Functional Outcome in Children with Hirschsprung’s Disease after Modified Duhamel’s Procedure: A Longitudinal Study
Correspondence Address :
Dr. B Ujjwal Singh Trivedi,
Kaleelil, Mulluvila, Vadakkevila P. O., Kollam-691010, Kerala, India.
E-mail: ujjwalbhadran1965@gmail.com
Introduction: The classical surgical treatment of Hirschsprung’s Disease (HD) is performed by resection of the aganglionic segment of the distal intestine and bringing down the ganglionic segment by Modified Duhamel’s procedure or other pull-through operations. The assessment of the postoperative functional outcome and continence scoring systems are important considerations during follow-up of these children. In the present study, a scoring system was used to analyse the postoperative functional outcome of HD as there are very few studies, that have objectively assessed the same.
Aim: To assess the functional outcome in children under six years of age, who underwent Modified Duhamel’s procedure for HD.
Materials and Methods: This longitudinal study was conducted in the Tertiary Teaching Institution at Government Medical College, Thiruvananthapuram, Kerala, India. The duration of the study was one year and nine months, from February 2017 to November 2018. Forty cases of biopsy proven HD, who underwent Modified Duhamel’s procedure were analysed. Postoperative follow-up was done at one, two, and three years after the definitive surgical procedure. Patients were classified as good, fair and poor outcome groups based on the Taylor-Duthies-Zachary continence scoring system. Statistical analysis was performed using Statistical Package for Social Sciences (SPSS) software version 22.0. Pretest comparison of scores and p-value were analysed by Wilcoxon signed-rank test. A p-value <0.05 was considered as statistically significant.
Results: The mean age of the study participants was 12.7±4.1 months and age ranges from three to 25 months. Median stool frequency per day after one, two, and three years of surgery, was 6.0, 4.0, and 3.0, respectively. The observed difference in median frequency between one and two year, one and three, and two year and three year was statistically significant (p<0.05). Median continence score to assess the anal sphincter control at one, two and three years after surgery were 2.3, 4.0 and 4.5, respectively. The observed difference in scores over successive years, was statistically significant (p<0.05).
Conclusion: The functional outcome in patients, who underwent Duhamel’s procedure was good and the postoperative issues like increased stool frequency, and sphincter control improved over successive years.
Aganglionosis, Colonic, Congenital, Megacolon
The HD is a developmental disorder of the intrinsic component of the enteric nervous system, that is characterised by the absence of ganglion cells in the myenteric and submucosal plexus of the distal intestine presenting with functional intestinal obstruction at the level of aganglionosis (1),(2). The incidence is approximately one in 5000 live births (3),(4). Histologic evaluation of rectal biopsy remains the gold standard diagnostic test. The classical treatment is surgical resection of the aganglionic segment of the distal intestine and bringing down the ganglionic segment to the anus by Duhamel’s (modified) procedure or other pull-through operations (2),(3). The other surgical procedures for HD, include the Soave endorectal pull-through and Swenson’s rectosigmoidectomy. Primary transanal endorectal pull-through and laparoscopic abdominal mobilisation for HD have gained popularity in the last few decades. The postoperative issues associated with endorectal pull-through include cuff abscess, cuff roll-back, and difficulty to adapt long segment HD (3),(4),(5).
The main complication associated with Swenson’s procedure is the neurogenic bladder, due to extensive perirectal dissection. The postoperative problems in children seen after Modified Duhamel’s operation include obstructive symptoms, soiling, and enterocolitis. The assessment of postoperative functional outcome after Duhamel’s procedure and continence scoring systems are important considerations during follow-up of these children. Many existing studies on the postoperative outcome of HD have not assessed the outcome based on objective criteria (5). In the absence of a specific scoring system to assess the postoperative outcome in HD, objective evaluation of the results become difficult. The aim of the study was to assess the functional outcome in children under six years of age, who underwent Modified Duhamel’s procedure for HD at a Tertiary Teaching Institution.
The longitudinal study was conducted in the Tertiary Teaching Institution at Government Medical College, Thiruvananthapuram, Kerala, India. The duration of the study was one year and nine months, from February 2017 to November 2018. The study included cases, which had completed one, two and three years of postoperative follow-up. Prior clearance was obtained from the Institutional Ethics Committee (IEC) letter number (IECNo.0/3/29/2017/MCT).
Inclusion criteria: Children under six years of age with biopsy-proven HD, who underwent Modified Duhamel’s procedure. Patients with short-segment disease, long-segment disease, and total colonic aganglionosis (based on barium enema findings, intraoperative findings, and histopathology report) were included in the study.
Exclusion criteria: Children, who underwent procedures other than Duhamel’s operation, children with chromosomal anomalies like Down’s syndrome/metabolic disorders/neurogenic impairment, hypothyroidism, and children with syndromic disorders were excluded from the study.
Study Procedure
A total of 40 cases of biopsy-proven HD who underwent Modified Duhamel’s procedure were analysed for the functional outcome (4). Postoperative follow-up were done at one, two, and three years of definitive surgical procedure. The parameters studied in each patient included basic demographic data and the analysis for daily frequency of stools, constipation and incontinence (as an index of the sphincter control). The data was collected from the outpatient and inpatient records, operative notes, postoperative records, follow-up charts and the questionnaire administered to parents during regular follow-up period. In the present study, the Taylor-Duthie-Zachary continence scoring system, to assess sphincter control in postoperative anorectal malformation has been used to objectively assess the functional status in postoperative HD during successive years (6). Patients were classified to have good, fair, and poor outcome depending upon the score. The highest total score was 5. The scoring assigned was: Good (3-5), Fair (1.5-3.5), and Poor (0-1.5). A digital rectal examination was done at the end of the examination period to assess the anal sphincter tone, rule out rectal spur and constipation with the loading of rectum.
Statistical Analysis
Statistical analysis was performed using SPSS software version 22.0. Patient’s continence scores were analysed and categorised into good/fair/poor outcome groups. Continuous variables were expressed as mean Standard Deviation (SD) and interquartile ranges. Categorical variables were expressed as frequency and percentage. Pretest comparison of scores and p-value were analysed by Wilcoxon signed-rank test. A p-value <0.05 was considered as statistically significant.
The average age of the study population was 12.7±4.1 months and age ranges from three to 25 months. A total of 18 out of the 40 patients were of age 9-12 months (Table/Fig 1). Out of the total 40 patients, 28 (70%) patients were male and 12 (30%) were females (Table/Fig 2). Average weight of the patients was 8.6±1.3 kg ranges from 5-12 kg. About 28 (70%) patients were in the 7-9 kg weight group (Table/Fig 3).
Two out of 40 (5%) patients have constipation after one year of surgery. Among the total of 40 patients studied, during successive years, only the cases with regular follow-up, complete follow-up data and filled questionnaires were included for analysis (Table/Fig 4). The integrity of the anal sphincter and its postoperative state was assessed by digital rectal examination performed during follow-up. The functional status of the anal sphincter after surgery is an important determinant of the outcome. Out of 40 patients, 5 (12.5%) were noted to have lax anal sphincter and 35 (87.5%) were noted to have normal anal sphincter at the final per rectal examination. Two patients were found to have postoperative ‘spur’. One patient underwent crushing of the spur and the other improved with conservative management. Median stool frequency per day after one, two and three years of follow-up was 6.0, 4.0 and 3.0, respectively. The observed difference in median frequency between after first year and second year, first year and third year and second year and third year was statistically significant (p<0.05). The incidence of postoperative soiling was found to be similar with an incidence of 5 (12.5%) patients. The stool frequency per day was significantly reduced in successive years (Table/Fig 5),(Table/Fig 6).
Median Taylor- Duthies- Zachary continence scores during postoperative follow-up at one year, two years, and three years were 2.3, 4.0 and 4.5, respectively. The observed difference in scores in successive years was statistically significant (p<0.05). There was a significant improvement in score after successive years (Table/Fig 7),(Table/Fig 8).
The outcome in 7 (17.5%) patients was poor according to continence score after one year. But during follow-up, only 1 (2.8%) patient was seen to have a poor outcome after two years and no case was observed to have poor outcome after three years (Table/Fig 9).
Duhamel’s procedure involves bringing the normal colon down through the bloodless plane between the rectum and the sacrum and joining the two walls to create a new lumen, which is aganglionic anteriorly and normally innervated posteriorly. Kocher clamp was used to join the walls initially and later Irani clamps were used to join the walls and left there till it falls off naturally, usually within a week. Presently, surgical staplers are almost always used instead (1),(2). The incidence of postoperative constipation was 5% in the first year, 5.6% in the second year and 3.3% after three years of postoperative period. Two patients were found to have postoperative ‘spur’. One patient underwent crushing of the spur and the other improved with conservative management. Various previous studies shows that, the incidence of constipation is less in Duhamel’s procedure. The incidence of constipation in various studies ranged from 4% to 13.4% (7),(8),(9). The findings in the present study was associated with the previous studies. There was a steep decline in the incidence of frequency of stools, as the age advances. Six patients developed acute diarrhoeal disease and one patient developed enterocolitis, which were managed conservatively. Median stool frequency per day after one year, two year, three year of surgery was six, four and three, respectively and this finding has significant bearing on outcome. The incidence of stool frequency in various studies ranged from three to seven per day. These findings are correlated with the previous studies (9),(10).
The continence score for the functional integrity of the anal sphincter was assessed. The score at one, two and three years after surgery was 2.3, 4 and 4.5, respectively. There is a significant improvement in score and it showed that, there was a good sphincter control over time. The findings of the present study also associated with previous studies (10),(11). In the present study, on digital rectal examination, 35 (87.5%) patients had a normal anal sphincter and 5 (12.5%) patients had lax anal sphincter. Functional improvement in the sphincter, occured over the time and associated with previous studies (1),(2),(12),(13),(14). The present study was compared with previous similar studies by Widyasari A et al., Aravind KL et al., and Shah S et al., as shown in (Table/Fig 10) on the functional outcome of HD after Duhamel’s procedure (12),(13),(14). The incidence of postoperative soiling was found to be similar with an incidence of 5/40 (12.5%) which is comparable. The incidence of constipation was found to be lesser than in most of the studies. The postoperative outcome was noted to be good/fair with normal continence in upto 38/40 (97.3%), which is higher than other studies.
Limitation(s)
The relatively short follow-up period and the use of continence scoring system adapted from anorectal malformations were limitations of the study. Ideally, long term prospective studies using continence scoring systems specific to HD, are required for the assessment of postoperative functional outcome.
The objective assessment of functional outcomes after surgery for HD using a continence scoring system gives valuable information regarding the status of bowel function. The majority of children were observed to have a good functional outcome with a low incidence of long-term postoperative complications. The continence score and bowel habits improved in successive years, as there was improvement in sphincter control.
DOI: 10.7860/JCDR/2023/62393.18248
Date of Submission: Dec 28, 2022
Date of Peer Review: Feb 28, 2023
Date of Acceptance: Apr 21, 2023
Date of Publishing: Jul 01, 2023
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was Ethics Committee Approval obtained for this study? Yes
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. NA
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Dec 28, 2022
• Manual Googling: Mar 14, 2023
• iThenticate Software: Apr 11, 2023 (4%)
ETYMOLOGY: Author Origin
EMENDATIONS: 6
- Emerging Sources Citation Index (Web of Science, thomsonreuters)
- Index Copernicus ICV 2017: 134.54
- Academic Search Complete Database
- Directory of Open Access Journals (DOAJ)
- Embase
- EBSCOhost
- Google Scholar
- HINARI Access to Research in Health Programme
- Indian Science Abstracts (ISA)
- Journal seek Database
- Popline (reproductive health literature)
- www.omnimedicalsearch.com