Esophageal Surveillance Practices in Esophageal Atresia Patients: A Survey by the Eastern Pediatric Surgery Network

IntroductionEndoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN).MethodsAn anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices.ResultsThe response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines.ConclusionsOur survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care.Level of EvidenceThis is a prognosis study with level 4 evidence.     

Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia

Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long‐term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health‐care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long‐term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short‐term and long‐term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long‐term surveillance.     

Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.     

Apparently increased trough levels of tacrolimus caused by acute infantile diarrhea in two infants with biliary atresia after liver transplantation

Two infants with biliary atresia who exhibited three-fold increased trough levels of tacrolimus and required reduced doses during episodes of acute infantile diarrhea within 5 months of liver transplantation are described. The cause of the increase was not explained simply by hemoconcentration as a result of significant loss of extracellular fluid during these episodes. It does highlight an important issue: that of the continuing need to carefully monitor the trough levels of tacrolimus in such infants.     

Bilateral choanal atresia and paranasal sinus hypoplasia in an adult patient with hypogammaglobulinaemia

We present an unusual case of a young female patient presenting with bilateral choanal atresia, which was probably acquired, associated with nasal and paranasal sinus hypoplasia and hypogammaglobulinaemia.     

Long-Term Outcome of Adult-to-Adult Living Donor Liver Transplantation for Post-Kasai Biliary Atresia

Our objective was to analyze problems in the perioperative management and long-term outcome of living donor liver transplantation (LDLT) for biliary atresia (BA). Many reports have described the effectiveness of liver transplantation (LT) for BA, particularly in pediatric cases, but little information is available regarding LT in adults (> or =16 years old). Between June 1990 and December 2004, 464 patients with BA underwent LDLT at Kyoto University Hospital, of whom 47 (10.1%) were older than 16 years. In this study, we compared the outcomes between adult (> or =16 years old) and pediatric (<16 years old) patients. The incidence of post-transplant intestinal perforation, intra-abdominal bleeding necessitating repeat laparotomy and biliary leakage was significantly higher (p < 0.0001, <0.001 and <0.001, respectively) in adults. Overall cumulative 1-, 5- and 10-year survival rates in pediatric patients were significantly higher (p < 0.005) than in adults. Two independent prognostic determinants of survival were identified: a MELD score over 20 and post-transplant complications requiring repeat laparotomy. Outcome of LDLT in adult BA patients was poorer than in pediatric patients. It seems likely that LT will be the radical treatment of choice for BA and that LDLT should be considered proactively at the earliest possible stage.     

提高毛发移植的覆盖率

目的探讨显微外科技术自体毛发移植的特点和方法,提高毛发移植成活的覆盖率。方法在较低温度(20°C)环境下,采用边切边缝方法取耳后或枕后毛发,在显微镜下分离成保留周围少量脂肪组织的毛囊族或单株,按需要移植部位不同进行不同的毛囊族或单株并套插的显微外科技术移植共31例。结果31例移植毛发后随访21例,随访时间6~38个月,移植后的毛发能基本覆盖无毛发区的皮肤和瘢痕,移植覆盖率较原来的移植方法提高33%,且外形自然,效果稳定。结论显微外科技术自体毛囊族状(微株小株)或单株移植,具有操作快、损伤小、低温保湿等特点,能较好地保留毛囊周围少量脂肪,在孔与孔的皮肤间隙内再用毛发移植针植入单株毛坯的“套插”移植,更有助于增加毛发覆盖率。     

腹腔镜辅助胆道造影在幼婴延迟性黄疸诊断中的价值

目的探讨腹腔镜辅助胆道造影在幼婴延迟性黄疸诊断中的价值。方法首先脐部置镜观察胆囊及肝脏情况,如果胆囊大小正常或基本正常,通过右肋缘下trocar孔拖出胆囊底部,部分患儿需游离胆囊系膜。切开胆囊,插管行胆道造影。如果胆囊呈纤维闭锁状态,不能拖出腹腔外,中转开腹。切开胆囊插管失败,放弃造影。结果12例患儿直接从拖出胆囊行胆道造影显示婴儿肝炎综合征或胆汁淤积8例;2例显示为胆管发育不良;2例为肝内胆管闭锁。5例从肝脏游离胆囊系膜后拖出造影,2例显示为胆管发育不良,3例提示胆道闭锁。其余21例患儿胆囊呈条索状,不同程度肝硬化,放弃胆道造影,诊断为胆道闭锁。所有胆道闭锁患儿均行Kasai手术。结论腹腔镜探查、胆道造影是诊断胆道闭锁、鉴别幼婴延迟性黄疸简便、准确、安全的方法,能够显示整个胆树的解剖结构,避免过大或不必要的创伤。