A 12-year-old female, who was the firstborn of a non-consanguineous marriage, was diagnosed with anotia on the left side, cyanosis, and facial asymmetry at birth. Figure 1 shows absent ear cartilage and external auditory canal on the left side. She was operated for truncus repair with 12mm aortic homograft and PDA ligation was performed at 2 months of life. She was then put on anti-failure drugs. On examination of the cardiovascular system, S2 had a wide split, and ejection systolic murmur was of intensity 3/6 at the lower-left sterna border. The oxygen saturation (SPO2) was 68 % in both the upper extremities. Findings of 2D Echocardiograph suggested of patent ductus arteriosus (PDA), truncus arteriosus type I, ventricular septal defect (VSD), and severe Pulmonary artery hypertension. Ultrasonography disclosed unremarkable study of kidney, ureter and bladder region. The patient grew with normal development and scholastic performance and doing well in school but had restrictions on physical exertion. Moreover, she had moderate growth failure. Upon audiogram, right ear mild conductive hearing loss and left moderately severe conductive hearing loss was found. High-resolution computed tomography (HRCT) of the Petrous Temporal was suggestive of non-visualisation of left external ear (pinna), left auditory canal suggested of agenesis of the left external ear. Both mastoid air cells demonstrated sclerosing and loss of pneumatisation, which was suggestive of bilateral sclerosing mastoiditis. Besides, the course of the left facial nerve was normal.
Patient started to have a deformity of upper back at the age of 10 years, which was progressive and caused mild left shoulder elevation. She did not present with neurological deficits. Her coronal and sagittal balance was good without truncal imbalance. The X-ray of the spine showed kyphoscoliosis as shown in Figure 2. Both left thoracic curve and right proximal thoracic curve were rigid in finding, with a rib hump of grade 3. Magnetic resonance imaging (MRI) of the whole spine showed the congenital fusion of C2 and C3 vertebrae with the presence of a rudimentary disc in between. C5 and C6 vertebrae showed partial fusion. D1, D2 and D7 level showed the presence of hemi vertebrae, while D5 and D6 showed butterfly vertebrae. The cervicodorsal region had marked kyphoscoliosis. The patient underwent posterior scoliosis correction with posterior instrumented fusion from D1 to D11 for the correction of double thoracic congenital scoliosis with D2 and D7 wedge vertebra (Figure 3). The patient was advised left pinna construction by plastic surgery for the cosmetic purpose to augment symmetry of face although it would not improve hearing ability of left ear. Furthermore, recent cardiac catheterization study showed severe obstruction across pulmonary homograft with grade II Pulmonary Regurgitation and hypertensive dysfunctional right ventricle warranting yet another surgery for homograft replacement. The patient s parents gave informed consent for reporting herradiography and clinical.