Journal of Plastic, Reconstructive & Aesthetic Surgery (2014) 68, e35ee36

CORRESPONDENCE AND COMMUNICATIONS Unlilateral microtia, dextrocardia and situs inversus totalis: A possible new association Dear Sir, Microtia is a congenital defect varying from minimal external ear underdevelopment to anotia with a prevalence of 0.8e4.2 per 10,000 births. Hereditary and environmental factors contribute to the pathogenesis of microtia.1 Microtia is often accompanied by hemifaical hypoplasia, congenital heart disease, cleft lip and upper limb deformity. Microtia is also associated with many syndromes, such as first and second branchial arch syndrome, Oculoauriculovertebral Spectrum (OAVS, Goldenhar syndrome), Treacher-Collins syndrome, Nager syndrome, DiGeorge syndrome, CHARGE syndrome, Klippel-Feil syndrome and Meier-Gorlin syndrome. Dextrocardia is a rare congenital condition with a prevalence of 1 in 10,000 births. There are two major types of dextrocardia: isolated dextrocardia and dextrocardia situs inversus. The most common heart defects seen with dextrocardia include: double outlet right ventricle, endocardial cushion defect, pulmonary stenosis or atresia, single ventricle, transposition of the great vessels and ventricular septal defect. We present an extremely rare case of microtia combined with dextrocardia. A 5-year-old boy presented to plastic surgery department for surgical treatment of unilateral microtia. The patient was diagnosed with right microtia, right pulmonary agenesis, dextrocardia, patent ductus arteriosus and situs inversus totalis (Figure 1). On physical examination, the right-sided concha-type microtia was combined with atresia of auditory canal, which was confirmed by coronal cranial CT scan. Hearing test showed right-sided partial hearing loss. The patient’s mental and motor development was normal. Plain radiography indicated dextrocardia, transposition of the great arteries and situs inversus totalis, which were further confirmed by chest and abdominal CT scan, showing the thoracic aorta located on the right front side of the thoracic vertebrae, the liver on the left position,

the stomach bubble on the left position and the spleen on the right position (Figure 2). Echocardiogram showed patent ductus arteriosus (1.5 mm). Oculoauriculovertebral spectrum (OAVS) displays several characteristics: epibulbar dermoid, microtia, hemifacial microsomia and vertebral anomalies. Rate of heart defect is ranging from 5% to 58% in OAVS patients. In our case, the patient showed no epibulbar dermoid or thoracic vertebral anomalies. Maymon reported a case of right pulmonary agnenesis, dextrocardia, signs of situs solitus (stomach) and ipsilateral microtia by antenatal sonographic detecting.2 However, Milani D diagnosed this case as a special form of OAVS.3 Derbent M reported a rare case of OAVS: left microtia, dextrocardia, DORV, VSD, complete fusion of C2eC3 and posterior fusion of T1eT2 vertebrae,4 which indicated that dextrocardia may be included in OAVS.

Figure 1 Right-sided concha-tye microtia without apparent facial asymmetry.

http://dx.doi.org/10.1016/j.bjps.2014.10.020 1748-6815/ª 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

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Correspondence and communications

Conflict of interest None.

References 1. Zhang QG, Zhang J, Yu P, et al. Environmental and genetic factors associated with congenital microtia: a case-control study in Jiangsu, China, 2004 to 2007. Plast Reconstr Surg 2009;124(4):1157e64. 2. Maymon R, Schneider D, Hegesh J, et al. Antenatal sonographic findings of right pulmonary agenesis with ipsilateral microtia: a possible new laterality association. Prenat Diagn 2001;21(2): 125e8. 3. Milani D, Selicorni A. Right pulmonary agenesis with ipsilateral microtia: a new laterality association or part of the oculoauriculovertebral spectrum? Prenat Diagn 2002;22(11):1053e4. 4. Derbent M, Yilmaz Z, Baltaci V, Saygili A, Varan B, Tokel K. Chromosome 22q11.2 deletion and phenotypic features in 30 patients with conotruncal heart defects. Am J Med Genet A 2003;116A(2):129e35. 5. Senen D, Ayhan M, Erdogan B. A rarely seen anomaly: situs inversus-pda microtia. Plast Reconstr Surg 2002;110(1):372e3.

Figure 2 Plain radiography and abdominal CT scan indicate dextrocardia (DE) and situs inversus totalis, including liver (LI), stomach bubble (ST) and spleen (SP).

Senen D reported a similar case of microtia, patent ductus arteriosus, short stature, hypoplasia of the right mandible and occlusion disorder.5 In conclusion, we suggest this special case of unclassified multiple microtia as a new association.

Ethical approval N/A.

Funding The study was supported by Supported by PUMC Youth Fund and the Fundamental Research Funds for the Central Universities (3332013091) and National Natural Science Foundation of China (81300863 to J-T.H., 81372085 to Q-G.Z., and 31371347, 31201006 to Y-B.Z.)

Jintian Hu Tun Liu Xu Zhou Department of Ear Reconstruction, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, Beijing, PR China Yongbiao Zhang Beijing Institute of Genomics (BIG) of Chinese Academy of Sciences, Department of Ear Reconstruction, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, No. 33 Ba-Da-Chu Road, Shi Jing Shan District, Beijing 100144, PR China Qingguo Zhang Department of Ear Reconstruction, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, Beijing, PR China E-mail address: [email protected] 20 April 2014

Unlilateral microtia, dextrocardia and situs inversus totalis: a possible new association.

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