G. Kakkar, A. Murarka, A. Sharma, K. Kulkarni, S. Gupta, A. Sharma, M. Dhaliwal, V. Raghunathan, A. Balasubrmaniam, M. Kapoor, M. Sharma

Amrita Institute of Medical Sciences, Faridabad, India

A 4-year-old boy born presented with craniofacial dysplasia with extreme orbital hypertelorism, corpus callosal dysgenesis, three skull-base defects leading to a nasoethmoidal encephalocoele, a trans-sphenoidal encephalocoele and a nasal lipoma. Single stage facial bipartition surgery and correction of encephaloceles was planned.

The multidisciplinary team including the craniofacial surgeons, paediatric neurosurgeons, paediatric neuro-anaesthetists, and paediatric intensivists grouped together to discuss the perioperative concerns of a an exceptionally challenging case. Preoperative assessment and  routine blood investigations were normal. Despite speech and feeding issues due to the facial defects, a general rapport could be established with the child with the help of audio visual aids. Airway examination revealed complete distortion of the airway anatomy due to the presence of basal encephaloceles and extreme hypertelorism. A good mouth opening was the only reassuring finding. There were no red-flag airway signs like resting or positional stridor. A CT reconstructed 3D model was used for airway and surgical planning. After extensive multidisciplinary counselling, the patient was taken up for surgery. On the surgical day the child was playful and aided by play therapy, he was shifted to the operating room where full AAGBI recommended monitoring was instituted. A gas induction was performed using a large size 3 adult facial mask considering the large cranio-facial defect. Prior to intubation, a delicate finger sweep was required to safeguard the encephaloceles in-order to make room for the laryngoscope blade. Airway was secured with a size 4.5 cuffed reinforced endotracheal tube using CMAC videolaryngoscope. Central venous access and arterial lines were secured and a cell-saver was primed for autologous blood salvage and transfusion. The surgical encephalocoele, hypertelorism correction and nasal reconstruction took 16 hours. Significant blood loss required transfusion of two packed cells and 950 ml of cell-salvaged blood. The patient was shifted to the paediatric intensive care (PICU) with a planned 48hr ventilation strategy. Sedation hold on day3 revealed impaired neurological recovery and concerns of left optic nerve injury due to orbital wall reconstruction. MRI brain revealed a small thalamic infarct probably due to the encephaolocele replacement in the cranial cavity. Considering the neurological deficits, a bedside tracheostomy was performed and the patient was weaned by the 6th post-operative day. Nutritional support was established using an orogastric tube. Aggressive neurological-rehabilitation helped to correct the left sided hemiparesis. At 1 month, there was a significant improvement and the child could walk with support. At 3 months follow-up, there was near complete resolution of the hemiparesis with full visual acuity and at 6 months the child had a mRS<2 and had started to attend a local playschool.  The parents were partners in the rehabilitation journey of this child and played a significant role in his physical and social recovery.




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