Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

Background Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. Case presentation We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. Conclusion We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

Combating Airway Issues with Elective Use of an I Gel in a Child with Goldenhar Syndrome

Introduction: Goldenhar syndrome is an oculoauriculovertebral spectrum attributed to the developmental anomalies of the first and second brachial arches. Its typical presentation in children with hemifacial microsomia poses a dual challenge for the anaesthesiologist on account of difficult airway often compounded with systemic abnormalities. Case report: We describe a case report wherein a 5 year female presented to the oculoplastic clinic of our hospital for surgical removal of limbal dermoid under general anaesthesia. Airway examination revealed classical facial asymmetry with underdevelopment of jaw bone coupled with protruding incisors. A predicted difficult airway, more so in a child led us to choose an anaesthesia technique with preservation of spontaneous breathing and planned use of supraglottic device in the form of an I Gel for airway management. Conclusion: The aim of this case report is to highlight the anaesthetic implications of this not so uncommon entity presenting to ophthalmology and ENT clinics. A thorough preoperative assessment, adequate preparedness and alternative plans are keys for successful airway management in such syndromic children. Keywords: Goldenhar syndrome, hemifacial microsomia, I Gel

The importance of a lifeboat-median artery forearm flap in Goldenhar Syndrome

We present a case of a 32-year-old male with left-sided Goldenhar Syndrome and delta phalanx of the thumb, who was offered free tissue transfer from the forearm to address an intra-oral soft tissue deficiency. Despite the presence of appropriately developed right radial artery, used in previous facial reconstruction, the left radial artery occurred to be hypoplastic. He ultimately underwent free flap transfer based on the anomalous persistent left median artery. We suggest that in face of an unusual hand anatomy, flexible flap creation techniques that allow a lifeboat strategy of adjusting flap design should be considered preoperatively. Level of evidence: Level V, therapeutic study.

GOLDENHAR SYNDROME-NO MORE A CHALLENGE TO ANAESTHESIOLOGIST

A patient K/C/O Goldenhar syndrome came for right external ear reconstruction. Patient had typical features of the syndrome with hypoplastic mandible and maxillae, malocclusion of teeth ,malaligned teeth ,retrognathia and hypoplastic hyoid bone . Anticipating a difcult intubation it was decided to go ahead with awake intubation in this patient . Blind nasal intubation was planned because of unavailability of breoptic in our institution and it was achieved with 7 mm ivory white north pole tube with dexmedetomidine infusion at rate of 0.5mcg/kg/hr and topical anaesthesia .