Anesthetic Management of a Child With Holt-Oram Syndrome Posted for Deformity Correction of Radial Club Hand: A Challenge

Holt- Oram Syndrome is an Autosomal Dominant disorder characterized by abnormalities in upper limb and congenital heart disease. We present to you an interesting case of a 7 month old child, a diagnosed case of Holt-Oram Syndrome with Acyanotic Heart disease (patent foramen ovale) and left radial club hand deformity. The child was posted for deformity correction under General Anesthesia. Pre-operatively, thorough history taking and investigations were done. After meticulous OT preparation, patient was induced and maintained intra operatively. Goals were directed towards maintenance of hemodynamic stability, maintenance of deeper plane of anesthesia and smooth recovery by providing analgesia in the form of Ultrasound guided nerve block. Our challenges included susceptibility to arrhythmia, hemodynamic instability, tendency for blood loss and chances of cardiac arrest in peri-operative period. Duration of the surgery was roughly 3 hours with signicant blood loss. Transfusion with 70 ml PRBC was done intra-operatively. The child maintained stable vital parameters throughout the surgery and in the post operative period.