Ahmed Salaar, Dilawar Bakhtawar, Moeen Sarosh, Dilawar Shahzadi, Sheikh Hafsa, Arain Muhammad Anwar
Medical College, Aga Khan University, Stadium Road, Karachi, 74800, Pakistan.
Department of Surgery, Aga Khan University, Karachi, Pakistan.
J Med Case Rep. 2024 Dec 22;18(1):619. doi: 10.1186/s13256-024-04976-4.
Kaposiform hemangioendothelioma is a rare vascular tumor primarily occurring in infants and children. The most common sites for kaposiform hemangioendothelioma are extremities, with very few cases of abdominal kaposiform hemangioendothelioma reported in neonates. Making a diagnosis of Kaposiform hemangioendothelioma can be challenging when the patient presents with generalized symptoms such as bilious vomiting and constipation that can be attributed to other more common causes of intestinal obstruction.
A 28-day-old Pakistani baby girl presented with bilious vomiting and constipation. The abdomen was distended, and scans showed generalized dilation of bowel loops. A preliminary diagnosis of intestinal obstruction due to volvulus was made, and the patient underwent an exploratory laparotomy. Postoperatively, the patient remained unwell with profuse aspirates in nasogastric and no improvement in ileus. Repeat scans were again suggestive of midgut volvulus, which was initially managed conservatively, but later redo surgery was done. Ischemic bowel was resected with histopathology showing kaposiform hemangioendothelioma. In the meantime, the baby developed an abdominal collection, infection, and short gut syndrome due to resection of the bowel. The family was counseled regarding the prognosis, and the patient was transferred from hospital to home upon request. The baby passed away 2 days later.
While extremely rare, abdominal kaposiform hemangioendothelioma can be an important and potentially lifesaving differential diagnosis to consider in neonates and infants with recurring and nonresolving intestinal obstruction. The management is complex, and prognosis can be poor in diffuse, congested lesions involving a large part of the gut.
卡波西样血管内皮瘤是一种罕见的血管肿瘤，主要发生于婴幼儿。卡波西样血管内皮瘤最常见的发病部位是四肢，新生儿腹部卡波西样血管内皮瘤的报道极少。当患者出现如胆汁性呕吐和便秘等可归因于其他更常见肠梗阻原因的全身症状时，诊断卡波西样血管内皮瘤具有挑战性。
一名28天的巴基斯坦女婴出现胆汁性呕吐和便秘。腹部膨隆，扫描显示肠袢普遍扩张。初步诊断为肠扭转导致的肠梗阻，患者接受了剖腹探查术。术后，患者病情仍未好转，鼻胃管引出大量液体，肠梗阻无改善。复查扫描再次提示中肠扭转，最初进行了保守治疗，但后来进行了再次手术。切除了缺血肠段，组织病理学显示为卡波西样血管内皮瘤。与此同时，由于肠切除，婴儿出现了腹腔积液、感染和短肠综合征。向家属告知了预后情况，患者应家属要求出院回家。婴儿两天后去世。
虽然极为罕见，但腹部卡波西样血管内皮瘤对于患有反复性和难治性肠梗阻的新生儿和婴儿来说，是一个重要的、可能挽救生命的鉴别诊断。其治疗复杂，对于累及大部分肠道的弥漫性、充血性病变，预后可能较差。
