Scholarlink

Background <p>Hereditary spherocytosis (HS) is an inherited hemolytic anemia in which splenectomy may be indicated in moderate to severe disease. Chronic hemolysis increases the risk of cholelithiasis and biliary complications, but optimal timing of cholecystectomy remains uncertain.</p> Methods <p>A retrospective single-center analysis of pediatric patients with moderate to severe HS who underwent laparoscopic splenectomy between January 2017 and January 2024. Evaluated variables included disease severity, demographics, cholelithiasis, biliary manifestations, requirement for endoscopic retrograde cholangiopancreatography (ERCP), operative time, postoperative complications, and length of hospital stay.</p> Results <p>Thirteen patients were included (5 male, 8 female; mean age 11.9 ± 4.3 years): 6 with moderate HS and 7 with severe HS. Cholelithiasis was present in 9 patients (69.2%). Major biliary complications (biliary pancreatitis or obstructive jaundice) occurred in 4/7 patients with severe HS (57.1%) compared with 1/6 patients with moderate HS (16.7%); ERCP was required in the same proportions. Mean operative time was 124.7 ± 44.7&#xa0;min and mean hospital stay was 6.1 ± 1.9 days. No major postoperative surgical complications occurred; one patient developed pleural effusion (Clavien–Dindo grade I). Hemoglobin, reticulocyte count, and bilirubin levels improved after surgery and remained improved during follow-up.</p> Conclusions <p>Laparoscopic splenectomy was safe and effective in this small cohort of children with hereditary spherocytosis. These preliminary findings suggest that children with severe HS and cholelithiasis may represent a subgroup in whom the potential role of concomitant or early cholecystectomy warrants prospective evaluation. Because of the small sample size, retrospective design and absence of a direct comparison between prophylactic and delayed cholecystectomy strategies, these findings should not be interpreted as a clinical recommendation. The data are insufficient to support a subgroup-specific conclusion for children with moderate HS.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Minimally invasive management of pediatric hereditary spherocytosis: severity-stratified biliary risk and indications for simultaneous cholecystectomy and biliary intervention at laparoscopic splenectomy – a single-center retrospective study

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年7月25日

查看全文
  • Radek Ambrož,
  • Petr Birke,
  • Jiří Kysučan,
  • Martin Stašek,
  • Štefan Kolcún,
  • Dušan Klos,
  • Martin Loveček,
  • Barbora Ludíková,
  • Zbyněk Novák,
  • Dagmar Pospíšilová

摘要

Background

Hereditary spherocytosis (HS) is an inherited hemolytic anemia in which splenectomy may be indicated in moderate to severe disease. Chronic hemolysis increases the risk of cholelithiasis and biliary complications, but optimal timing of cholecystectomy remains uncertain.

Methods

A retrospective single-center analysis of pediatric patients with moderate to severe HS who underwent laparoscopic splenectomy between January 2017 and January 2024. Evaluated variables included disease severity, demographics, cholelithiasis, biliary manifestations, requirement for endoscopic retrograde cholangiopancreatography (ERCP), operative time, postoperative complications, and length of hospital stay.

Results

Thirteen patients were included (5 male, 8 female; mean age 11.9 ± 4.3 years): 6 with moderate HS and 7 with severe HS. Cholelithiasis was present in 9 patients (69.2%). Major biliary complications (biliary pancreatitis or obstructive jaundice) occurred in 4/7 patients with severe HS (57.1%) compared with 1/6 patients with moderate HS (16.7%); ERCP was required in the same proportions. Mean operative time was 124.7 ± 44.7 min and mean hospital stay was 6.1 ± 1.9 days. No major postoperative surgical complications occurred; one patient developed pleural effusion (Clavien–Dindo grade I). Hemoglobin, reticulocyte count, and bilirubin levels improved after surgery and remained improved during follow-up.

Conclusions

Laparoscopic splenectomy was safe and effective in this small cohort of children with hereditary spherocytosis. These preliminary findings suggest that children with severe HS and cholelithiasis may represent a subgroup in whom the potential role of concomitant or early cholecystectomy warrants prospective evaluation. Because of the small sample size, retrospective design and absence of a direct comparison between prophylactic and delayed cholecystectomy strategies, these findings should not be interpreted as a clinical recommendation. The data are insufficient to support a subgroup-specific conclusion for children with moderate HS.