Ministry of health of Ukraine

Malformations of the colon. Hirschsprung's disease. Anorectal malformations.


1. Aktualnist topic. Today in Ukraine congenital malformations of the colon in children is not only a medical but also a social problem. Despite the possibility of early diagnosis of this disease, the development of new and improvement of known methods of surgical correction of these defects, Hirschsprung disease mortality ranges from 0 to 3.4%, while anorectal malformation may be absent if the child has no serious concomitant defects. In this regard, the study of clinical features, diagnosis, and treatment of these particular birth defects, allows to save valuable life most children with this disease, while achieving favorable functional outcomes and avoid serious complications.
2. Specific objectives:
1.Zasvoyity list of diseases that cause chronic constipation and violation of the act of defecation.
2. Analyze the major causes of violation of the act of defecation.
3. Recognize features of various clinical manifestations of chronic constipation.
4. Identify the peculiarities of individual defects that lead to the violation of the act of defecation.
5. Interpret helper methods (ultrasound, iryhohrafiya, manometriya, neyrohistolohiya, colonography, invertohrama, rectal examination), laboratory and biochemical parameters.
6. Demonstrate methods of history taking, examination of the patient and preparing children to Proctologic examination and surgery on the colon.
7. To analyze the causal relationships of congenital violation of the act of defecation, a syndrome of chronic constipation in some patients, justify and formulate preliminary clinical diagnosis.
8. To develop the algorithm of the physician in acute lower intestinal obstruction.
9. Summarize the terms and methods of surgical interventions in malformation of the colon and in anorectal malformation.
10. Interpret the principles of rehabilitation after surgery, be able to assess the prognosis of life possible violations after eliminating pathology of the digestive system.
4.2. Theoretical questions to studies
1. Etiology defects colon and rectum.
2. Characteristics of transit and yevakuatornoyi functions of the colon.
3. Classification of chronic constipation by ML bush and OI Lonyushkinym. What underlie it?
4. Pathogenesis of congenital colon and anorectal malformations.
5. Classification of Hirschsprung disease and anorectal malformations.
6. The main clinical manifestations of disease in various forms of Hirschsprung disease.
7. The main external clinical signs of anorectal malformations.
8. Methods of diagnosis of Hirschsprung disease.
9. Methods of diagnosis of anorectal malformations.
10. Preparation of the colon to Proctologic inspection and for operations.
11. Of surgical methods of treating disease Hihshprunha.
12. Motivation of landmark transactions in children with anorectal malformations.
13. Method of anorectoplasty by A. Peng
14. Rehabilitation of children after surgery for colon and rectum.

4.3. Practical work (tasks) to be performed in class.

1. Collect complaint history with the disease of the large intestine and in its malformation.
2. Demonstrate palpation. auscultation of the anterior abdominal wall.
3. Interpret data of laboratory and instrumental methods of diagnosis Hirschsprung's disease and anorectal malformation.
4. Recognize the clinical features that characterize Hirschsprung's disease and anorectal malformations.
5. Demonstrate: Technology-rectal digital examination anus and rectum; methodology for high-hypertensive treatment and enemas, methods of iryhohrafiyi and invertohramy; setting rectal probe.
6. Identify the indications for surgical treatment of Hirschsprung's disease and anorectal malformation.
7. On the example of a patient with Hirschsprung's disease or anorectal defect illustrate the clinical signs, determine the status of the patient, formulate a preliminary diagnosis.
8. Make a plan of survey and interpret ancillary methods (X-ray, endoscopy, ultrasound, CT, etc.)., Laboratory and biochemical analyzes of Hirschsprung's disease.
Content themes
First clinical picture of the disease described pediatrician Harald Hirschsprung in 1887 soon to determine the disease appeared terms "congenital megacolon," "congenital idiopathic megacolon" which is currently not used.
Hirschsprung's disease is very common among children: one in 2000-5000 newborns. Among patients, about 90% are boys. Some authors (Hayword, Camero, etc.). Indicate the hereditary nature of the defects. Great value in its origin with genetic factors. Found that ganglion cells intramural plexus with derivatives neyroblashiv who migrate kraniokaudalnomu direction of neural crest in the intestinal wall. At the 6th week of embryonic development in neuroblasts determine cardia of the stomach, on the 7th - in the small intestine, 8-10th, in the transverse colon and 12 th week - in the rectum. Migrating neuroblasts (nerve cells) in the downstream sections of the colon lasts from 9 th to 12 th weeks, from splenic bend to the outer muscle - the latches vidhidnyka (m. sphincter ani externus) - for four weeks, the formation of colon not going until until after the migration of neuroblasts. Submucosal nerve plexus formed by neuroblasts that migrate from musculo-intestinal plexus.
Thus, if not neuroblasts migrate into the intestinal wall into the 7th week of intrauterine period, there ahanhlioz entire colon. Violation of embryogenesis at the 9th week leads to ahanhliozu descending colon, sigmoid and rectum, 10-12th week - to ahanhliozu sigmoid colon and rektosyhmoyidnoyi plots in different variations.
Thus, Hirschsprung's disease - a congenital ahanhlioz distal colon. Absence, insufficiency or degenerative changes in the intramural nerve ganglia violation of nerve impulses in the reflex arcs of the bowel wall. This leads to gross structural changes in the muscle layer until his death. Nakano, Puri (2007) drew attention and proved by histochemical methods to reduce angiotensin 2, which can be responsible for the allocation of water and electrolytes from the intestinal wall. This may explain the occurrence of enterocolitis in Hirschsprung's disease.
Above the constriction formed by expanding gut, where the hypertrophy of muscle membrane caused hiperperystaltykoyu proximal colon to move contents through ahanhlionarnu area. It is noticed that in the area of ​​expansion is always secondary death hypertrophied muscle fibers with replacing them proliferating connective tissue, which leads to increased intestinal lumen over ahanhlionarnoyu area. Secondary changes occur in the submucous and mucous layers.
According to W. Schenach, 20% of patients ahanhlioz limited ekstraperytonealnoyu part (lower 1/3) of the rectum, in 60% of lesions captures rektosyhmoyidnu area of ​​the colon, 15% spread above the sigmoid colon and in 5% of patients affected the entire colon.
Classification of Hirschsprung disease suggested OI Lonyushkinym gives clear information about the size of the zone ahanhliozu, variants of clinical course of the disease and the feasibility of conservative treatment.
Classification of forms and stages of Hirschsprung disease (with AI Lonyushkinym, 1987).
A. Anatomical shape.
1. Rectal:
• with lesions perineal rectum (Hirschsprung's disease with superkorotkym segment);
• with lesions ampulyarpoho nadampulyarpoho and rectum (Hirschsprung's disease with a short segment).
2. Rektosyhmoyidna:
• with lesions distal sigmoid colon;
• with lesions greater part or all of the sigmoid colon (Hirschsprung's disease with a long segment).
3. Segmental:
• one ahanhlionarnym segment in rektosyhmoidniy area or sigmoid colon;
• Two ahanhlionarnymy segments and plot intact colon between them.
4. Subtotal:
• with lesions of the left half of the colon;
• the spread of the pathological process in the right half of the colon.
5. Total - defeat the entire colon (sometimes part of the small).
B. Clinical stages:
1. Compensated.
2. Subcompensated.
3. Decompensated: acute and chronic.
The leading symptom of Hirschsprung disease is the lack of self-emptying (chronic constipation). Terms of constipation, its further character mostly due to length ahanhlionarnoho segment features feeding, compensatory capabilities intestines. With a small area ahanhlionarniy delay discharge of meconium, and feces in infants often limited by days 3.2. Treatment is conservative by setting cleansing enemas. When the lesions are increasing signs of low obstructive ileus, which requires immediate conservative treatment.
For any form of Hirschsprung disease constipation constantly progressing. This is particularly evident in the introduction of complementary foods or switch to bottle-feeding. Fecal viscous consistency, compensatory possibilities gut decrease, constipation is permanent.
Conservative measures are not always conducive to the full release from intestinal feces, leading to condensation and accumulation of feces in the form of stones in the distal colon. Sometimes fecal stones can be very large, resulting in a false diagnosis of tumors of the abdominal cavity. Often these stones clog the intestine, then a hospital acute intestinal obstruction.
A typical symptom of Hirschsprung disease is bloating which appears as constipation from the first days of life. Chronic delays emptying and causes gas expansion symopodibnoyi guts, then other parts of the colon, leading to configuration changes and increase the size of the stomach. Thorax barrel shape through high standing diaphragm. Navel adjusted or turned over and is lower than normal. Offset enhanced gut at one time or the other side of the abdomen provides asymmetric shape during palpation increases peristalsis and determined visually distinct "walls" under the thinned anterior abdominal wall ("frog belly").
With age, the effects of chronic fecal intoxication is increasing, which is deteriorating general condition, retarded physical development, anemia, malnutrition, disturbance of protein metabolism and function of the liver, lungs, heart.
In the event of a long delay due to obturation bowel emptying or twisting may have vomiting and abdominal pain, intoxication, a sign of the threat of complications: intestinal obstruction or peritonitis due to perforation of the colon.
As a result of dysbiosis in the mucosa of the colon inflammatory changes occur, leading to diarrhea (paradoxical diarrhea).
Compensated form (mild, chronic).
In the neonatal period Hirshpruyha children with the disease do not differ from healthy. Sometimes there is a delay discharge meconium and stools, recurrent flatulence and vomiting, but the general condition of the child relatively satisfactory. Treatment is conservative by setting enemas or vapor tube.
The main symptoms of the disease appear in later periods (after 5-6 months), often after the introduction of complementary foods or transfer a child to artificial feeding. Originally intermittent constipation is light at regular intervals feces. But despite good care, the duration of constipation gradually increases.
The general condition and physical development of a child especially not suffer, but violations of the feeding and care of a child can lead to the formation of stones in the feces and intestines accompanied by nausea and bouts of abdominal pain. Children with chronic form of anemia and malnutrition are mild, signs of acute intestinal obstruction there.
Chronic disease caused by the presence of relatively short ahanhlionarnoyi zone while driving functional impairment of the colon are mild. Sometimes there is a discrepancy of clinical disease and the length of the zone ahanhliozu.

Subcompensated form (moderate or subacute).

This form of the disease is manifested by the end of the 1st month of life and pathogenesis due to large size areas ahanhliozu which is often in rektosyhmoyidniy area and descending colon. In case of inadequate child care possible deterioration of health and decompensation process and, conversely, if good care and adequate medical therapy overall can not change. We must, however, emphasize that over time the symptoms become more pronounced. Condition of the child progressively worsening constipation is more sustainable. Treatment is conservative by setting siphon enemas. Gradually develop signs of chronic fecal intoxication in poor care and occasional conservative treatment. In some cases, can be formed fecal stones that cause obturation at ahanhlionarnoyi zone and may be the cause of low acute obstructive ileus. Anemia and malnutrition develop gradually appear later.
Decompensated form (severe, acute).
For this form of Hirschsprung disease characterized by total or subtotal ahanhlioz colon. They exhibit a disease often immediately after birth. There is no discharge of meconium and faeces. Condition of the child worsens, there is flatulence, growing phenomenon of intoxication, vomiting initially stagnant gastric contents, then bile and feces. Abdomen sharply increased in volume, is spheric. At the anterior abdominal wall enhanced venous network konturuyutsya loop inflated colon, visible peristalsis. Setting enemas gives temporary effect, the child's condition is progressively deteriorating. Growing phenomenon of anemia and malnutrition. Prolonged fecal stagnation contributes to inflammation of the mucous membranes and the development of ulcerative-necrotic enterocolitis followed perfratsiyeyu wall of the colon and peritonitis in the newborn. Therefore, prevention of complications shown timely surgical intervention - applying fecal fistula.
Sometimes decompensated form of Hirschsprung disease occurs in older children as a result of complications during compensated form when there are pronounced intoxication and signs of intestinal obstruction.
Diagnosis. In studying the history of the disease is important to pay attention to the time of occurrence of constipation. If no self-emptying of the first days or weeks of life, it is the hallmark of innate ahanhliozu.
To diagnose the disease conducts finger rectal examination, which found an empty vial and hipoplazovanu rectum. Internal muscle tone - contactor vidhidnyka (m. sphincter ani internus) increased. If the surgeon can not hold a finger through the constriction zone, begins a rapid discharge of gases.
Mostly crucial in making the diagnosis are x-ray, which begins with observation radiographs of the chest and abdominal cavities upright. This makes it possible to detect changes in the lungs, heart and determine the position of the level of standing diaphragm. Ahanhlionarnu plot colon showing the absence of gas in it, proximal small and large intestines stretched from the level of liquid in them. The most informative study is radiopaque colon by introducing barium sulfate into the rectum through the vent tube (iryhohrafiya). Research should be carried out after the complete release from intestinal feces and gases by setting siphon enemas. After a tight filling of the colon through the catheter has four radiographs: two in direct and lateral projections at tight filling the colon to determine the degree of expansion suprastenotychnoyi zone and two radiographs after emptying to detect length ahanhlionarnoyi (constricted) zone. If necessary, make deferred shot. The main contrast material used for x-ray, barium sulfate is that prepared with 1% sodium chloride, dissolved in 1000 ml (400 g) of barium sulfate. Depending on the age of the child volume of contrast material ranged from 30 to 500 ml. In newborns and infants used water-soluble iodine preparations (verohrafin, urotrast et al.).
Newborn X-ray Hirschsprung disease is difficult because in the early days and weeks after the birth of the difference in diameter ahanhlionarnoyi zone and located above the colon is negligible. In the case of short segment ahanhlionarnoho expansion normally innervovanyh departments and funnel transition in ahanhlionarnu zone appears after 3-4-month life, so in neonates and infants with total ahanhliozom short or narrow areas, this method does not always provide an opportunity to establish the diagnosis. In doubtful cases, widely used functional diagnosis and determine the activity of tissue acetylcholinesterase (AChE) in the mucosa of the rectum.
Whereas in Hirschsprung's disease are malformations of the urinary system, all patients spend urological research before iryhohrafiyi.
Biopsy of the rectal wall, proposed by A. Swenson (1955), to diagnose Hirschsprung's disease. The main goal is to determine the presence of nerve cells musculo-intestinal (auerbahivskoho) plexus in bioptatiyi muscle layer of the rectum. Intramural nerve hanhliyiyi can be reliably detected by staining with hematoxylin and eosin (D.Yu.Kryvchenya and others., 2004). Lack of nerve cells in the specimen indicates Hirschsprung disease. In recent years, the material for rectal biopsy taken during surgery or radical imposition colostomy.
Manometric studies of anorectal area Hirschsprung's disease is based on the detection of inhibitory rektoanalnoho reflex, which is registered by rectal manometriyi. Characteristic signs include lack rektoanalnoho inhibitory reflex and increase the tone of the internal muscle - the latches vidhidnyka by 80-100% increase in the maximum volume of rectum ampulla of more than 100%. These changes are almost odnotypovymy not depend on the age of the child.
Electromyography rectum used to define the distal boundary of the zone ahanhliozu. If Hirschsprung disease amplitude of electrical slow waves in ahanhlionarnomu segment drastically reduced to 0.2-0.3 mV, ie more than 50%. Histochemical studies based PA detection of nerve fibers and determination of AChE activity in the mucosa of the rectum by 1.5-2 cm above the dentate line. In the absence of ganglia in the accumulation of AChE.
Treatment. Hirschsprung's disease treated only surgically. There are two possible surgical treatment:
1) one-stage radical surgery;
2) imposing a temporary colostomy with delayed radical surgery.
Conservative treatment of constipation is carried out to confirm the clinical need for radical surgery.
The majority of leading surgeons in the world, the most defensible tactic is conservative management of the patient for as long as it does not prepare for a radical surgery, and only if unsuccessful conservative therapy resorting to decompressive colostomy (S. Kleinhaus, OI Lonyushkin). The optimum age for radical surgery is 12-18 months. If subcompensated and decompensated disease indicated surgical intervention in these terms. In case of stable disease compensation can postpone surgery until 2-4-years old child. Performing the operation at a later date can lead to complications - dysbiosis, liver, disorders of protein and electrolyte metabolism (ML Kush, GA Bayirov, OI Lonyushkin).
Conservative treatment can postpone surgery until the age when the risk of radical surgery is minimal (YF Isakov).
So far there is no consensus on the need for blending colostomy. It forced surgeons use only in emergency situations: in case of complications which arose after setting siphon enema (perforation of the colon), the acute form of the disease in the newborn, acute decompensation of chronic, severe general condition of the child. Proximity kolostomu impose as distally, just above the transition zone, the intestine with normal neyromiostrukturoyu wall. While blending colostomy biopsy of the colon wall to confirm the diagnosis. While blending colostomy should hem colon to the peritoneum and the lateral fascia to prevent eventration colon near the colostomy in the postoperative period.
Classification of complications of intestinal Stom developed Yu.V.Paschenko (2007):
I. Early complications:
1) swelling of the stoma;
2) bleeding;
3) suppurating wounds;
4) abdominal abscesses in the area of ​​overlay stoma;
5) derived intestinal necrosis (with or without peritonitis);
6) retraction of the stoma (with and without peritonitis);
7) parastomichna eventration;
8) exudative enteropathy;
9) bowel obstruction;
10) Drive perforation of bowel loops.
II. Late complications:
1) perystomalnyy dermatyd;
2) evahinatsiya (free or fixed);
3) stenosis (sub-and uncompensated);
4) hernia (peri-and parastomalni);
5) retraction of the stoma; parastomalni fistula;
6) internal fistulas in the zone overlay stoma;
7) prestomichnyy ileyid;
8) prestomichnyy colitis;
9) mucous metaplasia;
10) syndrome lasted disconnected intestine.
III. Complications of a general nature:
1) sepsis;
2) Urinary tract infection in children with fistulas in the urinary system;
3) overgrowth;
4) depletion;
5) short bowel syndrome.
Perelikovani complications significantly affect the impact of the underlying disease and timing of reconstructive and restorative operations. The greatest number of complications (80.2%) was observed in children who stoma imposed at an early age. It is shown that the most significant changes is deactivated intestine appear in 6-12 months. The expediency of early recovery operations and the need for targeted rehabilitation disconnected intestine - the ozone decontamination saline containing ozone 6-8 mg / l (Yu.V.Paschenko, 2007). The author developed techniques of surgical correction decompensated stenosis and evahinatsiyi using endovideoskopichnyh technologies. Eliminate stoma prolapse is achieved by glue adhesion parietal peritoneum and the visceral surface Drive intestine under laparoscopic control.
Before radical surgery without imposing colostomy great importance to conservative treatment, which was originally conducted in a specialized compartment, and then home. The main objective of it is a regular release from intestinal feces. Assign a diet with foods that enhance peristalsis guts (oat and buckwheat porridge, prunes, beets and carrots, apples, fresh yogurt). It is advisable to carry out massage, exercise therapy, aimed at strengthening the abdominal muscles, breathing exercises. They need to spend 4-5 times a day for 15 minutes, preferably before feeding baby.
The complex conservative therapy must use different types of enemas (cleansing, hypertension, siphons). The structure siphon enemas are 1% solution of sodium chloride, or potassium permanganate furatsillina, petrolatum, solution temperature - 18-20 ° C.
Depending on the age of the child fluid volume ranged from 0.5 to 2 liters in infancy to 5-10 liters in older age. Junk is the use of laxatives. Recommended vegetable oil (CO ¬ nyashnykovu, olive) in young children and 1 spoon channa, preschool - 1 dessert spoon, school 1 tablespoon a day.
Important for preventing complications in the postoperative period and more rapid restoration of normal function of the colon is the definition of its microbial flora. Medicines that contain microorganisms - Representatives of the normal intestinal flora: Bifidobacterium bacterin-forte, bacterin, bifikol, hilak forte, Duphalac. Polyenzyme drugs: Creon, mezim forte, festal, panzinorm forte hepatoprotectors. If there dysbiosis, selective decontamination of conduct that aims to suppress even pathogenic microorganisms, but does not affect the normal microflora. Widely prescribed antibiotics: aminoglycosides, polymyxin B, cephalosporins, nystatin. Two days before surgery patients prescribed liquid food orally - metronidazole 25 mg per 1 kg of body weight per day, siphon enema.
Classical methods of surgery:
1) rektosyhmoyidektomiya (for Swanson - Isaac);
2) pozadurektalne cherezanalne relegation colon (by Duhamel-Bayirovym);
3) endorectal relegation colon without anastomosis (for Soave-Lonyushkinym);
4) endorectal colon relegation down with primary anastomosis (with Voleu);
5) anterior resection of the rectum (by Rehbein) with anal miektomiyeyu.
When subtotal resection of the colon performed relegation abandoned part of the colon on the left or right bryzhevomu sinus, or its reversal by 1800. In total ahanhliozi performed relegation ileum to the replacement of colon and rectum ileotransplantatom intrarektalnym with his erection and restoration of gut permeability overlay entero-enteroanastomozu "end-to-side" with invahinatsiynym valve.
Radical and pathogenetically grounded operation is abdominal-perineal resection constricted (ahanhlionarnoyi) zone with a plot extended colon (Fig. 1).
In the case of short rectal areas ahanhliozu used rectal miotomiyu (submucosal dissection of internal muscle - the latches vidhidnyka and muscle layer of the rectum at 4-5 cm posterior semicircle) or finger extension vidhidnyka (2-5 times at intervals of 1 week).
The basic principle of operation Swenson, Isakov is intraabdominal mobilization of the colon to the level of the internal muscle - vidhidnyka latches, while the rectum to the posterior-lateral surface emit slightly lower (by 1.5-2 cm). Then mobilized intestine evahinuyut the perineum, perform resection and impose oblique anastomosis.
According to the method of Duhamel-Bayirova rectum cross-level transient folds of peritoneum. The lower end is sewn up, and the upper (proximal) bring to the crotch formed by the channel between the sacrum and the rectum to the outside muscle - vidhidnyka latches.
Do resection ahanhlionarnoyi zone and extended parts of the intestine. Rear Pivot semicircle filed intestine to the rectum, and its front part and back part of the rectum impose special vikonchastyy clamp, which is due to aseptic necrosis promotes the formation of seamless anastomosis.
Operation by Soave-Lonyushkinym is to separate sero-muscular layer of the rectal mucosa to the level of the internal muscle - vidhidnyka latches. Colon evahinuyut the perineum, spending by sero-muscular cylinder rectum. Part nyzzvedenoyi rezektuyut intestine, leaving the area of ​​5-7 cm in length, which (excessive part) cut off during the second phase of the operation in 15-20 days and put anastomosis between mucous membranes nyzzvedenoyi and rectum.

Operation Voleu like operations Soave-Lonyushkinym but spend it in one step by the method Voleu-Kryvchenya. Circularly impose nodal sutures (vicryl, maxon, dexon 3/0-4/0) between all layers of the colon and rectal mucosa, forming pozasfinkterno anastomosis, draining mizhfutlyarnyy space anastomosis is replaced in the anal canal. In the lumen of the intestine nyzvedenoyi introduce flexible tube above the anastomosis. Sideways abdominal region sero-muscular Cases filed to serosa pivot colon. Overlay primary anastomosis as possible by hand, so also through circular staplers SPTU and KC-28.

Operation by Rehbein provides intra-abdominal or ekstraperytonealnu resection changed area and most of the rectum with vnutrishnotazovym overlapping direct anastomosis. It is supplemented by internal dissection muscle - the latches vidhidnyka (rectal miotomiya) from the crotch to the anastomosis.
Among these classic surgery is optimal blending of primary colorectal anastomosis, which may be accompanied by muscle dissection and excision of the pouch internal muscle - vidhidnyka latches. In the case of distal forms ahanhliozu perform sfinkterektomiyu by Lynn.
Repeated surgery in case of complications (stenosis) of the posterior sagittal perform access - layered cut soft tissue to the rectum in the midline from the coccyx to the outer muscle - the latches vidhidnyka with mandatory intraoperative electromyographic control muscle structures (A . Pena).
During the postoperative period, the following complications: enterocolitis, failure anastomosis suppuration mizhfutlyarnoho space, local abscess, hematoma mizhfutlyarnoho space anaerobic (klostridialna) infection. All complications leading to peritonitis, with the subsequent development of sepsis. Maybe recurrent constipation, rarely adhesive intestinal obstruction, encopresis varying degree. This is due to functional partial loss of anal control and slower adaptive-compensatory processes (M.I.Yurchenko and others., 2003).
Clinical supervision of a child engaged for 2 - 3 years. The course of conservative therapy, aimed at making the act of defecation reflex involves setting daily enemas at the same time of day, electric, outdoor workout muscle - the latches vidhidnyka, gymnastics These activities spend 15-20 days. With drugs prescribed Neostigmine.
Mortality (according to OI Lonyushkina) is 0.4%, in recent years is zero.