Details

Autor(en) / Beteiligte

• Yoon, Jiyoung
• Jung, Kee Wook
• Ham, Nam Seok
• Kim, Jihun
• Do, Yoon Suh
• Kim, Seon Ok
• Choi, Sang Hyun
• Kim, Dong Wook
• Hwang, Sung Wook
• Park, Sang Hyoung
• Yang, Dong‐Hoon
• Ye, Byong Duk
• Byeon, Jeong‐Sik
• Yoon, Yong Sik
• Kim, Chan Wook
• Yu, Chang Sik
• Jung, Hwoon‐Yong
• Yang, Suk‐Kyun
• Martin, Joanne E.
• Knowles, Charles H.
• Myung, Seung‐Jae

Titel

Adult‐onset megacolon with focal hypoganglionosis: A detailed phenotyping and prospective cohort study

Ist Teil von

• Neurogastroenterology and motility, 2023-09, Vol.35 (9), p.e14630-n/a

Ort / Verlag

England: Wiley Subscription Services, Inc

Erscheinungsjahr

2023

Quelle

Wiley Online Library

Beschreibungen/Notizen

• Background In this prospective cohort study, we evaluated features of “adult‐onset megacolon with focal hypoganglionosis.” Methods We assessed the radiologic, endoscopic, and histopathologic phenotyping and treatment outcomes of 29 patients between 2017 and 2020. Data from community controls, consisting of 19,948 adults undergoing health screenings, were analyzed to identify risk factors. Experts reviewed clinical features and pathological specimens according to the London Classification for gastrointestinal neuromuscular pathology. Key results The median age of the patients with adult‐onset megacolon with focal hypoganglionosis at symptom onset was 59 years (range, 32.0–74.9 years), with mean symptom onset only 1 year before diagnosis. All patients had focal stenotic regions with proximal bowel dilatation (mean diameter, 78.8 mm; 95% confidence interval [CI], 72–86). The comparison with community controls showed no obvious risk factors. Ten patients underwent surgery, and all exhibited significant hypoganglionosis: 5.4 myenteric ganglion cells/cm (interquartile range [IQR], 3.7–16.4) in the stenotic regions compared to 278 cells/cm (IQR, 190–338) in the proximal and 95 cells/cm (IQR, 45–213) in the distal colon. Hypoganglionosis was associated with CD3+ T cells along the myenteric plexus. Colectomy was associated with significant symptom improvement compared to medical treatment [change in the Global Bowel Satisfaction score, −5.4 points (surgery) vs. ‐0.3 points (medical treatment); p < 0.001]. Conclusions and inferences Adult‐onset megacolon with focal hypoganglionosis has distinct features characterized by hypoganglionosis due to inflammation. Bowel resection appears to benefit these patients. In patients with segmental megacolon with a focally narrowed colonic segment connecting the proximally dilated and distally non‐dilated segments, the focally narrowed segment showed hypoganglionosis. Patients who underwent colectomy showed significant improvements in global bowel satisfaction (GBS) score and bowel frequency per week. Bowel resection appears to benefit these patients.