Al Haq Afaq Mobin, Rasheedi Alaa, Al Farsi Muayed, Mehdar Abeer, Yousef Yasmin, Rasheed Khalid, Binyahib Soliman
Surgery Department, Section of Pediatric Surgery, Ministry of National Guard-Health Affairs, Jeddah, Saudi Arabia.
King Abdullah International Medical Research Center, Jeddah, Saudi Arabia.
Int J Pediatr Adolesc Med. 2021 Dec;8(4):212-220. doi: 10.1016/j.ijpam.2020.05.003. Epub 2020 Jun 18.
Basidiobolomycosis is a rare fungal disease, lately appearing in the gastrointestinal system of pediatric patients. Their clinical presentation resembles that of lymphoma or granulomatous inflammations. This non-specific presentation makes Gastrointestinal Basidiobolomycosis (GIB) a diagnostic challenge.
We are reporting the largest series of pediatric GIB, from Saudi Arabia. 12 patients were diagnosed between January 2012 and December 2019, between the ages of 16 months and 8 years.
The most common symptoms were fever and abdominal pain. Further examination revealed an abdominal mass. Biopsy of the mass was the mainstay of diagnosis, with histological findings of typical filamentous fungal hyphae and zygospores, surrounded by eosinophils.
Role of surgery was limited to establishing the diagnosis and dealing with complications. Antifungal medication was the cornerstone of treatment in all our patients. Three of our patients were exceptional with complications such as entero-cutaneal fistula, entero-enteric fistula and short bowel syndrome. These complications have not been previously reported. We have discussed the challenges related to their management.The diagnosis of GIB in pediatric patients with abdominal mass, needs a high index of suspicion. We believe outcome depends on the severity of disease, involvement of surrounding tissues and presence of complications at the time of diagnosis.
担子菌病是一种罕见的真菌病,最近出现在儿科患者的胃肠道系统中。其临床表现类似于淋巴瘤或肉芽肿性炎症。这种非特异性表现使得胃肠道担子菌病(GIB)成为一项诊断挑战。
我们报告了来自沙特阿拉伯的最大系列儿科GIB病例。2012年1月至2019年12月期间诊断出12例患者,年龄在16个月至8岁之间。
最常见的症状是发热和腹痛。进一步检查发现腹部有肿块。肿块活检是诊断的主要依据,组织学检查发现典型的丝状真菌菌丝和接合孢子,周围有嗜酸性粒细胞。
手术的作用仅限于确立诊断和处理并发症。抗真菌药物是我们所有患者治疗的基石。我们的3例患者出现了诸如肠皮肤瘘、肠肠瘘和短肠综合征等并发症,这些并发症以前未曾有过报道。我们讨论了与这些并发症处理相关的挑战。对于有腹部肿块的儿科患者,GIB的诊断需要高度的怀疑指数。我们认为预后取决于疾病的严重程度、周围组织的受累情况以及诊断时是否存在并发症。