Background and aims An adolescent presenting with bilateral tibia and fibula amelia, single kidney, hip dysplasia and severe kyphoscoliosis with abdominal muscle weakness causing femoral hernia is an anaesthetic challenge.
Methods An 18 year old girl, weighing 20 kg, height 80 cm, presented with left femoral hernia. Since birth she had bilateral tibial and fibular amelia, hip dysplasia, absent left kidney, external ear malformation, polydactyly and syndactyly. Presently she had severe kyphoscoliosis and mild restrictive lung disease with distorted anatomy of hip and surgical site. We avoided central neuraxial block in view of severe kyphoscoliosis, absence of lower limb with hip dysplasia and short stature. We planned general anesthesia and ultrasound guided TAP block. Patient was induced with inj Fentanyl 40mcg and inj Propofol 80 mg. Supreme LMA no. 3 was inserted. Muscle relaxant was avoided. Ultrasound guided TAP block was given with 0.25% Ropivacaine 20 ml on left side. Despite the anterior superior iliac spine being placed laterally and small in size, the internal oblique, external oblique and transversus abdominis appeared with similar thickness. Internal oblique was not larger than other two muscles. This could be attributed to her inability to walk and hip dysplasia. Procedure was uneventful.
Results Good postoperative analgesia for 20 hrs with TAP block, decreasing the need of opioid consumption in the postoperative period.
Conclusions Undiagnosed malformation syndrome put forth multisystem management challenges. Ultrasound guided block are a suitable aid in cases with malformation and distorted anatomy to facilitate surgical and postoperative analgesia.
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