Gockel Ines, Gaedertz C, Hain H-J, Winckelmann U, Albani M, Lorenz D
Klinik für Allgemein- und Viszeralchirurgie, Dr.-Horst-Schmidt-Kliniken GmbH, Wiesbaden.
Chirurg. 2003 Aug;74(8):753-6. doi: 10.1007/s00104-003-0680-0.
The Rapunzel syndrome is a rare manifestation of a gastric trichobezoar with a "tail" extending throughout the small intestine and sometimes even to the colon. We report on the surgical removal of such a bezoar in a 4-year-old patient by gastrotomy--the third published case in the German literature. The syndrome is mainly seen in young girls with trichophagia psychodynamically associated with early childhood deprivation and a high comorbidity of serious pediatric psychiatric disorders. The symptoms are nonspecific and may mimic those of other pathologic gastrointestinal conditions. Clinical characteristics are a movable mass in the epigastrium and alopecia. The therapy of choice is surgery of the trichobezoar together with the whole intestinal "tail," as in most cases endoscopic removal fails due to the large extension. Early diagnosis and treatment of the Rapunzel syndrome is of eminent importance in order to avoid later fatal complications such as gastric perforation and intestinal necroses. Intensive psychiatric follow-up is mandatory for preventing relapses.
长发公主综合征是胃内毛石症的一种罕见表现,其“尾巴”贯穿小肠,有时甚至延伸至结肠。我们报告了一例通过胃切开术为一名4岁患者切除此类毛石的病例——这是德国文献中报道的第三例。该综合征主要见于有食毛癖的年轻女孩,这种癖好与童年早期的情感剥夺以及严重儿科精神疾病的高共病率存在心理动力学关联。其症状不具特异性,可能与其他病理性胃肠道疾病的症状相似。临床特征为上腹部可移动肿块和脱发。首选治疗方法是连同整个肠道“尾巴”一起切除毛石,因为在大多数情况下,由于其延伸范围大,内镜切除往往失败。为避免后期诸如胃穿孔和肠坏死等致命并发症,早期诊断和治疗长发公主综合征极为重要。为防止复发,必须进行强化的精神科随访。