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

doi:10.4240/wjgs.v14.i7.656

Adult patients with allied disorders of Hirschsprung's disease in emergency department: An 11-year retrospective study

World J Gastrointest Surg. 2022 Jul 27;14(7):656-669. doi: 10.4240/wjgs.v14.i7.656.

Authors

Shuai Jiang^{1

2}, Cong-Ying Song^{1

2}, Meng-Xiao Feng^{1

2}, Yuan-Qiang Lu^{1

3}

Affiliations

¹ Department of Emergency Medicine, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang Province, China.
² Key Laboratory for Diagnosis and Treatment of Aging and Physic-chemical Injury Diseases of Zhejiang Province, Hangzhou 310003, Zhejiang Province, China.
³ Key Laboratory for Diagnosis and Treatment of Aging and Physic-chemical Injury Diseases of Zhejiang Province, Hangzhou 310003, Zhejiang Province, China. luyuanqiang@zju.edu.cn.

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; Clinical characteristics; Emergency department; Misdiagnosis and mistreatment; Timely diagnosis.