Rare Imaging Presentation In A Child With Clinical History Of Hearing Loss (original) (raw)

Hemifacial Microsomia with bilateral ear involvement

Applied Medical Research, 2015

Hemifacial microsomia is a rare form of craniofacial anomaly displaying abnormalities of the derivatives of the first and second branchial arches. A diagnosis of hemifacial microsomia is made if the patient presents with microtia, hypoplastic mandible, maxilla or the zygomatic arch on one side of the face. Bilateral involvement is rare. Here we present a rare form of the syndrome which presented with bilateral ear involvement and hearing deficit. Radiographs helped in diagnosing the case as hemifacial microsomia. The condition is extremely complex and heterogenous. Accurate diagnostic workup is necessary to differentiate this condition from other conditions of the first and second arch derivatives. We describe here the characteristic clinical and radiographic features which help to improve the knowledge on the disorder.

Hemifacial Microsomia – A Case Report and Review of Literature

Hemifacial microsomia is a congenital deformity in which there is a deficient amount of skeletal and soft tissues on one side of the face. It is a syndrome affecting mainly first and second branchial arches, which include underdeveloped temporomandibular joint, mandibular ramus, masticatory muscles, ears and defects in facial nerve and muscles. The soft tissue malformation is present in the external ear, and the affected ear is placed lower as compared to the contralateral side. In addition to this, underdevelopment of osseous components leads to hearing loss with diminished external auditory meatus. The incidence of this disorder is 1:3000‑26,000, which is usually seen at birth. Here, we present a case of 20-year-old girl, who complained of facial asymmetry on the left side of the face and deformity of left ear since birth with loss of hearing.

Hemifacial Microsomia - A Case Report With Review Of Literature

Kocaeli Üniversitesi Sağlık Bilimleri Dergisi, 2017

Hemifacial microsomia (HFM) is a congenital deformity characterised by unilateral deficiency in the volume of hard and soft tissue of face. It is a syndrome predominantly affecting first branchial arch featuring poorly developed temporomandibular joint, mandibular ramus, muscles of mastication and ear. People with HFM may present with an underdeveloped ear (microtia) or absent ear (anotia) on the affected side of the face. The incidence of this disorder is 1:3000-26,000, which is usually seen at birth. Here we present a case of hemifacial microsomia in a 12-year-old girl with hearing difficulties on the affected side with review of literature.

Hemifacial Microsomia: Case series and overview

International Journal of Clinical Images and Medical Reviews, 2022

A congenital facial abnormality called hemifacial microsomia (HFM) that causes hypoplasia of the first and second pharyngeal arches derivatives like the temporomandibular joint, mandibular ramus and body, masticatory muscles, the ear, and occasionally the facial nerve. In terms of developmental craniofacial anomalies, cleft lip and palate, which typically occurs unilaterally but can occasionally occur bilaterally, is the most prevalent, followed by HFM. There can be a number of defects, such as conductive hearing loss brought on by middle and external ear malformations. Diagnostic imaging and face structure categorization using the OMENS system are crucial for the pre-surgical evaluation of this abnormality. This developmental abnormality is managed in a multidisciplinary manner. We are showcasing a trio of examples with a variety of clinical and radiological characteristics, ranging from moderate ear deformity and facial asymmetry. Case Series Case 1 A 15 years old female was referred to department of Radio-diagnosis for cross-sectional Non-contrast CT scan of Face with 3D recon

Hemifacial microsomia: a series of three case reports

Journal of clinical and diagnostic research : JCDR, 2013

Hemifacial Microsomia (HFM) is an asymmetric craniofacial malformation which results in hypoplasia of the components of the first and second branchial arches. There can be various anomalies, which include conductive hearing loss which is caused by external and middle ear deformities. HFM is the second most common congenital facial anomaly which is seen after cleft lip/palate. For the pre-surgical evaluation of this anomaly, diagnostic imaging and classification of the facial structures, based on OMENS classification, is of prime importance. The management of this developmental malformation is multidisciplinary. We are presenting a series of three cases with diverse clinical and radiographic features which ranged from mild facial asymmetry and ear malformation to facial paralysis.

Hemifacial Microsomia in a Female Kid - A Clinico-Radiological report

2021

Otomandibular Auricular syndrome or Hemifacial Microsomia (HFM) is an asymmetric craniofacial malformation which results in hypoplasia of the components of the first and second branchial arches. HFM is the second most common congenital craniofacial anomaly after cleft lip/palate. The incidence of this disorder is 1:3000 26,000, which is usually seen at birth. Males are more commonly affected than females and right side is more affected than the left side. For the pre-surgical evaluation of this anomaly, diagnostic imaging and classification of the facial structures, based on OMENS classification, is of prime importance. The management of this developmental malformation is multidisciplinary. Here we report a case of Hemifacial Microsomia in a six-year-old female kid.

Hemifacial microsomia: a clinicoradiographic report of a case

2017

Hemifacial microsomia is a rare congenital malformation of craniofacial structures. Its characteristic features are unilateral underdevelopment of the face and ear malformations. This study describes clinical and radiographical features of a rare case of a 4-year-old hemifacial microsomia patient with underdevelopment of the left side of the face and preauricular skin tags on the affected side. Management of these patients requires a multidisciplinary approach to provide the most effective and appropriate treatment.

Hemifacial microsomia: clinicoradiological insight and report of a case

Ethiopian Journal of Health Sciences, 2017

BACKGROUND: Hemifacial microsomia is a congenital malformation characterized by deficiency in the amount of hard and soft tissue on one side of the face. It is primarily a syndrome of the first branchial arch, involving underdevelopment of the temporomandibular joint, masticatory muscles, mandibular ramus, ear and, occasionally, defects in facial nerve and muscles. CASE DETAILS: The clinical and radiological manifestations of a 14-year-old male patient having hemifacial microsomia is highlighted in this article to enhance our knowledge and diagnostic skill of this rare entity. CONCLUSION: This case illustrates that early diagnosis and intervention in a patient with hemifacial microsomia is quintessential for proper functioning and esthetics of the orofacial structures, which will lead to a better prognosis.

Congenital malformations of the ear

The Indian Journal of Pediatrics, 1992

Tile external ear develops from I and II branchial arches commencing on 38th day of fetal life. The middle ear is formed from the ends of Ist pharyngeal puch and the surrounding mesenchyme, which also is part of the I and II branchial arches. The congenital defects of the external and middle ear usually occurs in combination, and many times with congenital defects of other systems. The cochlear functions i.e. bone conduction is normal in 50% of these cases, thus rehabilitation of these patients with congenital anomalies of external and middle ear is possible. The coexistence of congenital aural atresia with varying degrees of malformation of inner ear may be more frequent than generally assumed. Moderate and severe forms of congenital aural atresia area encountered in about 1 in 10,000 to 20,000 individualJ Tile more severe forms of congenital auricular malformation are always associated with meatal atresia, whereas meatal atresia may, in a few cases be seen in patients with a normal pinna. Atresia of the meatus may be membranous or osseus, membranous atresia is much less common and is characterised by rudimentary cartilagenous canal sep~ated from the middle ear by a dense structure of con