Tambuzzi Stefano, Gentile Guendalina, Boracchi Michele, Zoja Riccardo, Gentilomo Andrea
Laboratorio di Istopatologia Forense e Microbiologia Medico Legale, Sezione di Medicina Legale e delle Assicurazioni, Dipartimento di Scienze Biomediche per la Salute , Università degli Studi di Milano, Via Luigi Mangiagalli 37, 20133, Milan, Italy.
Dipartimento di Scienza Giuridiche "Cesare Beccaria", Università degli Studi di Milano, Via festa del Perdono, 7, 20122, Milan, Italy.
Forensic Sci Med Pathol. 2023 Jun;19(2):273-279. doi: 10.1007/s12024-022-00562-6. Epub 2022 Dec 2.
The term "cocoon syndrome" defines a sclerosing encapsulating peritonitis (SEP) that involves a chronic fibrotic inflammatory reaction of the parietal peritoneum and of the viscera leading to a complete sclerosis. The cocoon that is formed causes an incarceration of the intestinal loops with severe complications leading to high mortality. We are presenting the case of a 15-year-old young man that underwent surgery for appendectomy and that was evaluated for having a regular abdominal state. During the post-surgery period, however, several episodes of intestinal occlusion required further surgical interventions leading to a right hemicolectomy. The presence of a fibrotic-adhesive ligneous peritonitis with blended intestinal loops, severely thickened walls, and intestinal scaring stenosis was observed during his second surgical operation. A stenosis of the colostomy led to a worsening of the vital signs of the young man with the onset of a cardiac failure and subsequent decease. Macroscopic autopsy examination and histological analysis confirmed the severe obstructive adhesive encapsulating abdominal context allowing to trace back the cause of death to a cocoon syndrome. Since no predisposing factor could be found, we hypothesized that this case could be characterized by an excessive peritoneal reactivity due to surgical appendectomy. Cocoon syndrome is a rare pathology, and its microscopic features are seldomly observed and could be underestimated. We present a directly observed case with a very substantial macroscopic and microscopic context.
“茧状综合征”一词定义了一种硬化性包裹性腹膜炎(SEP),它涉及壁层腹膜和内脏的慢性纤维化炎症反应,导致完全硬化。形成的“茧”会导致肠袢嵌顿,引发严重并发症,死亡率很高。我们报告一例15岁青年男性病例,该患者接受了阑尾切除术,术后腹部情况正常。然而,在术后期间,多次肠梗阻发作需要进一步手术干预,最终进行了右半结肠切除术。在第二次手术中,发现存在纤维化粘连性木质化腹膜炎,肠袢融合,肠壁严重增厚,伴有肠瘢痕狭窄。结肠造口狭窄导致该青年男性生命体征恶化,出现心力衰竭并随后死亡。宏观尸检检查和组织学分析证实存在严重的梗阻性粘连包裹性腹部病变,死因可追溯至茧状综合征。由于未发现诱发因素,我们推测该病例可能的特征是阑尾切除术后腹膜反应过度。茧状综合征是一种罕见的病理情况,其微观特征很少被观察到,可能被低估。我们展示了一个有非常丰富宏观和微观情况的直接观察病例。