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Is Anorectal Myectomy Useful for Hirschsprung's Disease and Its

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Is Anorectal Myectomy Useful for Hirschsprung's Disease and Its 日大医誌 76 (4-5): 169–173 (2017) 169 Review Is Anorectal Myectomy useful for Hirschsprung’s Disease and its Allied Disorders? Ryouichi TOMITA Department of Surgery, Nippon Dental University School of Life Dentistry The author reviewed the clinical features of children with Hirschsprung’s disease (HD) and its allied disorders [hypoganglionosis (Hypo) and intestinal neuronal dysplasia (IND)] before and after anorectal myectomy (ARM). The incidence was greater in males than in females (1.5:1). The patients were between 0 and 15 years of age and more than half were less than 6 years old. Neurohistochemical examination showed Hypo [mild type (3/5 or more ganglion cells per ganglia compared with normal ganglia), severe type (less than 2/5 ganglion cells per ganglia compared with normal ganglia), IND, and HD (aganglionosis). Mild Hypo was the most common, compared with HD and NID. Abdominal pain was the most common symptom, followed by soiling and anal pain. Spontaneous evacuation without the need for laxatives was noted in 70~75% patients with a good outcome, and 30~40% of those with a satisfactory outcome after ARM. Patients with a poor outcome still required laxatives and other med- ical treatment. Related causes of constipation, i.e., colonic inertia, psychological or central nervous disorders, oc- curred at a higher incidence in those with a poor outcome. The most common related cause of constipation after ARM was colonic inertia, i.e., redundant colon. Additional operations (subtotal colectomy and ileorectal anasto- mosis) were performed for the patients with a poor outcome. These patients were satisfied with the postoperative state of evacuation. In conclusion, the abnormal distribution of ganglia, i.e. Hypo, IND, and HD could be largely related to the defecation disorders observed in childhood patients with chronic constipation. Patients with severe Hypo, IND, and HD, especially those with long affected colon, psychological or central nervous disorders, may experience significant persistent constipation after ARM. Additional operations should be performed in patients with a poor outcome due to colonic inertia. Key words: Anorectal myectomy, Hirschsprung’s disease, Hypoganglionosis, Intestinal neuronal dysplasia (J. Nihon Univ. Med. Ass., 2017; 76 (4-5): 169–173) anisumus, puborectalis syndrome) in the anorectal region. Introduction On the other hand, transit study is very useful to detect Chronic constipation is a common complaint in out- slow transit type of chronic constipation type (colonic patients clinic. Patients have stools too difficult to expel inertia)9). and/or a feeling of incomplete evacuation after defecation. Anorectal myectomy (ARM) is appropriate for However, these symptoms are difficulty to quantify1, 2). treatment in patients with outlet obstruction including In general, stool frequency reported that patients with ultrashort or short-type of Hirschsprung’s disease (HD; chronic constipation often have stool frequencies of less aganglionosis), the allied disorders of HD such as hypo- than once per week despite the use of fiber and/or laxa- ganglionosis (Hypo) and intestinal neuronal dysplasia tives3). Stivland et al.4) also showed that chronic nonspe- (IND), and patients with high internal anal sphincter cific constipation (i.e., Chronic constipation) is defined pressure10–16) and encouraging results have been reported as a frequency of bowel motions less than three times per in children with short-type of HD and its allied disor- week and a resulting failure to respond to medical man- ders17–20). Short or ultrashort type of HD and its allied dis- agement for more than 6 months. In internal medicine, orders with colonic inertia, who have a very slow cecum chronic constipation is defined by Rome III. On the other to rectum transit, may still be constipated after ARM12, 21). hand, in surgeon for surgical procedure, chronic consti- ARM has been used for the histological diagnosis of pation has been divided into groups with colonic inertia patients in consecutive series of chronic constipation by and with outlet obstruction by transit study, defecography, most pediatric surgeons. In pediatric clinic, to detect HD anorectal manometry, and electromyography1, 2, 5–9). and its allied disorders (Table 1), first, anorectal manom- Defecography is useful for the diagnosis and evaluation etry which is useful to detect those diseases, to obtain a of the morphological and functional abnormalities (es- histological diagnosis in patients with chronic constipa- pecially outlet obstruction type of chronic constipation; tion, and second ARM is performed in them (Table 2). Received: February 7, 2017 Accepted: February 17, 2017 170 Ryouichi TOMITA 日大医誌 Table 1 Classification of Hirschsprung’s disease and its allied age. About one quarter of Hypo patients also have onset disorders of constipation at less than 1 month and there are no 1. Aganglia sexual differences8). Hirschsprung’s disease 2. Abnormal ganglia Clinical characteristics before and after ARM Immaturity of ganglia (Immature ganglionosis) in patients with ultrashort or short type of HD Hypoganglionosis (Oligoganglionosis) and its allied disorders Congenital hypoganglionosis (Hypogenesis, Hypoplasia) Acquired hypoganglionosis Patients with ultrashort or short type of HD and its Intestinal neuronal dysplasia allied disorders often have bowel movements less than 3. Normal ganglia 1 to 3 times per week despite the use of medical therapy Megacystitis microcolon intestinal hypoperistasis syndrome Segmental dilatation of intestine and/or dietary manipulation, and symptoms (soiling, ab- Intestinal anal sphincter achalasia dominal pain, anal pain, and so on) dominate the patient’s Chronic idiopathic intestinal pseudo-obstruction life style1–4). Almost patients before ARM complained of infrequent bowel habit less than 3 times per week, and difficulty in defecation despite the use of medical therapy Third, to clarify whether patients with HD and its allied such as suppositories, enemas, or anal dilatation. The disorders after ARM who do not show improvement have most common clinical feature is abdominal pain (Table colonic inertia, the colonic transit time by radio-opaque 3). Among the histological types, Hypo, especially severe marker method has been performed. In this review, we Hypo cases, have abdominal pain more than the other analyzed the clinical results of ARM performed in a con- cases. Garrett et al.10) concluded that the severity of the secutive series of chronic constipated patients. symptoms of constipation in any individual are more related to the extent of the abnormal innervation of the Operative procedure of the ARM bowel than to the actual length of bowel that is affected. ARM is performed under general anesthesia with Munakata et al.22) reported that most cases of both severe the patient in the lithotomy position. According to the Hypo and IND are not ultra-short type but short type patient size, the internal anal sphincter and rectal smooth from the rectum to the recto-sigmoid colon, and both muscle were dissected 0.5 cm in width and 3 to 5 cm in severe Hypo and IND are more frequently associated length8, 13–16). with severe symptoms of bowel dysfunction than the other histological types. In general, ARM is not useful Onset of constipation in patient with HD and its allied for patients with long segment type of HD, severe Hypo disorders or IND13–16, 21). In our results of ARM in patients at 2.6 In general, chronic constipation is often found among years follow-up, about three quarters of the patients (short children under the age of 5 years, occasionally at birth2). type of H disease, Hypo, and IND) had achieved good Little boys are more often constipated than little girls, improvement8). Spontaneous evacuation without the need but a reversal of ratio occurs in late life2). Almost patients for laxatives was recorded in about three quarters of those with HD, Hypo, and IND have abnormal defecation from patients. ARM for residual HD after radical operation was the early days of life10, 11, 17–20, 22, 23). Patients with HD or improved following operation. Disappointing results (poor IND have onset of constipation at less than 1 month of outcomes: no improvement after ARM) after ARM were Table 2 Histological classification o f chronic constipation Male Female Total Hypoganglionosis 3 8 (35.5) 3 3 (30.8) 7 1 (66.4) * * * * * * * 3 0 (28.0) * 2 1 (19.6) 5 1 (47.7) Mild * * * * * * * 8 ( 7.5) * 1 2 (11.2) 2 0 (18.7) Severe * * * * Normal ganglia 1 6 (15.0) * 1 3 (12.1) 2 9 (27.1) * * Intestinal 2 ( 1.9) 2 ( 1.9) neuronal dysplasia Aganglionosis 2 ( 1.9) 3 ( 2.8) 5 ( 4.7) Total 5 8 (54.2) 4 9 (45.8) 1 07 (100) * p < 0.05 , * * p < 0 .001, * * * p < 0.000 1, p < 0.0 0001 ( ) % 76 (4-5) 2017 Anorectal myectomy for Hirschsprung’s disease and its allied disorders 171 Table 3 Clinical features before ARM Soiling Abdominal pain Anal pain Yes No Yes No Yes No Hypoganglionosis 31 40 51 20 32 39 Mild 20 31 33 18 19 32 * Severe 11 9 18 2 13 7 Normal ganglia 13 16 20 9 7 22 Intestinal 1 1 2 0 1 1 neuronal dysplasia Aganglionosis 4 1 4 1 0 5 Total 49 (45.8) 58 (54.2) 77 (72.0) 30 (28.0) 40 (37.4) 67 (62.6) ( )% * * * * * * p < 0.05, * * p < 0.0001, * * * p < 0.00001 Table 4 Outocomes after ano-rectal myectomy (the median in patients with colonic inertia and severe constipation follow-up time after anorectal myectomy: 1.6 ± 1.3 years) is induced by a psychologically functional disorder27). Good Satisfactory Poor In many constipation cases, the cause is a poor diet and lack of parental control28). Poor eating habit has been Hypoganglionosis 51 8 12 found in about 20% of poor outcomes (no improvement after ARM)28). A large meal is the most powerful normal Mild 44 * 4 3 * * stimulus to colonic activity, the so-called colonic motor Severe 7 4 9 response to eating29).
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