Kaul Ajay, Kaul Kanwar K
Division of Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Department of Pediatrics, NSCB Medical College, Jabalpur, India.
Pediatr Gastroenterol Hepatol Nutr. 2015 Dec;18(4):224-9. doi: 10.5223/pghn.2015.18.4.224. Epub 2015 Dec 23.
Cyclic vomiting syndrome (CVS) is a functional disorder characterized by stereotypical episodes of intense vomiting separated by weeks to months. Although it can occur at any age, the most common age at presentation is 3-7 years. There is no gender predominance. The precise pathophysiology of CVS is not known but a strong association with migraine headaches, in the patient as well as the mother indicates that it may represent a mitochondriopathy. Studies have also suggested the role of an underlying autonomic neuropathy involving the sympathetic nervous system in its pathogenesis. CVS has known triggers in many individuals and avoiding these triggers can help prevent the onset of the episodes. It typically presents in four phases: a prodrome, vomiting phase, recovery phase and an asymptomatic phase until the next episode. Complications such as dehydration and hematemesis from Mallory Wise tear of the esophageal mucosa may occur in more severe cases. Blood and urine tests and abdominal imaging may be indicated depending upon the severity of symptoms. Brain magnetic resonance imaging and upper gastrointestinal endoscopy may also be indicated in certain circumstances. Management of an episode after it has started ('abortive treatment') includes keeping the patient in a dark and quiet room, intravenous hydration, ondansetron, sumatriptan, clonidine, and benzodiazepines. Prophylactic treatment includes cyproheptadine, propranolol and amitriptyline. No mortality has been reported as a direct result of CVS and many children outgrow it over time. A subset may develop other functional disorders like irritable bowel syndrome and migraine headaches.
周期性呕吐综合征(CVS)是一种功能性疾病,其特征为剧烈呕吐的刻板发作,发作间隔为数周或数月。虽然它可发生于任何年龄,但最常见的发病年龄为3至7岁。无性别优势。CVS的确切病理生理学尚不清楚,但患者及其母亲与偏头痛头痛有很强的关联,这表明它可能代表一种线粒体病。研究还提示,潜在的涉及交感神经系统的自主神经病变在其发病机制中起作用。许多个体的CVS有已知的诱发因素,避免这些诱发因素有助于预防发作。它通常呈现四个阶段:前驱期、呕吐期、恢复期和无症状期直至下一次发作。在更严重的病例中,可能会出现诸如脱水和食管黏膜Mallory - Wise撕裂导致的呕血等并发症。根据症状的严重程度,可能需要进行血液和尿液检查以及腹部影像学检查。在某些情况下,也可能需要进行脑磁共振成像和上消化道内镜检查。发作开始后的治疗(“顿挫性治疗”)包括让患者待在黑暗安静的房间、静脉补液、使用昂丹司琼、舒马曲坦、可乐定和苯二氮䓬类药物。预防性治疗包括使用赛庚啶、普萘洛尔和阿米替林。尚无CVS直接导致死亡的报道,许多儿童随着时间推移会自愈。一部分患者可能会发展为其他功能性疾病,如肠易激综合征和偏头痛头痛。