Epigastric heteropagus twinning with dextrocardia: a case report

A female infant weighing 4 kg was received on day 2 of life in the outpatient pediatric surgery department with heteropagus twinning. The infant was born after a full-term pregnancy, to an 18-year-old mother (G1, P1), from a consanguineous marriage. The pregnancy was unsupervised, and the infant was delivered via C-section.

The parasite was attached to the autosite at the thoracoepigastric region. The parasite consisted of a head with macrocrania, facial dysmorphia, a misshapen trunk, one ear, one immobile rudimentary leg, and no perineum (Fig. 1A).

Fig. 1figure 1

A Preoperative view, showing parasite attached to the chest wall of the healthy baby. B Barium contrast enema, X-ray showing shared bowel loops

The autosite was healthy and active. The physical examination revealed no abnormalities, and all neonatal reflexes were normal. The hematological and biochemical workup was also within normal limits.

The barium contrast enema (Fig. 1B) revealed mid-abdominal bowel loops predominantly in the healthy twin, and a single loop exhibited in the parasite twin. MRI showed that the brain and spine were unremarkable in the healthy twin. An abnormal corpus callosum, hypoplastic brain parenchyma, and hydrocephalus were seen in the parasite twin. The twins were fused anteriorly at the chest and abdominal cavity. A single large heart was noted in the healthy neonate. The single shared liver was present in the midline, with greater portion in the autosite twin. A single stomach was seen in the parasite twin, while majority of gut loops were in the healthy neonate. There was no connection except vessels connecting the neonates.

2-D color Doppler echocardiography showed dextrocardia with situs inversus. AV concordance was there. Atrial and ventricular septal walls were intact, and the heart showed good biventricular function.

On the 16th day of life, a comprehensive team surgery was performed, including pediatric, vascular, and plastic surgeons. The twins had anatomical connections spanning the skin layers and subcutaneous tissue. The vascular link was through the umbilical vessel. There was no bony connection between the twins. The twins did not share any connection at the chest wall. A release incision was given to separate the nonviable infant, vessels were ligated, and hemostasis was secured. The stomach and gut loops present in the abdominal wall of the parasite baby (Fig. 2) were preserved and transferred back to the healthy neonate. A reverse technique was employed to close the incision on healthy neonate.

Fig. 2figure 2

A and B Intraoperative details showing shared gut loops between twins. C Postoperative healthy twin

The postoperative recovery was uneventful. The baby recovered and was discharged on the 8th postoperative day. A follow-up was done at 3 months over a phone call, according to which the baby was healthy and gaining weight. A later follow-up conducted revealed that the baby passed away due to chronic constipation at 6 months of life.

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