A Challenging Case of Focal Extrahepatic Duct Obstruction/Hypoplasia in Alagille Syndrome

Abstract

In premature infants, adaptation of the bowel (including intestinal dilation and lengthening, growth of the villi, alterations of motility, and hormonal changes) is a important process that must be completed before adequate absorption of nutrients and weight gain can occur after intestinal surgery (2,5,6). Establishment of a high stoma can influence bowel adaptation and lead to various problems, such as fluid and electrolyte imbalance, abnormal acidbase status, nutritional deficiency/malnutrition, and specific stoma complications (stomal necrosis, prolapse, skin breakdown, parastomal hernia, and leakage from appliances) (2,4,7).The present study provides the first objective evidence that stool recycling has nutritional benefits for the preterm infant with a high stoma. As shown in Figure 1, weight gain was better in our infants who received stool recycling (recycling (þ) group) than in those without recycling. In addition, the recycling (þ) group recovered from anemia and serum albumin (an index of nutritional status) was normalized, whereas only 1 infant from the recycling (À) group had a normal level. Poor tissue integrity is considered to contribute to stomal prolapse in neonates with enterostomy (8). Although there was no significant difference of serum albumin between the recycling (þ) group and the recycling (À) group in our study, recovery from anemia and weight gain were better in the former group. Improvement of the nutritional state by recycling stool was considered to contribute to recovery of tissue strength, which may have reduced the incidence of stoma prolapse.Pataki et al (3) recently reported that storing stool from the oral side of the stoma led to a risk of bacterial overgrowth, whereas immediate or continuous aspiration from the stoma bag may prevent overgrowth. We used a system in which stool was aspirated continuously from the oral opening of the stoma and briefly stored in a sealed cylinder, after which it was recycled to the anal side of stoma as rapidly as possible. Before stool recycling was initiated, we infused probiotics with approximately 5 mL of glucose solution into the distal intestine, and then continuously infused 5% glucose solution with electrolytes (approximately 1-3 mL/h) for 1 or 2 days. To establish a more effective and safer recycling method and to better evaluate the effects of recycling, accumulation of more data on VLBW infants with a high stoma undergoing stool recycling is needed.

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants

“…Han et al ( 13 ) reported that the patent biliary tree of 3 infants with AGS was not presented by intraoperative cholangiography, resulting in a misdiagnosis of BA. Furthermore, 26 patients with AGS from six studies were misdiagnosed with BA and received KPE based on intraoperative cholangiography ( 14 – 16 , 30 – 32 ). Unfortunately, AGS exhibited considerable overlapping features with BA, leading to improper KPE and even worsening the patient’s outcome ( 33 ).…”

Province‐wide Biliary Atresia Home Screening Program in British Columbia

“…BA and AGS may be indistinguishable and pose diagnostic challenge for neonatal cholestasis. AGS can share biochemical, imaging, histological, and operative cholangiogram features with BA (23). Although genetic testing may help to differentiate the diseases, the results often take several weeks (>4 weeks) to return at a time when further delay in BA diagnosis and surgical intervention may compromise outcome.…”

“…BA screening using ISCC has been implemented in European and South American countries (15,16). ISCC screening has been reported to be highly feasible, effective, and cost-effective in both Taiwan and Japan (7,8,17,18) The Taiwanese national ISCC BA screening program, in place since 2004, has eliminated late BA referrals after 90 days of age with improved 5-year native liver survival (8). In the Tochigi Prefecture of Japan, where the stool color card was first developed and introduced in 1994 by Matsui, the native liver 5 and 10 year survival rates increased by >20% during 1994-2011 compared with other Japanese jurisdictions without an ISCC screening program (18).…”

Protocols of Investigation of Neonatal Cholestasis—A Critical Appraisal

“…Intraoperative cholangiography is considered the gold standard for diagnosing BA, but the method can be misleading in up to 20% of cases [ 7 , 46 , 47 , 48 , 49 ]. Pathophysiological considerations can help us understand why diagnostic errors occur in intraoperative cholangiography.…”

“…Pathophysiological considerations can help us understand why diagnostic errors occur in intraoperative cholangiography. Extrahepatic bile flow blockage does not occur exclusively from complete mechanical obstruction caused by BA but also from extrahepatic bile duct hypoplasia caused by decreased intrahepatic bile flow, or extrahepatic bile duct agenesis both associated with Alagille syndrome [ 49 , 50 , 51 ]. Inadvertently submitting an infant with Alagille syndrome to a Portoenterostomy based on a false-positive diagnosis of BA can lead to grievous prognostic consequences [ 52 , 53 ].…”