation duration (mean 61 min, range 30-105 vs. mean 67 min, range 35-120) and surgical technique (Mathieu/Snodgrass: 7/20 CA vs. 19/14 PB). Micturation was significantly less impaired in the PB than CA group (5/33 vs. 15/27; p < 0.05). Delayed or non-micturation was successfully treated in 4 and 14 patients, respectively, by cholinergic agonists. One patient of each group required a suprapubic drain on the first operative day after unsuccessful medical treatment. CONCLUSIONS: In our series, children undergoing distal hypospadia repair experienced significantly less impaired micturition when using penile block instead of caudal anesthesia. We recommend penile block as the first choice perioperative analgesia, when spontaneous postoperative micturition must be guaranteed.