PMC8150421_01
(A) Facial dysmorphism. (B and C) Low set ears with pre auricular tags. (D) Green colored arrow pointing at ano vestibular fistula. (E) Intra operative picture of mobilized rectum. (F) Post operative appearance.
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A day 2 female neonate born to non consanguineously married couple, full term vaginal delivery, with birth weight of 2.5 kg, was noticed to have absent anal opening (Figure 1d) with abnormal passage of stools from the vestibule. On examination; baby had dysmorphism like broad forehead, frontal bossing, hypertelorism, depressed nasal bridge, low set ears with left dysplastic ear having bilateral pre auricular skin tags (Figure 1a-c). When the baby was 6 months old with a weight of 6 kgs, a primary posterior sagittal anorectoplasty (PSARP) (Figure 1e) under general anesthesia was performed uneventfully in prone position having a mechanical ventilator stand by. Baby is now 4 years old, asymptomatic and is on regular follow up with normal stool pattern through the neo anus (Figure 1f).
Contrast enhanced computed tomographic images: (A) At the level of arch of aorta. Yellow straight arrow on right-showing right SVC, Green arrow showing arch of aorta, Red curvilinear arrow showing-pulmonary trunk and Red arrow on left shows Left SVC. (B) Yellow arrow on right shows RSVC, Blue arrow shows right atria, Curvilinear red arrow showing pulmonary trunk and Red arrow on left shows LSVC. (C) Red arrow -Left SVC draining into the coronary sinus. (D) Oblique section showing whole course of left SVC draining into coronary sinus.
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With essentially normal ultrasonographic findings on follow up ; 2D echocardiography was done, showed small patent foramen ovale having left to right shunt and left persistent superior vena cava draining into the coronary sinus; which was confirmed on contrast enhanced computed tomography(CECT)(Figure 2).