Ledda syndrome: symptoms, treatment
A painful condition associated with an abnormal position of the duodenum, blind and midgut is called Ledd's syndrome. On what grounds determine the presence in the body of this pathology and what methods are guided in its treatment - we will deal with these issues in our article.
An anomaly of bowel turn which is formed during fetal development( usually at 10-12th week) and is constriction of the duodenum with a caecum with a turn in the middle gut, has a specific name - Ledd's syndrome.
ICD-10 is a generally accepted classification of medical diagnosis coding developed by WHO.In accordance with this International Classification of Diseases of the 10th revision, Ledd's syndrome refers to diseases of the digestive system( class XI).The pathological condition is found no more often than in one case for 500 newborns, and boys are twice as likely to be diagnosed as to girls.
As a rule, the diagnosis is made in the first week of life of a small patient, a little less often - in the first month of life. To conduct a correct diagnosis, a pediatrician needs experience and a wide knowledge of pathology. In this regard, the disease is not always detected in a timely manner. The most obvious sign of pathology in the first days of life is vomiting with an admixture of bile.
Unfortunately, the severity of the condition of a patient with a similar disease may be exacerbated by concomitant congenital malformations, such as:
- , kidney or lung hypoplasia;
- heart disease;
- pulmonary artery stenosis, etc.
Leld's syndrome in adults is extremely rare. Intestinal obstruction can result from the mobility of the cecum, as well as the adhesive process in the abdominal cavity. Sometimes the formation of pathology is provoked by a hernia of the anterior abdominal wall. Not the last place among the factors influencing formation of a morbid state, benign and malignant tumors of various departments of an intestine play.
Symptoms of the disease
Ledd's syndrome in newborns may show unstable symptoms. In addition to vomiting and regurgitation of bile, infants occasionally experience abdominal pain. In some cases, relapses occur leading to acute intestinal obstruction with worsening, up to the collapoid state of the child.
General symptomatology of the syndrome:
- paroxysmal pain;
- stool retention, accumulation of gases;
- bloating of the epigastric region;
- abdomen lower abdomen;
- palpation does not provide complete information about the patient's condition.
Radiography of the abdominal cavity reveals two levels of fluid in the stomach and duodenum, as well as a small amount of gas in the intestine. If barium sulfate was used during the procedure, the result indicates the accumulation of contrast medium in the stretched stomach and duodenum. The contrast is evenly distributed over the loops of the small intestine.
Irrigography usually reveals the fixation of the caecum under the liver. But with the help of computed tomography, the mid-gum turns around the mesenteric artery.
Ledda syndrome is a pathology that requires immediate surgical intervention. As noted earlier, the symptoms of the disease are fickle and can be repeated at intervals of several days, weeks, or even years.
In a number of cases, the painful condition does not make itself felt for many years and is asymptomatic. Children at the same time suffer from a decreased appetite, lag behind in physical development.
In addition to classical Ledd's syndrome, two additional forms are distinguished:
- In the first case, there is no inversion of the midgut, but an abnormal position of the caecum is observed, which squeezes the duodenum.
- In the second case, there is only an isolated turn of the middle gut.
The course of pathology( Ledda syndrome) can be acute and subacute( chronic).In chronic form, there are not pronounced abdominal pains, like intestinal colic, sometimes vomiting, hypotrophy. There is a broken stool. The condition of the child is characterized as satisfactory. Involvement of the intestine does not lead to a violation of its blood supply. In the veins of the small intestine, only blood stagnation occurs and in the gastrointestinal tract there are jet changes.
Acute inversion of the intestine is a more serious condition, which, if not treated, leads to necrosis or gangrene of the intestine.
Treatment of Ladd's syndrome
The use of antispasmodics helps to improve the general condition of a patient with a chronic-recurrent course of the disease. However, this is only a temporary measure. The main technique in treating intestinal obstruction is nevertheless surgical intervention. The surgical treatment of pathology( Ledda syndrome) consists of several stages:
- First, vomiting and obstruction are eliminated.
- Then the small intestine is located in the right abdomen, thick - in the left.
- If there is a need for fixation of the intestinal loops within the parietal peritoneum, perform this manipulation. A similar measure is required if the patient has abnormal motility of the intestine.
- Appendectomy is performed.
Laparoscopic methods of diagnosis and treatment of the syndrome have recently become widespread in medicine. Such mini-invasive technologies are highly effective. However, performing a surgical procedure in this way is associated with some difficulties due to a small amount of the abdominal cavity, as well as a small thickness of the abdominal wall in children of the first months of life. Sometimes in the early postoperative period there are complications:
- intestinal bleeding;
- intestinal obstruction.
The chances for a full recovery will depend on the results of the operation performed. If all the departments of the gastrointestinal tract are preserved, the prognosis for a high level of quality of life is favorable. If there were significant intestinal resections that led to the syndrome of "short bowel", the patient has problems associated with eating and hypotrophy. With such results of surgical treatment, multiple hospitalizations are carried out to carry out parenteral nutrition. Sometimes patients will have to repeat operations. If the intestinal obstruction developed against cystic fibrosis, the prognosis for recovery is unfavorable.
Preoperative preparation of
Once a newborn is diagnosed with intestinal obstruction( Ledda syndrome), it is transferred to a surgical hospital, injected with a nasogastric tube and ensures a constant outflow of stomach contents. The time that is necessary to conduct preparatory preoperative measures, directly depends on the severity of congenital intestinal obstruction.
If a small patient is suspected to have a bowel turn, the following diagnostic measures are performed:
- blood test for the group and Rh factor;
- blood test for hemoglobin and hematocrit;
- blood coagulation assay.
Operative treatment is performed urgently, preoperative preparation takes a maximum of one hour. The child is given an infusion therapy, prescribes hemostatic drugs, pain medications, and sometimes artificial ventilation. A catheter is inserted into the central vein.
In the case of low intestinal obstruction, preoperative preparation may take up to 24 hours. During this time the newborn is additionally examined for the purpose of possible detection of accompanying pathologies. In this case, the drainage of the stomach is performed, the volumes of withdrawn masses are taken into account, the infusion therapy, antibiotics and haemostatic preparations are prescribed. Feeding a newborn is excluded.
The majority of children in the postoperative period undergo prolonged artificial ventilation, which can last up to five days. Antibiotic therapy uses drugs that are active against anaerobic bacteria. Microecological status is monitored at least twice a week. A day after the operation, drugs are prescribed to improve intestinal peristalsis, as well as biopreparations.