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Metods of surgical treatment and postoperative complications of hirschsprung's disease in adults
7. Ravi K. Bashyal et all. Complications After Pinning of Supracondylar Distal Humerus Fractures//J Pediatr/Orthop- Volume 29, Number 7, October/Novtmber 2009. P. 704-708.
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Mirzachmedov Murad Mirchaidarovich, Tashkent Medical Academy. E-mail: [email protected]
Metods of surgical treatment and postoperative complications of hirschsprung's disease in adults
Abstract: The material of the study has formed 82 sick, found on stationary treatment in Republican Scientific Centre Coloproktoloii since 1993 on 2014 years.
As it is seen, 82 sick mans was 58 (70,7%), womans 24 (29,3%). 21 (25,6%) sick were at age from 15 before 19 years, 32 (39%) at age from 20 before 24 years, 16 (19,5%) at age from 25 before 29 years and 5 (15,9%) sick from 30 years.
Diagnosis of Hirschsprung's disease in adults requires a comprehensive assessment of clinical symptoms, these studies X-ray inspection and results of functional studies of colon and rectal sphincter apparatus, morphological study. The best way to surgery for Hirschsprung's disease in adults is abdominal-anal resection of the rectum with bringing down the proximal colon to the anal canal, which, in terms of removal of hypo- or aganglionizis area meets the requirements of radicalism. Our proposed form of surgical treatment to reduce the number of postoperative complications, contributes to early recovery of motor-evacuation function of the colon and the normalization of self-defecation. Keywords: agangliozis, hipogangliozis, megacolon.
Introduction
Despite the progress made to date advances in the treatment of Hirschsprung's disease in adults, the problem is still far from being solved, as evidenced by very comforting percentage of the so-called secondary pathology, and most importantly — continuing treatment in specialized hospitals of patients undergoing primary corrective surgery [1; 3]. The reason for such appeals are persistent functional abnormalities that contribute to disability. In this context, efforts aimed at improving the treatment outcome of adult Hirschsprung's disease are highly relevant. Most researchers now suggest that Hirschsprung's disease is more common than diagnosed now. There are certain prerequisites for a slow, sometimes latent flow Hirschsprung disease in adults, is the existence of variants of congenital morphological changes, such as the short zone aganglioza, gipoganglionarnye form in the absence of marked degenerative processes in muscle tissue distal colon. Requires careful study and analysis of the causes of unsuccessful outcomes of surgical interventions, the improvement of a differentiated approach to the selection and determination of optimal methods for their correction, which was the goal of our work. [2; 4].
Various methods of surgical procedures and their modifications. In the arsenal of methods of surgical treatment of Hirschsprung's disease in children, there are various types of operations: Swenson, Soave, Duhamel and others in adults using these classical methods of pediatric surgery is difficult because of gross changes in the colon, the pelvic anatomy and relationships of the pelvic organs. At the same time, most attention in these publications is given to the results of surgical treatment of anomalies, and information regarding the clinic, diagnosis and surgical treatment for Hirschsprung's disease in adults, are presented only factual information without analysis because of the paucity of clinical observations.
The aim of our work was to study and analyze the causes of unsuccessful outcomes of surgical interventions and improvement of a differentiated approach to the selection and definition of best practices in surgery for Hirschsprung's disease in adults.
Material and methods
The Republican Scientific Centre Coloproctology in 19932014 years there were 82 patients with Hirschsprung's disease at the age of 16-30 years.
12 patients out of 82 patients in childhood have been performed different types of surgical treatment of Hirschsprung's disease (Swenson, Isakov, Soave). The program consisted of a survey of patients with functional studies closing apparatus of the rectum (sphincterometry, electromyography), rectoromonoscopy, colon X-ray inspection, endoscopy, ultrasound of the abdomen and pelvis. And also to clarify the diagnosis was performed transanal biopsy of the muscle layer of the rectum by Swenson. The differential diagnosis of Hirschsprung's disease in adults requires an individual approach, a comprehensive assessment of clinical symptoms, X-ray data and research results of functional studies of colon and rectal sphincter apparatus, morphological study. Radiographic methods allow to evaluate and determine the severity of megacolon, reveal a characteristic pattern of restriction and suprastenoticheskogo expansion. Particularly difficult diagnosis is very short (anal or overanal) hypo-or aganglios colon. It is not necessarily the presence of classical paintings in a narrower (hypo-or aganglios) area, as in children, is the physiological norm of the internal anal sphincter relaxation under tension received feces.
Results and discussion
Materials and methods
Consisting of ten subdivisions in which there is presented general characteristic of the patients, instrumental, laboratory, functional methods of examination and biopsy by Swenson. The work is based on the analysis of the data of examinations obtained from 82 patients (males — 58 and females — 24) with Hirschprung's disease at the age above 15 years, who received stationary treatment in The Research center of Coloproctology of the Ministry of Health of the Republic of Uzbekistan during the period from 1993-2014. Of them 21 (25,6%) patients were at the age 15-19 years, 32 (39%) — at the age 20-24 years, I6 (19,5%) — at the age 25-29 years, 5
Section 6. Medical science
(15,9%) — at the age of 30 years and more. The average age was 26,3±2.4 years.
For representation of the research the patients were divided into two groups. The studied group included 28 (34.1%) patients, who were performed resection of the rectum with pull-through of the proximal sites of the large intestine into the anal canal. Control group was consisted of 54 (65.9%) patients, which underwent the operation of Duhamel in modification. Twelve patients including 2 patients from studied and 10 ones from control group the surgical treatment was performed in two steps. He patients in these two groups were comparable by sex and age.
The biopsies on Swenson take from 50 (61%) beside 31 (57,4%) sick in checking group. Aganglioz is diagnosed beside 2 (6,4%) examined, hypogangliosis — beside 29 (93,6%). As a result ofbiopsies, which is organized beside 19 (67,8%) sick from the main group, agangliosis is diagnosed beside -1 (5,3%), hypogangliosis — beside 18 (94,7%).
There is given definition of the surgical tactics and choice of a method of operative treatment of Hirschprung's disease in the adult. Surgical tactics, preoperative preparation, choice of the access and volume of operative intervention in Hirschprung's disease in the adults are not developed to the end. The main purpose of the surgical treatment of Hirschprung' disease is removal of hypo- or agan-glionar zone, the adequate resection of decompensated dilated parts and saving of the functioning proximal part of the large intestine.
The refore success of surgical treatment of Hirschprung's disease in the adults directly depends on: the correctly made
diagnosis; adequate preoperative preparation; removal of hypo-or aganglionar zone and dilated parts of the large intestine. On our opinion, for each adult patient with Hirschprung's disease the individual tactics should be selected depending on time of preparation to Radical operation, duration of postoperative period, duration and degree of postoperative rehabilitation. We have developed the modified method of surgical treatment of Hirschprung disease in the adults. The surgery is performed synchronously by two teams of surgeons: one carries out peritoneal stage of intervention, the second team performs perineum stage of intervention.
The distinction of the offered method is that we recommend to perform mobilization close to the posterior wall of the rectum in order not to damage sacral veins as well as for reduction of the small pelvic cavity, traumatization of the nervous trunks directed to the small pelvis. Then we pull-through proximal site of the colon to the anal canal with excessive length 4-5 sm (it will be cut off in 14 days after operation). After pulling-through we drained presacral area (behind the pulling-through intestine) with use developed in out clinic special drainage tube for lavage performance and carefully restore the integrity of the pelvic peritoneum.
The success of the rectal PAR with pulling-through PAR equally depends on the correctly performed perineal stage of procedure. It is caused not only by its technical complexity, but also inevitable infringement of innervation and injury of the anal sphincter (Priority reference of the Agency of Intellectual Property of the Republic of Uzbekistan N IAP 2014 230 of 03.06.2014) (Fig.1).
a b c
Note: a-mobilization of the rectum from the side of abdominal cavity. b-pulling-through of the proximal sites of the large intestine with excess. c-Cutting off of excessive pulling-through with application of coloanal anastomosis.
Figure 1. Stages of the rectal PAR with pulling-through
The technique of surgical treatment of Hirschsprung's disease in the adults depended on the length of megacolon, intoxication intensity, severity of the general state of the patient, visual assessment of the preparation quality to the operation of the large intestine (good, satisfactory, unsatisfactory result) and efficacy of the conservative measures performed. At a stable state of patients, megacolon, limited by one or two parts of the colon, good or satisfactory preparation to operation on the large intestine, at absence of complications of disease there was performed one-step radical surgical treatment — removal of hypo- or aganglionar zone with expanded decompensated parts of the large intestine and formation of primary coloanal anastomosis (one step operation).
One-step surgical treatment was carried out in 70 (85,3%) adult patients with Hirschprung's disease: 26 (37,2%) from them underwent PAR of the rectum with pull-through of the proximal parts of the large intetineintoanal canal with application of coloanal anasto-
mosis (group 1, studied): 44 (62,8%) — operation of Duhamel in modification (group 2, control).
Proximal border of the resection of the colon was determined by approaching intestinal diameter to the normal sizes, good definition of shadows. In the early postoperative period the complication have arisen in 18 (22%) operated patients. One patient died (1,2%). The late postoperative complication after PAR of the rectum observed in 8 (9,8%) patients: in 2 (25%) of them was partial stricture of the pull-through intestine. After operation of Duhamel in modification complications as strictures of colorectal anastomosis were developed in 6 (75%) patients.
The long-term functional results of treatment after peritoneal-anal resection of the rectum with pull-through of functional proximal parts of the large intestine in the anal canal were observed in 26 (31.7%) patients. The good and satisfactory results were obtained in 85 and 7,1%, of cases, respectively.
Comparative evaluation of various surgical corrections of hirschsprung's disease in adults
At the inefficiency of preoperative preparation, progressive decompensation of the large intestine with signs of intestinal obstruction, expanded dilatation of the large intestine, presence of fecal stones in the rectum and colon, unsatisfactory preparation to operation on the large intestine at worsening of the general health state induce by chronic intoxication and disturbance of metabolism, the surgical treatment were carried out in two-steps.
Multi-staged surgical treatment were performed in 12 (146%) patients (2 from studied and 10 from control group).
Study of the long-term functional results in the patients of the both group was performed by questionnaire, sphincterotomy and anorectal manometry in 3, 6 and 12 months after operation.
In the studied group after operation par of the rectum in 12 months the number of patients with unsatisfactory results reduced from 7,7. to 4,5%. At the same time the quantity of good (69%) and satisfactory (23,1%) results increased from 23,1 to 86%.
The study offunctional result was performed by two methodic: by scale of incontinence W exner (1993) (table 7) and with help of original questionnaire for study of functional results (Fig.2). Totally there were examined and interviewed 26 patients from studied and 35 — from control groups. The results of interview with use of scale of incontinence Wexner (1993) showed that average value in the studied group was 4.1 (1-13) and in the control — 6.3 (1-12). Unsatisfactory result, that is, more then 4 points, was registered in 2 (7,7%) patients having operation PAR of the rectum. After methods
of Duhamel in modification the unsatisfactory results were more — in 9 patients (25,7%).
Conclusion
1. One-step radical surgical intervention treatment is executed in both group patient in 85,3%. The Evidences to much-step to treatment are: of the preparation to the operation on the large intestine, presence coprolitis, progressing decopension large intestine with sign of the intestine impassability, deterioration of the general condition of the patient. The First stage must be directed on liquidation of the intestine impassability, megakolon and zones hipo- or aganglioz. After recovering the functions of the large intestine and improvements of the general condition sick restore intestine continuity.
2. Performance of peritoneal-anal resection of the rectum with voiding functionning proximal parts of the large intestine division in anal channel simplifies the technology to operations in contrast with method Duhamel and avoids the area hipo- or agangliozis.
3. The Remote functional results of the treatment (through 1224 months) turned out to be greatly better in the main group in contrast with operation Duhamel: observations incotinenction on scale Wexner was in 3,3 times less (7,7% and 25, 7% accordingly); the physiological volume of the imperative urge to defecation was above on 45 ml, differentiated urge to defecation had 82,5% patient in the main and 75% in checking group.
Surgical treatment and rehabilitation of these patients should be done only in specialized hospitals.
References:
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2. Alagumuthu M., Jagdish S., Kadambari D. Hirschsprung's disease in adults presenting as sigmoid volvulus: a report of three cases.//Trop. Gastroenterol. - 2011. - Vol. 32, № 4. - P. 341-343.
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Navruzov Sarimbek Navruzovich, Mirzachmedov Murad Mirchaidarovich, Republican Scientific center of Coloproctology Uzbekistan
E- mail: [email protected]
Comparative evaluation of various surgical corrections of hirschsprung's disease in adults
Abstract: In 1990-2014, eighty patients with Hirschsprung's disease were on an inpatient treatment in the Republican research centre for colon proctology of the Republic of Uzbekistan Ministry of Health in Tashkent.
Diagnostics was based on the scheme which included general clinical examination, radiological and endoscopic studies, USI of organs of the abdominal cavity and the small pelvis, proctosigmoidoscopy, functional studies of the colon and the sphincteric apparatus of the rectum, morphological studies (Swenson biopsy). The differential diagnosis of Hirschsprung's disease in adults requires an individual approach, an integrated assessment of clinical symptoms, data of an X-ray contrast study and results of functional studies of the colon and the sphincter apparatus of the rectum as well as undertaking a morphological study. Radiological methods make it possible to estimate and identify expression of the megacolon and reveal a characteristic picture of narrowing and suprasthenic expansion. Diagnostics of a very short (anal or supra-anal) hypo- or aganglionosis of the colon is especially complicated. The presence of a classical picture in the form of narrowed (hypo- or aganglionic) zones, as that in children, is not obligatory; a physiological norm is relaxation of the internal anal sphincter in its stretching with fecal masses. Keywords: megacolon, agangliozis, hipogangliozis, megarectum.
Introduction or a significant decrease in a number of neurons in ganglions of intra-
Hirschsprung's disease is characterized by congenital absence mural nervous plexuses of the colon. Hirschsprung's disease is one
