Hirschsprung s disease in adults: Clinical and therapeutic features

Transcription

1 International Research Journal of Surgery Vol. (), pp. 9-7, March, 5. ISSN: x IRJS Research Article Hirschsprung s disease in adults: Clinical and therapeutic features Haithem Zaafouri¹*, Skander Mrad¹, Manel Mabrouk², Noomen Haoues¹, Mariam Bel Hadj Salah³, Rabii Noomene¹, Ahmed Bouhafa¹, Anis Ben Maamer¹, Abderraouf Cherif¹ * Department of general surgery Habib Thameur hospital Tunis, Tunisia. Department of internal medicine Razi hospital Tunis, Tunisia. 3 Department of Pathological Anatomy and Cytology Habib Thameur hospital Tunis, Tunisia. Hirschsprung s disease (HD) is rare in adults and it is thus often undiagnosed or misdiagnosed. Through this series of patients we try to study the clinical characteristics of this pathology, to define its diagnostic clues and to assess the different therapeutic approaches. Definitive diagnosis is established on histology of specimens from the rectum and colon. The disease involved the rectum and the sigmoid colon in patients and was confined to the rectum, in the others. Treatment was in all cases surgical consisting of recto-colic resection associated with coloanal anastomosis and a protective right lateral ileostomy. We conclude that Hirschsprung s disease is rare in adults but by no means exceptional. It should be considered in young adults with a history of chronic constipation. Diagnosis is first of all clinical. When barium enema appearances are pathognomonic we needn t resort to histology to confirm the diagnosis. Anorectal manometry does not usually show RAIR. Current primary treatment of HD diagnosed in adults consists mainly of surgical resection. Key words: Hirschsprung, rectoanal inhibitory reflex, intestinal obstruction, coloanal anastomosis, Duhamel. INTRODUCTION Hirschsprung s disease (HD) is a rare pathology affecting about in 5 births. It is usually diagnosed in the newborn period; it is the most frequent cause of bowel obstruction in children. A diagnosis of adult HD is made when the patient is older than the age of years (Miyamoto M 5) at the time of diagnosis. This disease is often undiagnosed, but its real incidence is estimated at % (Grove K 9). More than 55 cases of adult HD have been reported in the English literature since 95 (Doodnath R, ). Surgical treatment of HD consists in resecting or excluding the aganglionic zone in the digestive tube and preserving normal anal sphincter function. Several procedures have been described in the literature. The choice of the procedure depends on the postoperative outcome and on the experience of the surgical team. Prognosis is usually good and the results are habitually satisfactory after surgery. Objective Management of adult HD poses a double problem: - Diagnosis is not easy given the non-specific presentation of the disease which may manifest in advanced years (up to 73 years of age in some reported cases). - Treatment remains surgical but should the same procedures as used for HD in infancy be employed? In other words, there s some question as to whether to use the same procedures as used for HD in infancy or to employ more adapted techniques. Corresponding author: Dr Haithem Zaafouri, Assistant professor, Department of general surgery Habib Thameur hospital Tunis, Tunisia. zaafouri.haithem@hotmail.fr, Tel:

2 Zaafouri et al. 9 Table. Clinical assessment of incontinence (Holschneider 983) Frequency of bowel movements Consistency of stools Soiling Anorectal sensitivity Ability to hold on Discrimination between stools and gas Need for treatment (enemas, medication) - normal(-/ daily) - often(3-5/daily) - very often - normal - soft - loose - absence - stress incontinence diarrhea - permanent - positive - incomplete - absent - minutes - seconds - impossible - normal - incomplete - absent - no - occasional - always Normal intestinal function Good result: good bowel control, minor discomfort in social life Fair result: regular discomfort in social life Poor result: in case of fecal incontinence or permanent soiling Intestinal function: Normal: 4 points Good (good bowel control, minor discomfort in social life): -3 points Fair(regular discomfort in social life): 5-9 points Poor(total rectal incontinence): -4 points The aim of this work is to assess the diagnostic and therapeutic approaches adopted in our department and to compare them to data from the literature. MATERIAL AND METHODS This is a descriptive retrospective study of hospital records of patients admitted to the department of general surgery of Habib Thameur Hospital in Tunis from January, to December 3, 3. The study which involved cases, focused on the clinical presentation, diagnostic procedures, treatment given and course of the disease. In order to assess the functional outcome, we collected all the relevant information obtained during follow-up visits or by telephone conversation. The findings were assessed according to Holschneider s score (Holschneider AM 996) (Table ). We noted the daily frequency of bowel movements, the consistency of stools, the possibility to discriminate between stools and gas, the possibility to hold on in case of call of nature and if there were any soilings while specifying the circumstances of their occurrence in case of stress or concomitant diarrhea. We also noted if the patients had taken any laxatives or on the contrary medications to slow down the bowel movements. During the follow-up visits we tried to assess the anorectal sensitivity and the sphincter tone. RESULTS The study involved men and women aged 3 on average (range -5 years) who had presented, in most cases, with an obstructive syndrome. In fact, 8 patients (66%) hadn t passed stools or gas for 4 hours, two patients (6%) had presented to the emergency department with a peritoneal syndrome, and the two other patients (6%) had been treated in the gastroenterology department for chronic constipation and were referred to our department for further investigations and surgery after confirmation of the diagnosis of HD. History-taking revealed chronic constipation and manifestations of sub-obstructive syndrome since childhood in 6 of our patients (5%). Physical examination showed: Important asymmetrical abdominal distension, a tympanic percussion note and a fecaloma on digital rectal examination in the eight patients, who presented with acute intestinal obstruction. A temperature of 38.5C, generalized guarding with painful mobilization of the pouch of Douglas on digital rectal examination in the two patients admitted to the emergency department for a peritoneal syndrome. Plain X-ray abdomen examination was performed in all patients (Fig and ); results are shown in Table. Barium enema was performed in of our patients only.

3 Int. Res. J Surg. Table. Findings of plain X-ray abdomen examination Radiologic findings Number N Percentage % Absence of pelvic aeration 83% Bowel distention 83% Hydro-aeric levels 8 66% fecaloma 6 5% pneumoperitoneum 8% Figure and. Appearance of a fecaloma and of distended colon on different plain X-ray radiographs of the abdomen It showed the transition zone and thus determined the level of the aganglionosis (Fig 3). Abdominal CT scan was performed in the patients who had presented to the emergency department with an obstructive syndrome (n=8) to confirm the organic nature of the obstruction, determine its site and above all rule out a tumoral origin of the obstruction which is more common at this age in our patients. Plain X-ray radiographs obtained in our patients revealed an important abdominal distention without an obvious obstacle proximal to a fecaloma. Anorectal manometry was performed in 3 patients (5% of cases) aged, and 9, respectively. Recto-anal inhibitory reflex RAIR) was absent in the three cases. Biopsies were practiced in all patients: endoscopic biopsy in patients and open rectal biopsy in patients. The absence of ganglion cells and hyperplasia of the myenteric nerve plexus (nerve nets) were confirmed in all cases. Histologic examinations revealed that it was a short rectal form in patients (83% of cases) and a rectosigmoid form in patients (7% of cases). Expectant treatment was prescribed to patients rushed to hospital and in whom the diagnosis of HD hadn t been made yet. Left lateral colostomies (9 by open surgery and one by laparoscopy) were performed in the patients with an obstructive syndrome. As for the two patients with a peritoneal syndrome, a peritoneal lavage was carried out with resection of the perforated colonic segment and colostomy, using the Bouilly-Volkmann procedure in one patient and the Hartmann procedure in the other. Definitive treatment was proposed as a first intention treatment to the two patients referred by the gastroenterology department, and as a second intention treatment to the rest of patients who had had derivation colostomies (3 months on average after lateral colostomy and 6 months on average in the patients who had had acute peritonitis). In each patient, surgery consisted of a colorectal resection involving the affected zone and the dilated colonic segment with a coloanal anastomosis on a J- shaped colic reservoir protected by a right lateral

4 Zaafouri et al. Figure 3. Barium enema radiograph obtained in a year-old patient showing the level of the aganglionosis Table 3. Follow-up and functionnal outcome according to Holschneider s score in 6 of our patients patients sex Age (years) Follow-up time (months) Holschneider s score M F M M 36 5 F M 36 ileostomy. Restoration of continuity was achieved 4 months postoperatively on average. There were no deaths in our series. There were two cases of coloanal anastomosis which required repeated dilatations under general anesthesia before restoration of continuity achieved 5 months and months after surgery, respectively. Soiling was noted in a patient who was referred to a specialized center for functional rehabilitation. Eight months later, the results were considered satisfactory. Mean follow-up time was 4 months. We managed to call six of the twelve patients. Four of them considered their functional outcome fair; whereas the two others considered it good according to Holschneider s score (Table 3). DISCUSSION Through the study of these cases, the authors underline the characteristics of adult Hirschsprung s disease which, in spite of its rarity, poses diagnostic and therapeutic problems: The absence of specific symptoms and the regular use of laxatives and even of cleansing enemas account for the high frequency of acute bowel obstruction as an initial complaint. Two therapeutic approaches were adopted in the management of our patients: The first approach consisted of a two-stage surgical operation comprising a colostomy in its first phase, for bowel cleaning by removal of the fecaloma, followed, a few months later by surgical resection. The second approach was based on a single-stage operation in which the resection, restoration of continuity and coloanal anastomosis were all performed during the same intervention. After a follow-up period of 4 months, 66% of the patients we got in touch with considered their intestinal functional outcome fair and 34% of them considered it good. Even though Duhamel s operation is the most practiced procedure in the world for the management of adult HD, we think, after this study, that colorectal resection with coloanal anastomosis when they are performed by experienced surgeons, give in the long run excellent results with a low morbidity and

5 Int. Res. J Surg. Table 4. Examples of published cases of adult HD Years Authors Number of cases References authors 7 [5-8] [-] 985 Elliott 39 [] 99 Starling 8 [] 995 Wu 5 [3] 5 Miyamoto [] Vorobyov 9 [4] Duncan [5] no surgery-related mortality. Adult HD was first described by Rosin in 95 (Rosin JD 95). Since then, several other cases have been reported in the literature: Hiatt in 95 (Hiatt RB 95), Kempton in 954 (Kempton JJ 954), Lee in 956 (Lee CM Jr 956) and others (Rehbein F 966; Marshall WH 96; State D 963; Madsen CM 964; Briggs HC 97; McGarity 974; Horovitz IL 974; Metzger PP 978; Lesser PB 979; Swenson O 957; Maglietta ED 96; Myers MB 966). About 55 cases have been reported in the English literature since 95 (Doodnath R, ) consisting mainly of case reports and of some retrospective studies (Table 4). The incidence of adult HD is still unknown, though grover announces that it can be as high as % of the population (Kunal G 9). Some authors (Parc R 99) think that there are no cases of adult HD (cases of HD of adult onset) but there are only cases which go undiagnosed or misdiagnosed until adulthood. The very good body care given to children by their mothers and which consists mainly of regular cleansing enemas, allows these HD patients to reach adulthood before having to undergo surgery. Some other authors (Miyamoto M 5; Barnes PR 986) have reported cases of HD in patients aged over 3 without a history of constipation or of other relevant symptoms and signs in their childhood. Rich (Rich AJ 983) reported a case of newly diagnosed HD in a 74-year-old patient. A diagnosis of adult HD is made if a patient (with colorectal aganglionosis) is older than the arbitrary age of years at the time of diagnosis (Miyamoto M 5; Barnes PR 986; Fairgrieve J 963; Mc Cready RA 98). As with HD presenting in the neonatal period, adult HD is predominant among males (Table 5). Like in our series, a history of chronic constipation was reported by most patients in the different studies (Elliot MS 985; Fairgrieve J 963; Todd IP 977). Habitually, it was a major constipation that required laxatives and often enemas and whose course was interrupted by unexplained episodes of remission or on the contrary by an intestinal obstruction that may have necessitated a laparotomy and even a colectomy. All of Duncan s (Duncan N D ) and Wang s (Lin Wang 4) patients suffered from unexplored chronic constipation. Mean duration between two defecations for Duncan s patients was 3 to 4 weeks, with an extreme of weeks. Acute intestinal obstruction was the most frequent chief complaint of our patients with 66% of cases against.8% of cases in the meta-analysis by Doodnath (Doodnath R ). Habitually, it was a lower obstruction caused by a fecaloma. There were, however, other cases where the obstruction was due to a volvulus of the sigmoid colon (5 cases) or to a volvulus of the transverse colon ( patient). Alagumuthu (M Alagumuthu ) reported in three new cases of adult HD revealed by a volvulus of the sigmoid colon. Severe abdominal distension caused by a fecaloma may lead, in the absence of urgent and adequate management, to urinary complications such as acute urinary retention which in turn may lead to the diagnosis of HD like in the case reported by Loganathan in 3 (P Loganathan 3), or to chest and respiratory tract complications such as mediastinal deviation, pulmonary atelectasis or pneumonia (Metzger PP 978; Crocker NL 99). Barium enema examination reveals a typical funnelshaped transition zone (Teitelbaum DH 6) and permits thus to determine the level of the aganglionic segment (Starling JR 986). This zone may, however, be absent in the very short forms (less than 5cm) of HD (Crocker NL 99; Keighley MRB 993). Kim (Kim HJ 8) was unable to detect this radiologic feature in % of his patients. The second sign is the retention of the product of contrast that we can visualize on late radiographs. Barium enema led to the diagnosis in 84.3% of Vorobyov s patients (Vorobyov G I ). All of Kim s patients (Kim HJ 8) had benefited from abdominal CT scans that permitted to exclude the other differential diagnoses and to detect the radiologic transition zone (the level of the aganglionosis) in each patient. Abdominal CT scan examinations were

6 Zaafouri et al. 3 Table 5. sex ratio of HD in adults Year Authors Sex ratio M/F References 98 McCready 4 (4/) [3] 985 Elliott (6/3) [] 99 Starling (8/) [] 995 Wu 4 (4/) [3] 995 Kim.75 (7/4) [3] Vorobyov.6 (65/5) [4] Duncan.6 (8/3) [5] practiced in our patients who had presented to the emergency department with an obstructive syndrome mainly to rule out a tumoral cause which is more common at this age. Histologic examinations of the different operative specimens led to the same conclusion as the CT scans, i.e. the findings revealed by both methods totally agreed as to the level of the aganglionosis. Anorectal manometry is not a specific diagnostic procedure of HD: RAIR is absent in idiopathic megacolon (Barnes PR 986) and may be present in very short aganglionoses limited to the anal canal (Penninckx F 975; Yoshioka K 987). Histologic confirmation was obtained prior to surgery for all our patients by open rectal biopsy (7% of cases) or endoscopic biopsy (83 of cases). In Duncan s series (Duncan N D ), however, the diagnosis of HD was confirmed by open rectal biopsy in patients out of (9.9% of cases). Rectal suction biopsy provided an inadequate specimen whose results couldn t be fully exploited in a third of patients in some series (Teitelbaum DH 6; Alizai NK 998). Croffie [46] concluded that this technique gives better results when it is used in children less than 3 years old. Histological data provided by biopsies from HD patients determine the form of the disease which can be ultra short, rectal, rectosigmoid or totally colonic. Resection of the aganglionic zone depends on the level of the aganglionosis. Myers (Myers MB 966) was the first to describe in 966 a case of HD in a 37-year-old man in whom the aganglionosis and hyperplasia of the nerve nets involved all the colon. In our series there was a marked predominance of the rectal form (83% against 7% for the recto-sigmoid form). Our results are in accordance with those published by Doodnath in (Doodnath R, ). In fact, out of 39 patients, 79.8% of them had lesions that were confined to the rectum against.5% with rectosigmoid lesions. Fairgrieve (Fairgrieve J, 963) suggested in 963 an early therapeutic approach for the management of adult HD. This approach was based on a two-stage operation in which the first stage consisted in performing a colostomy to facilitate bowel cleaning by evacuating the fecaloma, and the second stage, carried out a few months later, was devoted to surgical resection and coloanal anastomosis. The author defended his strategy arguing that a time-interval between operations is necessary to allow the initially dilated colon to return to its normal diameter after colic derivation and to the surgeon to form the anastomosis without any bowel disparity. However, initial colostomy does not totally guarantee successful fecal clearance. In fact, of the 3 patients from Duncan s series (Duncan N D ) who had undergone colostomy for bowel cleaning, two of them had to be operated on without totally evacuating the fecaloma. A second therapeutic approach that was adopted by several surgeons, was based on a single-stage operation-i.e. the patient is brought to the operating theatre only once. This is the case with Vorobyov (Vorobyov G I ) who chose this approach for 67.8% of his patients. Two-stage surgery is reserved, according to its defenders, for patients who have initially undergone emergency surgery for acute intestinal obstruction or for perforated colic peritonitis. This was the case for 83% of our patients and 3.% of Vorobyov s patients (Vorobyov G I ). Surgical treatment of HD consists in resecting or excluding the aganglionic zone in the digestive tube while avoiding nerve injury and preserving normal anal sphincter function. The choice of the procedure depends not only on morbid-mortality and on its functional outcome, but also on the habits and experience of the team of surgeons and other care givers. The difference in terms of results between the different procedures is not significant and should be considered cautiously given the small number of published cases of adult HD and the absence of prospective randomized and comparative studies. The Duhamel procedure is the most practiced technique in the world: according to a British review study published in (Doodnath R, ), a total of 3 patients (47.%) among 49 patients who had undergone surgery were operated on by the Duhamel procedure. This technique does not require resection of

7 Int. Res. J Surg. 4 Table 6. Postoperative complications occurring in 49 adult HD patients between 95 and Complications Number Percentage (n=) % Anastomotic structure. Anastomotic dehiscence Pelvic abscess. Perineal abscess. Pre-sacral abscess 3.6 Bleeding requiring transfusion.4 Necrosis or retraction of colon 3.6 Enterocolitis 5. Pulmonary embolism. Impotence 3.6 Anastomotic fistula 5. the aganglionic rectum; the rectum is only excluded and thus the patient is spared any extensive pelvic dissection that may damage the pelvic sensory nerves. For these reasons, several authors (Miyamoto M 5; Elliot MS 985; Todd IP 977) think that it is the procedure that is associated with the lowest rate of postoperative complications. As we said earlier, the choice of the technique often depends on the habits and experience of the team of surgeons. Like Duncan (Duncan N D ), we didn t in our department use this procedure for our patients. Wang (Lin Wang 4) published in January 4 the long-term results obtained over 44 months and the quality of life of 59 patients operated on at Jinling Hospital (Ning Li 3). The technique adopted, called Jinling technique, consisted in performing a subtotal colectomy with a modified Duhamel procedure. The different stages of this technique are as follows: a subtotal colectomy associated with an appendicectomy, an end-to-side colorectal mechanical anastomosis on the posterior wall of the rectum at cm from the pectinate line and a side-to-side colorectal mechanical anastomosis over 5 to 6 cm forming a kind of colorectal pouch. Forty-two percent of the patients from this series were operated on by laparoscopy. The distinctive technical feature of the Soave procedure is that it can entirely be performed by lower approach. According to data from the literature (Kleinhaus S 979) it is associated with the highest rate of postoperative complications (anastomotic stricture, for example). Myectomy that was first described in 966 by Lynn (Lynn HB 966) and practiced in Mayo Clinc, is mainly reserved for the short or recto-sigmoid forms, but can also be used in combination with laparoscopy for the treatment of more extensive forms. Its advantage over other procedures is that it can be limited to a perineal approach. In 95, State (State D 95) described the anterior resection of rectum and colectomy as therapeutic possibilities in the management of HD in 3 patients aged between and. In our department, we have chosen, out of habit, to perform a colorectal resection and to fashion a coloanal anastomosis with a shaped reservoir in all our patients. The rate of specific morbidity was low and the functional outcome was rather satisfactory. Post-operative complications in adult HD patients were mostly minor. Non-specific complications or specific ones such as anastomotic dehiscence, anastomotic stricture, pelvic abscess, anastomotic fistula, retraction or necrosis of colon may occur. Here we have in the following Table 6 a summary of the postoperative complications occurring in 49 adult HD patients between 95 and. In Table 7, we sum up to postoperative complications occurring in 9 adult HD patients reported in the English literature up to 5. Eighty-two percent of Vorobyov s patients (Vorobyov G I ) considered their sphincter functional outcomes good, 5% of them thought they were satisfactory and only 3% found them poor. The author concluded that, based on statistically significant data (p<.5) the longterm functional outcome after a Duhamel procedure depended on the extension of the aganglionic zone and on the extent of dilatation proximal to it: the more

8 Zaafouri et al. 5 Table 7. Postoperative complications in adult HD patients Techniques Number of cases Postoperative complications major minor Duhamel % 5.7% Swenson % 5.7% Soave 3 9.4% 9.7% Myectomy 35.9%.9% LAR/myectomy 8 % % LAR/colectomy 33.% 6.% Table 8. Functional outcome after surgery in adult HD patients Techniques Number of cases Functional outcome Good Fair Poor Duhamel % 9.5% % Swenson % % 4.3% Soave % 6.5% 6.% Myectomy % 5.7% 48.6% LAR/myectomy 8 % % % LAR/colectomy % 6.% 5.% confined to the rectum the lesions are and the dilatation limited to the sigmoid, the more satisfied the patient is. In Wang s series (Lin Wang 4), patients had 7 major postoperative complications such as anastomotic dehiscence, rectal incontinence, intestinal obstruction, sexual or urinary complications. Statistically significant data revealed that the patients who had had this procedure were satisfied with the functional outcome in 97% to 98% of cases (evaluation made by the scores of Wexner, BFS and GIQLI). We sum up in Table 8 the functional outcomes occurring after surgery in 9 adult HD patients published in the English literature up to 5. It is clear that the Duhamel procedure is the most practiced by surgeons all over the world, that it is associated with the lowest rate of postoperative complications and at the same time with the best longterm outcomes. CONCLUSION Adult Hirschsprung s disease is rare but by no means exceptional. It should be considered in every young patient presenting with a history of long-standing constipation. Diagnosis is first of all clinical. Barium enema radiographs may be pathognomonic and spare the patient further investigations to confirm the diagnosis Recto-anal inhibitory reflex is usually absent on manometry. Primary treatment of adult HD is surgical. The choice of the procedure depends not only on morbid-mortality and on its functional outcome, but also on the habits and experience of the team of surgeons and other care givers. Even though the Duhamel procedure is the most practiced in the world for the treatment of HD in adults, we believe after this study that colo-anal resection with

9 Int. Res. J Surg. 6 coloanal anastomosis gives in the long-run satisfactory and even excellent results with no mortality and a low morbidity rate, provided that the operation is performed by an experienced team. AUTHORSHIP Conception and design of study: Zaafouri H, Ben Maamer A Acquisition of data: Mrad S, Mabrouk M Data analysis and or interpretation: Haoues N, Bel Hadj Salah M Drafting of manuscript: Zaafouri H and Noomene R Approval of final version of manuscript: Bouhafa A and Cherif A REFERENCES Alizai NK, Batcup G, Dixon MF, Stringer MD (998). Rectal biopsy for Hirschsprung s disease: what is the optimum method? Pediatr Surg Int, 3: 4. Barnes PR, Lennard-Jones JE, Hawley PR (986). Hirschprung s disease and idiopathic megacolon in adults and adolescents. Gut, 7: Briggs HC (97). Aganglionic megacolon in adults. Conn Med 35: Crocker NL, Messmer JM (99). Adult Hirschsprung s disease. Clin Radiol 44: Croffie JM, Davis MM, Faught PR (7). At what age is a suction rectal biopsy less likely to provide adequate tissue for identification of ganglion cells? J Pediatr Gastroenterol Nutr, 44: 98. Duncan N D, Plummer J, Dundas S E, Martin A, McDonald AH (). Adult Hirschsprung s disease in Jamaica: operative treatment and outcome. Colorectal Disease, 3: Doodnath R, Puri P (). A systematic review and meta-analysis of Hirschsprung s disease presenting after childhood. Pediatr Surg Int, 6:7. Elliot MS, Todd IP (985). Adults Hirschsprung s disease: results of Duhamel procedure. Br J Surg, 7: Fairgrieve J (963). Hirschsprung s disease in the adult. Br J Surg, 5:56 4. Grove K, Ahlawat SK (9). Hirschsprung disease in adults. South Med J, :7 8. Hiatt RB. The surgical treatment of congenital megacolon (95). Ann Surg, 33:3 9. Holschneider AM, Ure BM, Pfrommer W et al (996). Innervation Patterns of the Rectal Pouch and Fistula in Anorectal Malformations: A Preliminary Report. J Pediatr Surg, 37: Horovitz IL, Baier I (974). An unusual case of aganglionic megacolon. Dis Colon Rectum, 7: Keighley MRB, Williams NS (993). Adult Megacolon and megarectum. In Surgery of the Anus, Rectum and Colon, : Kempton JJ (954). Hirschsprung s disease in a man aged 3. Proc R Soc Med, 47: Kim CY, Park JG, Park KW, Parc KJ, Cho MN, Kim WK (995). Adult Hirschsprung s disease. Result of the Duhamel procedure. Int J Colorectal Dis, :56 6. Kleinhaus S, Boley SJ, Sheran M, Sieber WK (979). Hirschsprung s disease, a survey of the members of the surgical section of the American Academy of Pediatrics. J Pediatr Surg, 4: Kim HJ, Kim AY, Wook C et al (8). Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation. Radiology, 47: Kunal G, Sushil K (9). Hirschsprung Disease in Adults. Southern Medical Journal, :7-8. Lee CM Jr (956). Experience with the extended postoperative care of congenital megacolon. Am Surg, :75-7. Lesser PB, El-Nahas AM, Luki P (979). Adult-onset Hirschsprung s disease. JAMA, 4: Lin Wang, Qi He, Jun Jiang, Ning Li (4). Long-term outcomes and quality of life after subtotal colectomy combined with modified Duhamel procedure for adult Hirschsprung s disease. Pediatr Surg Int, 3:55 6. Lynn HB (966). Rectal myectomy for a ganglionic megacolon. Mayo Clin proc, 4: Madsen CM (964). Hirschsprung s disease congenital intestinal aganglionosis. Munksgard, Copenhagen, 6:45-6. Alagumuthu M, Jagdish S, Kadambari D (). Hirschsprung s disease in adults presenting as sigmoid volvulus: a report of three cases. Tropical Gastroenterology, 3(4):34 3. Maglietta ED (96). Congenital aganglionic megacolon in adults. Arch Surg, 8: Marshall WH, Kutz JE (96). Adult Hirschsprung s disease. J Ky Med Assoc, 6:56-9. Mc Cready RA, Beart RW Jr (98). Adult Hirschsprung s disease: Results of the surgical treatment at Mayo Clinic. Dis Colon Rectum, 3:4-7. McGarity WC, Cody JE (974). Complication of Hirschsprung s disease in the adults. Am J Gastroenterol, 6:39-3. Metzger PP, Alvear DT, Arnold GC (978). Hirschsprung s disease in adults: Report of a case and review of the literature. Dis Colon Rectum, :3-7. Miyamoto M, Egami K, Maeda S et al (5). Hirschsprung s disease in adults: report of a case and review of the literature. J Nippon Med Sch, 7:3. Myers MB, Bradburns D, Vela R (966). Total aganglionic colon in an adult: First reported case. Ann Surg, 63:97-. Ning Li, Jun Jiang, Xiaobo Feng, Weiwei Ding, Jianlei Liu, Jieshou Li (3). Long-term Follow-up of the Jinling Procedure for Combined Slow-Transit Constipation and Obstructive Defecation. Dis Colon Rectum, 56:3. Parc R (99). Traitement de la maladie de Hirschsprung chez l'adulte. Traité de Techniques chirurgicales - Appareil digestif, 4:6-7.

10 Zaafouri et al. 7 Penninckx F, Kerremanns R (975). Evaluation of anorectal motility and rectoanal reflex in Hirschprung s disease and functional constipation. Acta Paediatr Scand, 48:47 5. Loganathan P, Vipperla K, Umapathy C,Gajendran M (4). Acute urinary retention from coprostatic colon: an unusual presentation of adult Hirschsprung s disease. QJMed doi:.93/qjmed/hct9. Rehbein F (966). Surgical problem in congenital megacolon Hirschsprung s disease. J Paediat Surg, :56-8. Rich AJ, Lennard TW, Wilsdon JB (983). Hirschsprung s disease as a cause of chronic constipation in the elderly. Br Med J, 87: Rosin JD, Bargen JA, Waugh JM (95). Congenital megacolon of a man 54 years of age: report of case. Proc Staff Meet Mayo Clin, 5:7 5. State D (963). Segmental colon resection in the treatment of congenital megacolon (Hirschsprung s disease). Am J Surg, 3:93-. State D (95). Surgical treatment for idiopathic congenital megacolon (Hirschsprung s disease). Surg Gyne Obst, 85:-. Swenson O (957). Follow up on patients treated for Hirschsprung s disease during a ten years period. Ann Surg, 46:76-4. Starling JR, Croom RD III (986). Hirschsprung s disease in young adults. Am J Surg, 5:4-9. Teitelbaum DH, Coran AG (6). Hirschsprung s Disease and related neuromuscular disorders of the intestine. In: Pediatric Surgery, 6: Todd IP (977). Adult Hirschsprung s disease. Br J Surg, 64:3. Vorobyov G I, Achkasov S I, Biryukov O M (). Clinical features diagnostics and treatment of Hirschsprung s disease in adults. Colorectal Disease, :4-8. Wu JS, Schoetz DJ, Coller JA (995). Treatment of Hirschsprung s disease in the adult: Report of five cases. Dis Colon Rectum, 38:65-5. Yoshioka K, Keighley MRB (987). Anorectal myectomy for outlet obstruction. Br J Surg, 74: Accepted 9 February, 5. Citation: Zaafouri H, Mrad S, Mabrouk M, Haoues N, Salah MBH, Noomene R, Ahmed Bouhafa, Anis Ben Maamer and Abderraouf Cherif (5). Hirschsprung s disease in adults: Clinical and therapeutic features. International Research Journal of Surgery, (): 9-7. Copyright: 5 Zaafouri et al. This is an openaccess article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are cited.