1 - Department of Pediatric Surgery, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa
- IX Congresso Ibero-Americano de Cirurgia Pediátrica
Introduction: Superior Mesenteric Artery (SMA) syndrome is rare in pediatric patients. It is characterized by loss of angle between the SMA and descending Aorta following acute weight loss with subsequent extrinsic compression of the third duodenal limb. Related conditions include eating disorders, trauma, burns, tumors and anatomical variations of Treitz ligament. The clinical picture is usually one of proctrated inespecific epigastric pain, vomits and weight loss.
Clinical case: A sixteen-year-old boy with Joubert syndrome was referred to our emergency room for intestinal occlusion. He had an history of weight loss (30% of body weight in 8 months), no previous surgeries. He presented with vomits described as fecal for 16 hours with no fever or diarrhea. On admission he was hypotensive, tachycardic, with fever and a low level of oxygen saturation; he was pale, with fecal nasogastric drainage and diminished lung sounds on auscultation. The abdomen was tense, with no bowel sounds, tender, with no palpable masses. Laboratory evaluation revealed leukocytosis, negative C Reactive Protein and elevation of blood creatinine, urea, sodium and chloride levels. On blood gas samples pH was 7.3, HCO3 18 mmol/L, lactates 9.8 mmol/L and glucose 243 mg/dL. Hypernatremic dehydration with acute prerenal kidney injury (AKI) was assumed. Abdominal Computed Tomography revealed dilation of stomach and duodenum proximal to the 3rd portion, pointing to a likely mechanical obstruction. Exploratory laparoscopy was made, with massive gastric and duodenal dilation and transmural necrosis of the anterior gastric wall, due to compression of the third portion of duodenum by SMA. Strong procedure was performed with segmental gastrectomy.
Discussion: In this clinical case SMA syndrome presented acutely, with massive gastric dilation and necrosis, an unusual presenting form. Confounding factors for the diagnosis were the patient inability to communicate and the absence of accompanying relatives; fecal vomits, without other signs of low obstruction, in “conflict” with CT suggesting D3 as the obstruction point; AKI precluded contrast use on CT scan compromising anatomical vascular evaluation. Although imaging studies can point towards a diagnosis, the surgeon may need to proceed for exploratory laparotomy when faced with a critically ill patient with inconclusive investigation.
Palavras Chave: superior mesenteric artery syndrome