Median arcuate ligament syndrom (MALS-syndrom): clinical presentation and the challenges of diagnosis in a teenager

Abstract

Celiac trunk compression syndrome (MALS-syndrome) occurs due to compression of the abdominal trunk of the aorta by the median arcuate ligament of the diaphragm or its internal legs, and is characterized by varying degrees of abdominal pain, weight loss, some patients have symptoms of autonomic dysfunction and transient unconjugated hyperbilirubinemia. The frequency of anomalies in the structure of the aortic orifice of the diaphragm is 10–24%, however, the clinical symptoms of MALS-syndrome are observed only in 0.4–1.0% of cases, most often in patients of adolescent and young age, mainly female with asthenic physique.

In the article was presented clinical case of a girl with compression syndrome of the abdominal aortic trunk hospitalized in the older childhood department of the SI «Institute of Pediatrics, Obstetrics and Gynecology named of academician O.M. Lukyanova NAMS of Ukraine», with complaints of febrile temperature, severe abdominal pain for a long time, resistant to analgesic and spasmolytic drugs, episodes of diarrhea, constipation, nausea, vomiting, dizziness, fainting, tachycardia, weight loss. From the anamnesis known that the child was repeatedly examined in clinics at the place of residence, chronic gastroduodenitis and autonomic dysfunction syndrome was diagnosing. The pathogenetic and symptomatic therapy which was appointed showed no significant effect. Due to the attacks of paroxysmal tachycardia, radiofrequency catheter ablation was planned. In hospitalization, a condition of moderate severity, pallor, asthenization, adynamism, severe abdominal pain, dyspeptic syndrome, tachycardia were revealed. At examination general clinical, biochemical parameters were within physiological norm. According to fibroesophagogastroscopy, signs of reflux esophagitis, erythematous gastropathy, duodenogastric reflux of II degree were found. According to the ultrasound scan, pathological changes were not observed. Hydrosonography of the upper digestive tract showed signs of pronounced duodenostasis, it was impossible to exclude dynamic upper intestinal obstruction; signs of reflux esophagitis, gastroduodenopathy, gastroptosis. Due to severe abdominal pain syndrome, weight loss, persistence of constipation, partial intestinal obstruction CT of the abdominal cavity and pelvis was carries out resulting in revealing a compression of the abdominal trunk by the middle arcuate ligament of the diaphragm, the caliber of the vessel in the area of the mouth gauge was 3 mm, the distal caliber reached 6 mm. After clinical and paraclinical examinations, the child was transferred for consultation to surgeon and laparoscopic decompression of the abdominal aortic trunk was performed. The girl was examined 1.5 months after surgery, the condition of the child improved significantly: abdominal pain, dyspeptic syndromes decreased, tachycardia attacks regressed, and appetite improved. The girl continues to adhere to dietary recommendations, observed by pediatrician, pediatric surgeon.

Conclusions. MALS-syndrome must be included in the diagnostic search algorithm in the presence of abdominal pain syndrome of unknown origin with severe and uncontrolled disorders of the autonomic nervous system.