Agarwal Krati, Agarwal Radhika, Agarwal Vinayak, Goel Sonal Ratnakar
Department of Microbiology, AIIMS, Gorakhpur, India.
Department of Pathology, M.S. Pathology, Gorakhpur, India.
Int J Surg Case Rep. 2025 Jan;126:110714. doi: 10.1016/j.ijscr.2024.110714. Epub 2024 Dec 16.
Rapunzel syndrome is a rare condition that results from trichotillomania (compulsive hair pulling) and trichophagia (hair eating), causing a trichobezoar (hairball) to form This syndrome typically affects young females with psychiatric conditions and presents with symptoms like chronic abdominal pain, nausea, vomiting, and malnutrition. The condition is often diagnosed late, leading to serious gastrointestinal complications.
A 19-year-old female from a rural community presented with chronic abdominal pain, vomiting, and nutritional deficiencies, including scaly skin and koilonychia. Over time, her symptoms worsened, and she discovered a palpable abdominal mass. Clinical evaluation, including an upper gastrointestinal endoscopy, revealed a large trichobezoar extending from the lower esophagus to the pylorus. The patient had a history of pica and compulsive behaviors, suggesting psychiatric involvement.
The endoscopy revealed a 20 × 13.5 × 9 cm trichobezoar. After successful surgical removal, the patient's gastrointestinal symptoms improved. Post-operatively, she received nutritional support and was referred for psychiatric evaluation to manage trichotillomania and trichophagia, with the aim of preventing recurrence in a private practice setting.
This case highlights the need for early recognition of Rapunzel syndrome in patients with chronic gastrointestinal symptoms and nutritional deficiencies. A multidisciplinary approach is essential for effective management and preventing recurrence.
长发公主综合征是一种罕见病症,由拔毛癖(强迫性拔毛)和食毛癖(食毛)导致毛粪石(毛球)形成。该综合征通常影响患有精神疾病的年轻女性,表现为慢性腹痛、恶心、呕吐和营养不良等症状。此病常被误诊,导致严重的胃肠道并发症。
一名来自农村社区的19岁女性,出现慢性腹痛、呕吐以及包括皮肤鳞屑和匙状甲在内的营养缺乏症状。随着时间推移,她的症状加重,并且发现腹部有可触及的肿块。包括上消化道内镜检查在内的临床评估显示,一个巨大的毛粪石从食管下端延伸至幽门。该患者有异食癖和强迫行为史,提示有精神方面的问题。
内镜检查发现一个20×13.5×9厘米的毛粪石。成功手术切除后,患者的胃肠道症状得到改善。术后,她接受了营养支持,并被转介至精神科进行评估,以治疗拔毛癖和食毛癖,目的是在私人诊所环境中预防复发。
该病例凸显了对于有慢性胃肠道症状和营养缺乏的患者,早期识别长发公主综合征的必要性。多学科方法对于有效管理和预防复发至关重要。