消化系统疑难疾病诊疗思维及病例解析(第二辑)
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三、参考文献

[1] FOO KT,NG KC,RAUFF A,et al.Unusual small intestinal obstruction in adolescent girls:the abdominal cocoon.Bri J Surg,1978,65:427-430.

[2] LIU,HY,WANG YS,YANG WG,et al.Diagnosis and surgical management of abdominal cocoon:results from 12 cases.Acta Gastroenterol Belg,2009,72:447-449.

[3] TARQUINI R,COLAGRANDE S,ROSSELLI M,et al.Complete resolution of primary sclerosing peritonitis(“abdominal cocoon”)following long term therapy for Tropheryma whipplei:a case report and review of literature.BMJ Case Rep,2009,2009:pii:bcr04.2009.1810.

[4] IBRAHIM NA,OLUDARA MA.Abdominal cocoon in an adolescent male patient.Trop Doct,2009,39:254-256.

[5] SUH WN,LEE SK,CHANG H,et al.Sclerosing encapsulating peritonitis(abdominal cocoon)after abdominal hysterectomy.The Korean Journal of Internal Medicine,2007,22:125-129.

[6] NAKAMOTO H.Encapsulating peritoneal sclerosis——a clinician's approach to diagnosis and medical treatment.Peritoneal Dialysis International,2005,25(Suppl 4):S30-38,2005.

[7] Wani I,Ommid M,Waheed A,et al.Tuberculous abdominal cocoon:Original article.Turkish Journal of Trauma & Emergency Surgery Tjtes,2010,16(6):508-510.

[8] LI N,ZHU W,LI Y,et al.Surgical treatment and perioperative management of idiopathic abdominal cocoon:single-center review of 65 cases.World Journal of Surgery,2014,38:1860-1867.

[9] SINGH B,GUPTA S.Abdominal cocoon:a case series.Int J Surg 2013,11:325-328.

[10] SERTER A,KOCAKOC E,CIPE G.Supposed to be rare cause of intestinal obstruction;abdominal cocoon:report of two cases.Clinical Imaging,2013,37:586-589.

[11] SALAMONE G,ATZENI J,AGRUSA A,et al.A rare case of abdominal cocoon.Annali Italiani Di Chirurgia,2013:ppi:S2239253X13021531.

[12] MALIK SA,JAVED MA,MIAN,MA.Abdominal cocoon(sclerosing encapsulating peritonitis):a rare cause of intestinal obstruction.JCPSP,2012,22:171-173.

[13] KAUR R,CHAUHAN D,DALAL U,et al.Abdominal cocoon with small bowel obstruction:two case reports.Abdominal Imaging,2012,37:275-278.

[14] ACAR T,KOKULU I,ACAR N,et al.Idiopathic encapsulating sclerosing peritonitis.Ulusal Cerrahi Dergisi,2015,31:241-243.

[15] YAVUZ R,AKBULUT S,BABUR M,et al.Intestinal obstruction due to idiopathic sclerosing encapsulating peritonitis:a case report.Iranian Red Crescent Medical Journal,2015,17:e21934.

[16] SOLMAZ A,TOKOCIN M,ARICI S,et al.Abdominal cocoon syndrome is a rare cause of mechanical intestinal obstructions:a report of two cases.The American Journal of Case Reports,2015,16:77-80.

[17] HABIB SM,BETJES MG,FIEREN MW,et al.Management of encapsulating peritoneal sclerosis:a guideline on optimal and uniform treatment.The Netherlands Journal of Medicine,2011,69:500-507.

[18] CORNELIS T,OREOPOULOS DG.Update on potential medical treatments for encapsulating peritoneal sclerosis; human and experimental data.International Urology and Nephrology,2011,43:147-156.

[19] SOLAK A,SOLAK I.Abdominal cocoon syndrome:preoperative diagnostic criteria,good clinical outcome with medical treatment and review of the literature.Turk J Gastroenterol,2012,23(6):776-779.

[20] MESHIKHES AW,BOJAL,S.A rare cause of small bowel obstruction:Abdominal cocoon.International Journal of Surgery Case Reports,2012,3:272-274,2012.

专家点评

患者为中年男性,以反复呕吐入院。呕吐的病因复杂多样,涉及多个系统,迅速确定病因对于正确施治十分重要。

腹茧症又称硬化性腹膜炎、局限性小肠外薄膜包绕症、先天性小肠禁锢症、包膜内粘连性肠梗阻和小肠节段性纤维包裹症等。我们将发病原因不明、无腹部手术及外伤史者称为原发性腹茧症,可能因先天性发育畸形所致。另一类相对有较明确的病因,为继发性腹茧症。

腹茧症术前诊断困难,一般腹痛呕吐反复发作,可见腹部包块,无腹膜炎、腹部手术及外伤史,肠梗阻原因不明者应考虑到本病。影像学检查除提示肠梗阻或腹部包块外,也有很大的病因诊断价值。X线检查特点为阶梯状液平。B超可见腹部包块处肠管扩张明显、有蠕动,不能探及实性或囊性肿物,外被一层厚的弱回声组织包绕。CT特点为扩张小肠肠襻固定在某一部位,被增厚的腹膜所包裹或分隔,肠系膜挛缩。发现包膜症可直接诊断为腹茧症。电子结肠镜和钡剂灌肠可除外结肠病变。术中见到全部或部分小肠为一层特异的灰白、质韧、厚硬的纤维膜包裹,形似蚕茧,其他邻近脏器如肝、胃、部分结肠亦为此膜覆盖,大网膜缺如时,可以诊断为腹茧症。病理显示该膜为纤维组织,伴或不伴局灶性炎症反应,未见恶性细胞或其他病原体,腹水细菌培养阴性。确诊的腹茧症患者,可不予处理或采用非手术治疗;若引起消化道梗阻症状或出现腹部包块者,应给予手术治疗。

点评专家:刘思德

本病患者反复发作腹痛、呕吐、停止排气排便,不全性肠梗阻起病,各种生化指标、多次腹部影像学资料、内镜检查、剖腹探查手术,排除了机械性梗阻、缺血性肠病、假性肠梗阻等常见病因。

不明原因腹痛还需考虑代谢性疾病可能性。本病例主诊医生病史资料询问详细,体格检查认真仔细,临床思维缜密,抽丝剥茧的推理对疾病诊断非常重要。结合患者有多血质面容,光过敏性皮肤损害,夏重冬轻;存在自主神经和周围神经症状;发作性腹痛,但腹部体检体征轻微;尿酸化煮沸实验阳性,高度提示血卟啉病可能,如疾病发作时实验室检查提示血、尿卟啉前体如δ-氨基-γ-酮戊酸(δ-amino levulinic acid,ALA)、胆色素原(porphobilinogen,PBG)明显升高,则更支持诊断。对存在遗传性基因突变的患者,基因检测有助于疾病的确诊和亲属筛查。

治疗以避光防晒、消除诱因(酗酒、诱导ALA-合成酶的药物)、营养支持、对症处理为主。

点评专家:沈锡中