P93 Situs viscerum inversus and apple peel atresia: a rare association. case report

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Abstract

Background and aims

Duodenal atresia is a relatively common anomaly and its frequency ranges from 1 in 3000 to 5000 live births. Association of duodenal atresia with situs viscerum inversus is rare and fewer than 20 patients have been reported in literature.

Methods

We present a sub-Saharan 1 years old female baby, born at 31 weeks with prenatal diagnosis of situs viscerum inversus with dextrocardia, ventricular septal defect, ductus venous agenesis, duodenal atresia and intestinal malrotation. We considered risk factors, clinical and surgical management, complications and evolution of this rare association.

Results

A 31 weeks female baby was born by emergency caesarean section for cardiotocographic changes. Her mother history was positive for pre-gestational diabetes on insulin therapy and for Streptococcus beta-haemolytic infection. Incomplete intrapartum amplicillin prophylaxis was made.

Results

The baby weighed 2300 g and her Apgar scores were 5 and 5, needing non-invasive ventilation with nCPAP.

Results

Because of suspected intestinal obstruction, parental nutrition was started one day after birth and a radiological examination was performed showing the mirror image of a double-bubble configuration of duodenal atresia. She underwent laparotomy at 6 days of life, confirming situs inversus and intestinal necrosis secondary to volvulus on common mesentery (apple peel type 3b): duodeno-cecal anastomosis with saving the ileocecal valve was performed. Histology of the surgical specimen confirmed the transmural necrosis.

Results

Six days after, enteral nutrition was progressively introduced with good tolerance and weight gain; parental nutrition was continued with subsequent cholestasis needing the modification of the lipid composition.

Results

At 3 months of age, she underwent a Bianchi intestinal lengthening procedure and cholecystectomy.

Results

The onset of post-surgical duodenal stenosis two months after required one endoscopic pneumatic dilation (8–10 mm) and a gastrostomy button was placed too. Upper endoscopies performed 1 month and 6 months later were normal.

Results

Enteral and parental nutrition was continued and the oral feeding was gradually introduced.

Conclusions

Duodenal atresia and situs inversus are rare combinations. Outcome is good with appropriate multidisciplinary management and follow-up. Maternal diabetes could be factor risk of laterality, as suggested in literature.

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