Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Jul 27, 2022; 14(7): 656-669
Published online Jul 27, 2022. doi: 10.4240/wjgs.v14.i7.656
Adult patients with allied disorders of Hirschsprung’s disease in emergency department: An 11-year retrospective study
Shuai Jiang, Cong-Ying Song, Meng-Xiao Feng, Yuan-Qiang Lu
Shuai Jiang, Cong-Ying Song, Meng-Xiao Feng, Yuan-Qiang Lu, Department of Emergency Medicine, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang Province, China
Shuai Jiang, Cong-Ying Song, Meng-Xiao Feng, Yuan-Qiang Lu, Key Laboratory for Diagnosis and Treatment of Aging and Physic-chemical Injury Diseases of Zhejiang Province, Hangzhou 310003, Zhejiang Province, China
Author contributions: Jiang S and Lu YQ conceived and designed the study; Jiang S and Feng MX collected the clinical data; Jiang S and Song CY provided statistical advice on study design and analyzed the data; Jiang S drafted the manuscript; and all authors contributed substantially to manuscript revision; Lu YQ takes responsibility for the paper as a whole.
Supported by the Foundation of Key Discipline Construction of Zhejiang Province for Traditional Chinese Medicine, No. 2017-XK-A36.
Institutional review board statement: This study was approved by the Ethics Committee of the First Affiliated Hospital, Zhejiang University School of Medicine (No. 2021271).
Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous clinical data that were obtained after each patient agreed to treatment by written consent.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yuan-Qiang Lu, MD, PhD, Professor, Department of Emergency Medicine, The First Affiliated Hospital, School of Medicine, Zhejiang University, No. 79 Qingchun Road, Hangzhou 310003, Zhejiang Province, China. luyuanqiang@zju.edu.cn
Received: August 17, 2021
Peer-review started: August 17, 2021
First decision: September 12, 2021
Revised: September 21, 2021
Accepted: June 24, 2022
Article in press: June 24, 2022
Published online: July 27, 2022
Abstract
BACKGROUND

In the past years, only a few studies with a limited number of adult patients analyzed clinical features of allied disorders of Hirschsprung’s disease (ADHD), most of which were individual case reports or lacked detailed clinical information. Although many studies have reported patients presenting to the emergency department (ED) with recurrent abdominal symptoms for a number of disorders, there are few data involving ADHD. However, owing to a lack of awareness of the disease, misdiagnoses and mistreatments are common. Severe complications such as perforation, bleeding, malabsorption, and even death in ADHD had been reported by many studies.

AIM

To assist ED clinicians in having a more comprehensive understanding of this disease and making an early suspected diagnosis of ADHD more effectively.

METHODS

We enrolled 53 patients who visited the ED and were eventually diagnosed with ADHD over the past 11 years in our hospital. Their basic information, clinical manifestations, and imaging findings were analyzed. Blood indices were compared between the ADHD and irritable bowel syndrome (IBS) groups.

RESULTS

Adult patients with ADHD had a mean age of 48.8 ± 14.3 years, and 77.4% had been treated before admission. The transverse colon was the most common dilated part (73.6%), and constipation (67.9%) was the most common symptom. ADHD patients can present with uncommon symptoms and false-negative imaging findings. Logistic regression analysis indicated that body mass index (BMI) [odds ratio (OR) = 0.786, P = 0.013], cholinesterase (per 1000 units; OR = 0.693, P = 0.008), and blood chlorine (OR = 0.816, P = 0.022) were determined to be independent related factors between the ADHD and IBS groups. The area under the receiver operating characteristics curve of these three indices combined was 0.812 (P < 0.001).

CONCLUSION

Emergency physicians should be vigilant regarding patients with chronic constipation, abdominal pain, or abdominal distension, and consider the possibility of ADHD despite its rarity. Abdominal computed tomography examination is recommended as a useful tool in the suspected diagnosis of ADHD. BMI, cholinesterase, and blood chlorine have good discriminative abilities between ADHD and IBS. The nutritional status of adult patients with ADHD is worthy of further attention. Surgical treatment for adult patients with ADHD is important and inevitable.

Keywords: Allied disorders of Hirschsprung’s disease, Emergency department, Clinical characteristics, Misdiagnosis and mistreatment, Timely diagnosis

Core Tip: Emergency physicians should be vigilant regarding patients with chronic constipation, abdominal pain, or abdominal distension, especially those with recurrent and intolerable symptoms. Allied disorders of Hirschsprung’s disease (ADHD) should be considered in such cases despite its rarity. Abdominal computed tomography examination is recommended as a useful tool to make a suspected diagnosis of ADHD. Clinicians should also be wary of uncommon symptoms and false-negative imaging findings. Body mass index, cholinesterase, and blood chlorine have good discriminative abilities between ADHD and irritable bowel syndrome. The nutritional status of adult patients with ADHD is worthy of further attention.