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[一名因急性发热性肠梗阻接受手术的患者出现肠系膜脂膜炎、急性阑尾炎和克尼格综合征的罕见三联征:病例报告]

[A rare triple combination of mesenteric panniculitis, acute appendicitis and König´s syndrome in a patient operated for acute febrile intestinal obstruction: a case report].

作者信息

Banza Manix Ilunga, Kasanga Trésor Kibangula, Mukakala Augustin Kibonge, Nafatalewa Dimitri Kanyanda, Milinganyo Eddy Wasso, Lisasi Wolf, Kisimba Emmy Manda, Ngoma Mylord Kambu, Yumba Serges Ngoie, N'dwala Yannick Tietie Ben

机构信息

Département de Chirurgie, Cliniques Universitaires de Lubumbashi, Lubumbashi, République Démocratique du Congo.

Département de Réanimation-Anesthésie, Cliniques Universitaires de Lubumbashi, Université de Lubumbashi, Haut Katanga, République Démocratique du Congo.

出版信息

Pan Afr Med J. 2023 May 24;45:57. doi: 10.11604/pamj.2023.45.57.19448. eCollection 2023.

Abstract

Mesenteric panniculitis is a primary inflammation of the mesentery with variable necrosis, inflammation and fibrosis of the fatty tissue. It can be idiopathic (primary) or secondary (associated) to other diseases, asymptomatic and accidentally discovered or revealed by abdominal pain or complications (intestinal obstruction or peritonitis). We here report the case of a 53-year-old patient, admitted with acute abdominal pain, cessation of the transit of materials and gases, in a febrile context. Patient's history included chronic abdominal pain suggesting König´s syndrome and epigastralgia lasting several years. Physical examination showed sore face and abdominal examination revealed mild bloating, marked tenderness in the right iliac fossa (RIF) and in the periumbilical region, without guarding or rigidity and borygms heard on auscultation and with normal rectal examination. A diagnosis of bowel obstruction and fever was made, with suspicion of meso-celiac appendicitis. Abdominal X-ray without preparation and ultrasound confirmed the diagnosis of bowel occlusion. Exploratory laparotomy revealed functional stenosis of the ileum (König´s syndrome) at 1.20m from the ileocecal junction, with multiple adhesions. Adhesiolysis revealed hyperemic appendix measuring 15cm long, whose anatomo pathological examination showed a mucous membrane with inflammatory infiltrate and a wall rich in polynuclear cells. Infiltration of the ileal mesentery causing color change (reddish and greyish in some areas) and small nodosities with friability and tearing on simple handling led to suspicion of mesenteric panniculitis, then confirmed by anatomopathological examination, showing inflammatory reaction in the fatty tissue specimen with infiltration by macrophages, associated with necrotic patches and degeneration. Treatment was based on bowel emptying, anterograde appendectomy, and a combination of corticosteroid (Dexamethasone 24 mg/day) and chymotrypsin (10000 IU/day). The patient´s outcome was good and he was discharged in the 10 postoperative day. The patient underwent clinical and paraclinical follow-up (3 months) for another unknown associated pathology or a pathology that may have occurred early.

摘要

肠系膜脂膜炎是一种肠系膜的原发性炎症,伴有脂肪组织不同程度的坏死、炎症和纤维化。它可以是特发性(原发性)的,也可以是继发性(与其他疾病相关)的,可无症状,偶然发现,或由腹痛或并发症(肠梗阻或腹膜炎)引发。我们在此报告一例53岁患者,因急性腹痛、停止排气排便,伴有发热入院。患者既往史包括提示克尼格综合征的慢性腹痛和持续数年的上腹部疼痛。体格检查显示面部压痛,腹部检查发现轻度腹胀,右下腹(RIF)和脐周区域有明显压痛,无肌紧张或强直,听诊可闻及肠鸣音,直肠检查正常。诊断为肠梗阻和发热,怀疑为中腹部阑尾炎。未做准备的腹部X线检查和超声检查证实了肠梗阻的诊断。剖腹探查发现距回盲部1.20米处回肠功能性狭窄(克尼格综合征),伴有多处粘连。粘连松解术中发现阑尾充血,长15厘米,其解剖病理检查显示黏膜有炎症浸润,壁富含多核细胞。回肠系膜浸润导致颜色改变(某些区域呈红色和灰色),并有小结节,质地脆,轻轻触碰即撕裂,这引发了对肠系膜脂膜炎的怀疑,随后经解剖病理检查证实,显示脂肪组织标本中有炎症反应,伴有巨噬细胞浸润,并有坏死灶和变性。治疗基于肠道排空、顺行阑尾切除术,以及皮质类固醇(地塞米松24毫克/天)和糜蛋白酶(10000国际单位/天)联合使用。患者预后良好,术后第10天出院。患者接受了临床和辅助检查随访(3个月),以排查其他未知的相关病理情况或可能早期出现的病理情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/231e/10460100/6d69d09706c5/PAMJ-45-57-g001.jpg

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