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Dysphagy at neuromuscular pathology
The dysphagy is caused by many system neurologic and muscular frustration listed in tab. 12-5. In detail it is told about them in other sections of this manual (see the index).
Table 12-5. The neuromuscular frustration causing a dysphagy
Cerebral palsy (especially often) Dermatomyositis
Infections — diphtheria, poliomyelitis, tetanus
Muscular dystrophy (especially often)
Riley's syndrome — Dai
Some defects of cranial nerves of Bolezn Verdiiga — Goffmanna
The spasm of a perstneglotochny muscle, or achalasia of an upper esophageal sphincter, causes an intermittlruyushchy dysphagy. Supertension in a throat and an upper part of a gullet can lead to formation of a diverticulum of a back wall of a throat. The diagnosis is made by means of X-ray cinematography or a manometriya (inability of an upper esophageal sphincter to relax at the time of swallowing is visible). Good results are yielded by a myotomy. similar to that which is carried out at a hypertrophic pyloric stenosis (section 12.23).
Cricopharingeal diskoordination at babies
Diskoordination of reductions of perstneglotochny muscles is usually shown soon after the birth. The act of suction passes normally, but swallowing often is followed by a poperkhivaniye or aspiration of milk. Such children have jaws of the small sizes and companies badly opens. On X-ray records in back part of a throat the repeated movements of contrast weight forward - are visible back. Symptoms usually disappear to 6-month age, and till that time of the child it is necessary to feed very carefully from a spoon or through a probe. The reason of this frustration is unknown.
Bulbar paralysis (defeat of a nadjyaderny zone or motor neurons) can cause a dysphagy. Suction is broken, the child hardly chews and swallows of dense food, jaws are convulsively squeezed; along with a dysphagy there are other symptoms of the central spastic paralysis. Defeat of motor neurons with development of sluggish bulbar paralysis and a bilateral plegiya of mimic muscles indicates Möbius's syndrome.
Paralysis of an upper guttural nerve
This state at newborns is shown by a dysphagy, weakening of a vermicular movement of a gullet, a characteristic pose (the head is always turned in one party), unilateral weakness of mimic muscles. Believe that this syndrome is caused by the unusual pre-natal provision of a fruit at which the specified nerve is squeezed between a thyroid cartilage and a hypoglossal bone. Spontaneous recovery happens within the first year of life.
Passing dysfunction of muscles of a throat
Dysfunctions of muscles of a throat and soft palate often proceed combined; they are caused, apparently, by a delay of normal development or a cerebral palsy. The main symptoms — a poperkhivaniye during food and hypersalivation. At X-ray cinema research define paralysis of constrictors of a throat and weakness of a soft palate. The most terrible complication — aspiration — can be prevented feeding through a probe within several days or weeks. With growth of the child emergence of other dysfunctions of a nervous system is possible.
The achalasia — inability of the lower esophageal sphincter to relaxation at the time of swallowing — causes relative impassability which is aggravated with lack of peristaltic waves in a gullet (fig. 12-3,6). The achalasia of a gullet occurs mainly at adults, children till 4 flyings make less than 5% of all patients. Achalasia cases at sibs are noted. The amount of neurons in gangliya is often reduced; they are surrounded with inflammatory cells. The increased reaction of muscles of a gullet to metasincaline is regarded as the proof of the hypersensitivity caused by denervation, however the actual reason of this state is established only at a Chagas disease.
Clinical manifestations and diagnosis. The complicated swallowing, vomiting by food, the cough caused by flowing of liquid food in a trachea, the slowed-down body weight increase belong to the main symptoms. The diagnosis is made by means of a X-ray analysis; in pictures narrowing of the cardia and lack of peristaltic waves in a gullet are visible. At long narrowing of the cardia there is a considerable esophagectasia so on the roentgenograms made in vertical position of the patient liquid level is visible. Owing to constant flowing of contents of a gullet in respiratory tracts there are inflammatory processes in lungs, up to a bronchietasia. The long delay of liquid and food in a gullet leads to development of an esophagitis.
Fig. 12 — 6. Types of hernias of an esophageal opening of a diaphragm. A. The sliding hernia, the most widespread type. B. Paraezofagealyiaya hernia.
Treatment. Acute symptoms can be removed temporarily, having expanded narrowing by means of the esophagoscope or a mercury buzh, however surgical correction, namely a section of muscles of an expanded segment of a gullet and cardial part of a stomach (operation of Haler) gives lasting effect. Unfortunately, this operation, liquidating narrowing, leads to throwing of contents from a stomach in a gullet, to a secondary esophagitis and, in rare instances, to formation of a stricture. Sometimes the positive resistant take at children of more advanced age is received at careful dilatation by means of the pneumatic cylinder placed in the area of narrowing under radiological control.
Hernia of an esophageal opening of a diaphragm
Protrusion of part of a stomach in a thorax through an esophageal opening of a diaphragm at one patients happens paraezofagealno, at others — to involvement of a gullet (fig. 12-6). At the first option the cardia is located normally, and the part of a stomach leaves in a thorax through an expanded esophageal opening of a diaphragm. Typical symptoms — weight after food and pain in an upper half of a stomach; rare complication is the heart attack of the dropping-out part of a stomach.
At the second option the cardia and part of a stomach are in a thorax. This state usually inborn is also often combined with a secondary gastroesophagal reflux. Also other congenital anomalies are at the same time observed. There are data on the genetic nature of this illness. Remains not clear whether the hernia of an esophageal opening of a diaphragm which is often found at adults is the acquired illness or late display of inborn pathology. Treatment is directed not to elimination of hernia, and to prevention of a gastroesophagal reflux.
Insufficiency of the lower esophageal sphincter leads to excessive throwing of gastric contents in a gullet that can be shown by the expressed symptomatology. For designation of this state in the USA often use the term "halaziya" while in Europe "the chest stomach" or "phrenic hernia" prefer to speak.
Etiology. The reasons of a gastroesophagal reflux are various at children and adults. At children the reflux is often connected with diafragmalpy hernia. At the adults suffering from a reflux, the tone of the lower esophageal sphincter is constantly lowered, at children the picture is much less clear. Switching function of the lower esophageal sphincter is supported, apparently, by several factors, and some of them play more important role at children's age. So, in addition to a tone at children intra belly localization of a zone of supertension is of great importance that favors to function of a sphincter. Along with it a part is played a skladchatost of a mucous membrane in this zone and a corner under which the gullet connects to a stomach.
Clinical manifestations. The symptomatology directly depends on extent of impact of gastric contents on a mucous membrane of a gullet. At 85% of patients plentiful vomiting arises within the first week of life, and at 10% of patients it develops during 6 weeks. To 2-year age when the child considerable part of days is in vertical position and eats firm food, clinical manifestations disappear without treatment in 60% of cases (tab. 12-6). At other children symptoms remain at least till 4 flyings.
Table 12-6. Dynamics of symptoms of a gastroesophagal reflux at uncured patients (from: Carre K. J. — Arch. Dis. Child., 1959, 34:344)
Symptoms at the 3rd age (98%)
Disappearance of symptoms by 2 years
60 — 65%
lack of improvement at reception of firm food, existence of symptoms at children is more senior than 4 years, lack of strictures
Lethal outcome (aspiration and insufficiency of food)
Severe vomitings at a reflux are caused by the reflex pylorospasm caused by irritation of a gullet. Every third child with this illness at chest age has an aspiratsionpy pneumonia; at more advanced age chronic cough, the complicated breath and recurrent pneumonia are often observed. Among other symptoms it is necessary to call a rumination (see below). Approximately in 2/3 cases growth and body weight increase slowly owing to insufficient intake of nutrients which is caused by persistent vomitings. The most terrible complication of an esophagitis — esophageal bleeding which is shown by a hematemesis or a melena. At 25% of patients the iron deficiency anemia develops, and the blood loss which caused it often has the hidden character. Retrosternal pains are rare, but in far come cases because of a dysphagy the child refuses food. Occasionally progressing of an esophagitis leads to development of a stricture.
Diagnosis. In mild cases the careful assessment of clinical symptoms is sufficient for statement of the correct diagnosis which is confirmed by efficiency of adequate treatment. In hard difficult cases the diagnosis is made on the basis of X-ray contrast research. Detection of folds of a stomach over a diaphragm — one of cardinal symptoms of phrenic hernia (fig. 12-7). It is easier to find these folds in children at the fallen-down, blank gullet. As the gastroesophagal reflux arises incidentally, even considerable throwing does not manage to be recorded on roentgenograms in 10% of cases. In such situation the correct diagnosis can be made at repeated researches or by detection of an acid gastric juice by means of the rn-meter sensor placed in a gullet. The dose of a baric suspension on volume has to correspond to food volume at one time. The patient inspect in situation with the hung head when pressing a pas a front abdominal wall.
The small reflux is possible at each child, but at the same time the gullet is quickly cleared of gastric contents. The repeated reflux at the child is more senior than 6 weeks — the pathological phenomenon. The stricture can be found easily at X-ray contrast research. The heavy esophagitis can be suspected at a X-ray analysis, having seen the rough jagged contour of a mucous membrane, but as the main diagnostic method it is necessary to recognize an ezofagoskopiya.
Treatment. Medical actions at a reflux are usually rather effective. Results of medicinal therapy it is better at babies, than at children of more advanced age. In mild uncomplicated cases it is enough to keep the child in vertical position in time and within an hour after feeding and to try not to provoke vomiting. In hard cases the child has to be in forced situation round the clock. If it is in a semi-sitting position or lies on spin, the head end of a bed has to be lifted approximately on 50 °; if it lies facedown, rise on 30 ° suffices (fig. 12-8). Often the use of more dense food helps. At an esophagitis it is recommended to give antacids in intervals between feedings.
Fig. 12-7. Ezofagogramma showing a gastroesophagal reflux. The stricture is caused by a peptic esophagitis. Longitudinal folds of a stomach over a diaphragm indicate the accompanying hernia of an esophageal opening of a diaphragm.
Fig. 12-8. Position of the patient at treatment of a gastroesophagal reflux. The child is stacked it obyuazy. that between legs there was a ledge wrapped by a soft tissue, and fix in this situation.
Even at intensive treatment of a positive effect sometimes it is possible to reach only in 2 weeks; often first sign of improvement is the body weight increase. In one research it is shown what betanekhol in a dose of 8,7 mg / (sq.m of days), appointed in 3 receptions to food, reduces vomiting and promotes increase in body weight.
If during 6 weeks intensive medicamentous care does not yield any result, then surgical intervention is shown. At repeated aspirations and threat of an apnoea it is necessary to accelerate the solution of a question of operation. Existence of a stricture at a reflux - an esophagitis serves as the indication to operation even without attempt of position therapy. Bougieurage of a stricture eliminates for a short time dysphagy signs, but if the reflux is not liquidated, then the stricture recurs. If it was succeeded to cope with a reflux, then repeated bougieurage usually is not required. In pediatric practice fundoplication is most widespread on Nissen or her options. Positive takes receive more than in 90% of cases. At considerable shortening of a gullet intrathoracic operation on Nissen is more preferable. In certain cases the stricture is expressed so sharply that the esophagoplasty a colic transplant is necessary.
Rumination — a rare, but dangerous form of chronic vomiting. It can cause a growth inhibition, especially in the second half of the first year of life. Its etiology is unknown. At some patients an important role is played by a psychological factor. The relations between mother and the child are often broken that is caused generally by inability of mother adequately to carry out the parent functions. Believe that a rumination — a special type of the repeating self-stimulation by means of which the baby compensates shortage of the corresponding external stimuluses. In one cases such children a long time are deprived of the calming tactile, visual or acoustical feelings. In others gullet dysfunction, especially heavy gastroesophagal reflux is the cornerstone of a rumination. At most of patients dysfunction of a gullet at least contributes to development of a rumination. The chewing movements, continence in a mouth of fingers or regurgitirovanny gastric contents often precedes a rumination or accompanies it. Attentively watching the child, it is possible to see that it causes an emetic reflex fingers or language. X-ray inspection with barium reveals an easy reflux or phrenic hernia, and also helps to exclude other pathology: gullet stricture, achalasia or ulcer of a duodenum.
Treatment. When there are no cordial relations between mother and the child, it is necessary to put a maximum of efforts that to recover them. Regular visual contact often leads to reduction of regurgitation. At a gastroesophagal reflux position therapy is shown. If the state does not improve, it is necessary to resort to surgical treatment of a gastroesophagal reflux which usually eliminates a rumination and promotes increase in body weight.
Peptic esophagitis. A peptic esophagitis with pains, a loss of blood and in certain cases with a gullet stricture — the most frequent form of an esophagitis. This illness is caused by throwing of gastric contents in a gullet.
Retroezofagealny abscess usually arises owing to distribution of retropharyngeal abscess down in retroezofagealn space. Besides, perforation of a gullet, foreign bodys, pleurisy, a pericardis, backbone osteomyelitis, a trophic ulcer after an intubation or from a tracheostomy tube, diphtheria of a throat and purulent lymphadenitis of a mediastinum can be its cause. Abscess forms behind or around a gullet, often displaces it aside, squeezing at the same time more motionless trachea.
Abscess symptoms — short wind, cough with a metal shade, a dysphagy, and at trachea shift forward — neck hypostasis. The palpation of a neck can be painful, hypodermic emphysema is sometimes observed. On the lateral roentgenogram the expanded retrotracheal space is visible even without use of a contrast agent. If abscess arose owing to perforation of a gullet, then X-ray contrast research is contraindicated.
Forecast. Abscess can break in a pleural cavity, a trachea or lungs. The death can occur owing to a trachea prelum with posledstvuyushchy asphyxia or erosion of a large vessel and massive bleeding.
Treatment. The urgent drainage of abscess is shown. If it is located highly, then access to retroezofagealny space opens from a neck on a first line of a grudinoklyuchichno-mastoidal muscle. From this access it is possible to reach the level 4 of a cervical vertebra. The back mediastinotomy is necessary for opening of the abscess located below this level. At the same time appoint the corresponding antibiotics, but it must be kept in mind that an antibioticotherapia can disguise the accruing mediastinal infection and that only repeated lateral roentgenograms of a neck and thorax allow to assess a situation correctly.
The gullet moniliasis usually arises yl of the patients receiving chemotherapy concerning a hematologic or oncological disease. The oral cavity at the same time can be an intaktna. Pain and the complicated swallowing are expressed. On roentgenograms the rough relief of a mucous membrane or multiple roundish defects of filling are defined. At an ezofagoskopiya the friable bleeding mucous membrane with superficially lying whitish plaques is visible. Treatment: nystatin orally on 200 000 PIECES each 2 h or Amphotericinum In parenterally. Other antibiotics should be cancelled whenever possible. The forecast is defined by character of a basic disease.
Diphtheritic defeat of a stomatopharynx can extend to a gullet. Treatment is same, as at diphtheria.
Tubercular damage of a gullet in rare instances arises upon transition of process from a throat or adjacent lymph nodes.
The causative agent of a herpes simplex can cause an acute esophagitis. At this disease temperature is increased, and swallowing is followed by such severe pains that meal inside is completely excluded. At survey of a throat typical vesicles are visible, and at an ezofagoskopiya similar elements are observed in a gullet. The illness lasts, as a rule, several days. The relief is given by intake of 2% of solution of lidocaine (2 — 3 ml) each 4 g In hard cases it is possible to use arabinozid adenine.
Korrozivny esophagitis. The most frequent reason of a korrozivny esophagitis from the outcome in a stricture — impact on a gullet of household chemical drugs. Usually "responsible" are the cleaning drugs which part hydrochloric and sulfuric acids, chloric lime or strong alkalis are. Availability of the specified drugs to the child, and also corrosive burns on hands, serve as weighty indirect confirmations that the child drank caustic substance in a mouth or other parts of a body. The acute period with hypostases and a dysphagy proceeds 2 — 4 weeks. Then there comes the asymptomatic period lasting several weeks during which strictures gradually form. They conduct to the esophageal stenosis which is shown a dysphagy and vomitings.
Treatment. Prevention — the only effective type of treatment. Parents have to be informed on danger of many household drugs and watch that caustic substances were stored in the place, unavailable to the child. Urgent actions include reception of a large amount of water for washing of a gullet and neutralization of chemicals. The gastric lavage is contraindicated. At throat hypostasis sometimes it is necessary to resort to a tracheostomy. Ezofagoskopiya should be led during the first 48 h to define existence and weight of corrosive burns as lack of changes on a mucous membrane of a mouth or a throat does not exclude damage of a gullet. In rare instances caustic substance can get into a stomach, almost without having had the damaging effect on a gullet and to cause heavy gastritis with perforation or a late stricture. If burns are not found, then it is optional to continue medical actions. In the presence of burns appoint ampicillin and prednizol on 2 mg/(kg-days) in stages within 10 days. Prednisolonum, apparently, reduces probability of cicatricial narrowings. Early detection and dilatation of the forming stricture — an important stage of long maintaining such patients. In some cases there comes the full obliteration of a gullet or so dense hems are formed that dilatation becomes impossible. In such situation the struck piece of a gullet is substituted with a fragment of the large intestine or a tube created from a stomach.
Perforation of a gullet. The gullet is most often perforated when carrying out tool researches. Also spontaneous gap at sharp increase of intra esophageal pressure is possible, for example owing to severe vomiting, during road accident and even at a prelum in a parturient canal. At 95 ° / about Children the perforative opening is formed on the left side of distal part of a gullet, however at newborns the right side is perforated. The leading symptoms — vomiting which the strongest retrosternal pain, cyanosis and shock follow. Great diagnostic value has detection on an ezofagogramma of signs of penetration of a water-soluble contrast agent out of gullet gleam limits.
Mallori's syndrome — Weiss. Strong tension at vomiting can cause a rupture of a mucous membrane and a submucosal layer of a gullet that is shown by a hematemesis (Mallori's syndrome — Weiss). From other, more serious reasons of bleeding from an upper part of a digestive tract it is necessary to apply an ezofagoskopiya to difference of this state. At Mallori's syndrome — Weiss at children there comes spontaneous recovery therefore medical actions are reduced to a diet and hemotransfusion.
Gullet varicosity. The varicosity of a gullet arises at children as complication of portal hypertensia. The main signs — repeated profuse vomitings scarlet blood, a tar-like chair and reduction of volume of the circulating blood. At children with a gullet varicosity more than in 50% of cases gastrointestinal bleeding arises out of a gullet. The diagnosis is made by means of X-ray contrast research (having found expanded veins in pictures); endoscopic diagnosis yields more exact results. Treatment of portal hypertensia and acute bleeding from a digestive tract is covered in section 12.102.
Foreign bodys of a gullet. Some of the objects swallowed by children pass on a digestive tract without what - or complications. In some cases foreign bodys get stuck in a gullet in the field of one of three physiological narrowings: below a perstneglotochpy muscle, at the level of an aortic arch and over a diaphragm. The delay of a foreign body in other place indicates existence of some pathology of a gullet.
Clinical manifestations. The foreign body can cause a fit of coughing or asthma. Usually there is pain, a dysphagy (especially after reception of dense food), and also an asthma owing to a throat prelum. After the initial asymptomatic stage the hypostasis and an inflammation causing impassability of a gullet develop. Perforation of a gullet is followed by pains, fever and shock.
Diagnosis. X-ray contrast foreign bodys come to light very easily. Flat objects (for example, coins) are better visible in a profile in lateral pictures. Identification of plastic or glass objects is often complicated, but they can be found, having given to the patient several drinks of baric weight. To define the provision of a foreign body, it is optional to use the cotton wool moistened with liquid sulfate of barium; it only complicates
Treatment. The foreign body is deleted by means of the esophagoscope under sight control. Just before procedure it is necessary to make a repeated picture to be convinced that the foreign body did not pass in a stomach and did not leave with an emetic masses. For removal of flat objects (for example, coins) use the following technique: under roentgenoscopic control Foley's catheter cylinder is got for a foreign body, then the cylinder is inflated and extended for a catheter outside together with a foreign body, paying attention that it did not get into airways. It is impossible to try to push a foreign body in a stomach at all. After removal of a subject it is necessary to watch the patient within 1 days to reveal signs of possible perforation or impassability.