Laparoscopic Dismembered Spiral Flap ( Culp - Deweered) Pyeloplasty in 2 months Preterm with duplex kidney

2 years old boy with trisomy 21 was referred to PICU with respiratory distress and multiple Lung consolidations. Child was stabilized and assessed thoroughly which revealed presence of extremely rare bilateral Morgagni’s hernia in the diaphragm. Almost half of thoracic cavity was filled with abdominal contents leading to collapse consolidation of bilateral lung tissues. 20×15 cm2 diaphragmatic defect through which most of colon, right lobe of liver, stomach , omentum and small bowel was found herniating into the chest. The contents were reduced into abdomen, the hernial sac was excised from chest and heart, the diaphragmatic defect was repaired and space was created for the lungs to expand and function properly There is no scar on her abdomen or perineum which is a great cosmetic benefit to the girl child. Fetal volvulous is an extremely rare condition and a challenge to diagnose and manage. Only few cases have been reported in the world literature with instances of fetal death , still births in neglected cases. It’s a complex multidisciplinary approach with state of the art NICU care which helps in survival of potentially morbid and lethal condition.

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