Endoscopic fenestration treatment for pneumatosis cystoides intestinalis in patient with recurrent colonic intussusception

Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by intestinal mural pneumatized cysts that appear most commonly in the colon [1] [2]. Although primarily asymptomatic, PCI occasionally causes intussusception that usually requires surgical treatment [3] [4]. Hyperbaric or high-concentration oxygen therapy is reportedly effective, though complete remission is uncertain [5]. We present the first report of an endoscopic fenestration procedure used for successful treatment of PCI with recurrent intussusception.

A 16-year-old male patient experienced repeated severe abdominal pain over a 2-year period. Computed tomography revealed intussusception in the ascending colon along with mural gas-filled cysts ([Fig. 1]), while colonoscopy showed multiple submucosal tumor-like lesions ([Fig. 2 a, b]). The diagnosis was idiopathic PCI accompanied by intussusception. Oxygen therapy was ineffective, thus endoscopic treatment was performed. The therapeutic protocol was approved by the medical ethics committee of our institution and registered with the Center for Clinical Trials, Japan Medical Association (JMA-IIA00240), with written informed consent obtained from the patient.

Fig. 1 Contrast-enhanced computed tomography examination findings obtained when patient noted abdominal pain. Intussusception in the ascending colon (yellow arrowheads) and sequential obstructive ileus of the small intestine (red arrowheads) were observed.

Fig. 2 Endoscopic images obtained before and after endoscopic treatment. a, b Multiple tense gas-filed cysts with a bumpy surface were observed in the ascending colon (a) and hepatic flexure (b). A large multinodular cyst in the ascending colon (yellow arrowheads) was considered to be the lesion causing intussusception. c A mucosal incision was performed in multiple directions for cyst fenestration. d During the procedure, examination of the cyst interior revealed a multilocular structure with a thick fibrous partition. e, f Follow-up colonoscopy findings obtained 3 months after treatment showed remarkable reductions in multiple cysts in the ascending colon (e) and hepatic flexure (f).

Needle aspiration was ineffective for the large cysts and endoscopic fenestration was subsequently performed ([Video 1]). First, a mucosal incision was made with a 3-mm cutting-wire needle-knife (KD-10Q; Olympus Co., Tokyo, Japan), followed by fenestration of multilocular cysts with a thick fibrous partition ([Fig. 2 c, d]), as well as multidirectional fenestration on the inner side of an imaginary line of the proper muscular layer. Mucosal incision and fenestration procedures were done using a high-frequency generator (VIO300; ERBE Elektromedizin, Tübingen, Germany) in Endocut mode (60 W, effect 2). Minor bleeding was the only procedure-related complication.

Video 1 Endoscopic fenestration using a needle-knife for refractory, symptomatic pneumatosis cystoides intestinalis.

Follow-up colonoscopy showed remarkable reduction of PCI ([Fig. 2 e, f]), while X-ray imaging revealed a long-term therapeutic effect ([Fig. 3]). After 6 years, no symptoms have developed.

Fig. 3 X-ray images obtained before and after endoscopic treatment. Mural emphysema decreased the day after treatment and had nearly completely disappeared 3 months later. A follow-up examination performed 19 months later showed that the disease was well controlled without recurrence.

Endoscopic fenestration using a needle-knife for refractory symptomatic PCI may be a good therapeutic alternative to surgery.

Endoscopy_UCTN_Code_TTT_1AQ_2AF

Publication History

Article published online:
24 February 2023

• References

• 1 DuBose JJ, Lissauer M, Maung AA. et al. Pneumatosis Intestinalis Predictive Evaluation Study (PIPES): a multicenter epidemiologic study of the Eastern Association for the Surgery of Trauma. J Trauma Acute Care Surg 2013; 75: 15-23
• 2 Ohmiya N, Hirata I, Sakamoto H. et al. Multicenter epidemiological survey of pneumatosis intestinalis in Japan. BMC Gastroenterol 2022; 22: 272
• 3 Wu LL, Yang YS, Dou Y. et al. A systematic analysis of pneumatosis cystoids intestinalis. World J Gastroenterol 2013; 19: 4973-4978
• 4 Wayne E, Ough M, Wu A. et al. Management algorithm for pneumatosis intestinalis and portal venous gas: treatment and outcome of 88 consecutive cases. J Gastrointest Surg 2010; 14: 437-448
• 5 Alpuim CostaD, Modas DanielP, Vieira BrancoJ. The role of hyperbaric oxygen therapy in pneumatosis cystoides intestinalis – a scoping review. Front Med (Lausanne) 2021; 8: 601872