Preauricular Tags Research Articles

A Novel Procedure for the Removal of Supernumerary or Accessory Tragus on Neonates

The majority of supernumerary or accessory tragus in humans are noted soon after birth, and are generally benign isolated lesions not associated with other genetic abnormalities. When present, these lesions are typically managed by the primary care provider, but occasionally the caretakers opt to refer the patient to a surgeon to have the lesion resected surgically as an outpatient. This practice may place an unnecessary financial burden on the patient’s family, and may pose added difficulty due to the availability of the subspecialist. The current literature lacks other practical and effective methods for dealing with these lesions despite the incidence of up to 1.5% of the population [1]. Traditionally, however, these lesions are managed by pediatricians or the PCP by placing a suture ligature at its base so that the distal portion of the tragus will fall off after the ischemic necrosis has occurred [2]. This approach is the current standard of care, and is the method being taught at most pediatric training programs. When successful, this process can take days if not weeks to run its course. Another approach may be to refer these patients to a Plastic Surgeon or a Pediatric Surgeon for care which may be to have the lesions managed by means of application of surgical clips [3] at their base thus achieving a similar effect as a ligature. Alternatively, the lesions can be permanently surgically excised later when the patient is older.

Ligaclip for Preauricular Skin Tags in the Newborn.

Skin tags are benign lesions, that often represent only an aesthetic problem; if subjected to trauma, they can occasionally bleed with possible infection and pain. When they occur in the preauricular region, attention should be paid to the diagnosis and approach; in fact, controversy exists in the differential diagnosis between hair follicle nevi, accessory tragus, and skin tag. Misdiagnosis and failure of treatment can lead to serious consequences, such as chondritis. In our retrospective study, we evaluated 19 newborns affected by single, unilateral skin tag in the preauricular region. Each patient underwent a careful clinical examination; lesions without a pilosebaceous unit and with a thin, soft pedicle were treated in the nursery with Ligaclip (Johnson & Johnson). Skin tag falls between day 7 and 10. We had no cases of edema, cellulitis, clip loss, or bleeding. Scarring results were extremely satisfactory at 3-month follow-up. We believe that after a careful clinical examination, cases of skin tags in the preauricular area can be selected and treated with Ligaclip. This procedure can be considered rapid, safe, economical, and simple in the newborn patients.

Incidence and characterization of early- and late-onset skin diseases in neonates – A hospital-based cross-sectional study

Background: Neonatal skin diseases occur in almost every newborn baby. Many are transient, involute rapidly and require no management. Objective: The objective of the study was to study the incidence and characterization of early and late onset of various skin diseases in the neonates. Methods: A cross-sectional observational study was carried out among 200 neonates in a tertiary care hospital in Central India. Skin lesions which appeared on or before 48 h before birth were categorized as early onset and skin lesion appearing after 48 h were considered as late onset and the characteristics of lesions were highlighted. Results: The most common skin lesions identified within 48 h of birth were Epstein pearl’s (77.0%), followed by Mongolian spot (45.5%), desquamation of skin (35%), sebaceous gland hypertrophy (31.5%), and milia (28.4%), respectively. Less incidence of salmon patch, erythema toxicum neonatorum, impetigo, miliaria rubra, accessory auricles, pigmented nevi, port-wine stain, and lamellar ichthyosis also was recorded. Impetigo (11.2%) and candidiasis (11.2%) were commonly seen after 48 h of birth followed by dermatitis 6.4% and a few cases of birthmarks. Conclusion: Along with increasing awareness of neonatal diseases, our study results also aid in better identification and diagnosis of these diseases.

Goldenhar syndrome: rare case reported from secondary health care facility in Himachal Pradesh

Dr. Maurice Goldenhar, a renowned Swiss ophthalmologist classified the clinical features and named the malformation complex as Goldenhar Syndrome and described it as a congenital defect characterized by constellation of malformations classically involving the face, eyes and ears. Principal deformities of the Goldenhar syndrome are often combined with various malformations, like Cleft lip and/or palate, tongue cleft, unilateral tongue hypoplasia, and parotid gland aplasia, Rib anomalies, Congenital heart disease (ventricular septal defects). A 12-year-old female patient diagnosed with Goldenhar syndrome at secondary care Regional Hospital Bilaspur, Himachal Pradesh. She was diagnosed with a case of Goldenhar syndrome on clinic-radiological investigations. The patient reported to ophthalmology OPD of the hospital with the chief complaint of soft tissue structure in left eye with decreased vision. On examination limbal dermoid was observed. Patient was having pre-auricular tissue tag on both sides, hemifacial micrognathia and microstomia. Correlating the history and physical findings a provisional diagnosis of Goldenhar Syndrome was made with differential diagnosis of Franschetti syndrome or Treacher – Collins syndrome, Nager syndrome or acro-facial dysostosis and Townes-Brocks syndrome. Based on the clinical and radiographic findings a diagnosis of Goldenhar syndrome was made. Severe cases of GS can affect the routine and social life of the patient. Early detection can help avoid complications at a later stage of life. Such patients will have an increased risk for psychosocial difficulties. The study of this condition is still controversial because the symptoms and the physical features may vary greatly in range and severity from case to case.

Varied ocular manifestations of goldenhar syndrome

Goldenhar syndrome, also known as oculo-auriculo-vertebral syndrome, is a rare congenital disorder due to defect in the development of the first and second branchial arches during blastogenesis. This syndrome consists of epibulbar dermoids, eyelid coloboma, auricular abnormalities, preauricular appendages, and mandibular hypoplasia. There can also be associated cleft palate, malocclusion of teeth, and involvement of the axial skeleton (vertebrae and ribs). Congenital heart disease and anomalies of the genitourinary system are some rare manifestations of this syndrome. We report two cases of Goldenhar syndrome with varied ocular manifestations. The first patient was an 18-year-old female who presented with an inferotemporal limbal dermoid with protruding hair involving the inferotemporal cornea. She also had external ear malformation, preauricular skin tag, and mandibular hypoplasia. The second patient was a 3-year-old boy with low intelligence who presented with an upper eyelid coloboma and a temporal limbal dermoid in the left eye with telecanthus and epicanthal folds. There were associated mandibular hypoplasia and preauricular skin tag. Both the patients did not have any vertebral anomaly or congenital heart defect.

Cutaneous lesions of the auricular portion of external ear

Aim: Diseases coming from the external ear (outer ear canal and auricle) skin can be evaluated by many medical disciplines. External ear cutaneous lesions can be nonneoplastic, preneoplastic or neoplastic. Our aim in this study is to evaluate these surgically treated diseases.Material and Methods: All external ear tumors and tumor-like lesions that were surgically excised between 2013 and 2018 were evaluated retrospectively. The relationship between demographic features and histopathological findings and prognostic factors were analyzed.Results: Lesions were excised in the external ear in a total of 141 patients. While 91 (64.5%) of the cases were male and 50 (35.5%) were female, the average age was 33.9 (between 1 and 98 years old). While the average age of all benign tumors and non-neoplastic lesions was 27.7, 60.5% were male and 39.5% were female. The average age of malignant tumors and precursors was 67.2, the male rate was 86.4%, and the female rate was 13.6%. While cystic lesions are the most common lesion group with 27.7% (39 cases), benign non-epithelial tumors 27% (38 cases); malign epithelial tumors 15.6% (22 cases); hamartomas and similar lesions 11.3% (16 cases); inflammatory and infectious conditions 7.8% (11 cases); other formations (cutaneous deposit etc.) 7.8% (11 cases), and benign epithelial tumors 2.8% (4 cases), respectively. The most common cystic lesion was epidermal cyst and the most common benign and non-epithelial lesions were intradermal melanocytic nevi and keloid. Malignant epithelial tumors mostly detected in the external ear, squamous cell carcinoma and basal cell carcinoma. Among the hamartoma or congenital lesions and inflammatory lesions, accessory tragus and hair follicular nevus and foreign body granulomatous reaction were the most frequently observed, respectively.Among benignepithelial tumors, seborrheic keratosis was the most common.Conclusion: There are rare cutaneous lesions of the external ear that are treated with various medical and surgical specialities. In this anatomical location, tumors seen at a young age are mostly benign, while malignant tumors are more common in older ages and males, while hamartoma and similar congenital lesions are observed at a young age. A multidisciplinary approach in treatment is important both in terms of prognosis and aesthetics.

Unilateral Nevus of Ota with Palatal and Optic Disc Pigmentation with Coincidental Preauricular Tag- A Case Report

Nevus of Ota also known as oculodermal melanosis presents as hyperpigmentation of face involving ophthalmic and maxillary branches of trigeminal nerve associated with ocular hyperpigmentation. It is due to confinement of melanocytes in the dermis. Most commonly it is unilateral but sometimes it may have bilateral presentation. Typically, it presents at birth but can also be an acquired condition. Frequently seen in Japanese and rarely in Indian subcontinent. It has more predilection towards females. Less frequently, hyperpigmentation is seen in other sites like oral mucosa, tympanum and nasal mucosa. These patients are at high risk of developing glaucoma and malignancy. The author reported a case of 12-year-old male child with unilateral pigmentation of left side face involving forehead, periorbital and cheek, along with ocular pigmentation. Hyperpigmentation of conjunctiva, iris and angles is present in left eye with intraocular pressure being normal in both eyes. Fundus showing optic disc pigmentation in the left eye with cup disc asymmetry in both eyes. Child has coexistent preauricular tag on the left side.

Accessory Auricle Deformity of Eustachian Tube.

Accessory Auricle Deformity of Eustachian Tube.

Ear anomalies (congenital) at referral hospital

Background: In otorhinolaryngology region, about half of the malformation affects the ear. Congenital anomalies of the ear can affect the external ear, middle ear, and inner ear. The ear malformations can be unilateral or bilateral. In this study, we enrolled patients with preauricular cyst, tag, accessory tragus, preauricular sinus, pinna sinus, microtia, anotia, EAC atresia, and congenital sensorineural hearing loss (SNHL). Hearing loss may lead to delayed language development and poor school performance. Universal newborn hearing screening allows for earlier detection and intervention in infants with hearing loss, which may result in improved language development in affected children. Estimated rates of congenital SNHL range from 1/1000 to 3/1000 live births. Aim and Objective: Proper and early diagnosis can change the future of a child by providing hearing rehabilitation and speech therapy. Materials and Methods: It is a prospective study of 122 patients of ear-related anomalies, conducted at the Government Medical College and Sir T. Hospital, Bhavnagar, Gujarat, India, from January 2016 to July 2018. Results: we have observed patients with otological malformations which includes external, middle and inner ear malformations. Conclusion: This study contains 122 patients, of which more than half of the patients are male (54.91%). We obsereved patients with Congenital SNHL, pinna anomalies, congenital facial weakness. We have also obtained cases with Waardenburg syndrome and Goldenhar syndrome.

Clinical Presentation of Congenital Cholesteatoma of the External Auditory Canal in Goldenhar Syndrome: A Case Series

Background: Goldenhar Syndrome, also called Oculoauricular Vertebral Dysplasia, includes abnormalities of the first and second branchial arches. Ear abnormalities are present in approximately 92% of those with Goldenhar Syndrome [1]. There is a wide spectrum for ear anomalies; they can range from preauricular tags and pits, microtia, external auditory canal (EAC) atresia, ossicular malformations, and facial nerve hypoplasia, which can lead to an array of clinical findings [5]. Hearing loss is an unfortunate consequence of these clinical findings, and often results in subsequent speech and language delays [4]. Despite the widely reported auricular and audiologic manifestations of this disease, a review of the literature revealed no detailed description or analysis of congenital canal cholesteatoma specifically in this patient population. Objective: This study aims to characterize congenital external auditory canal cholesteatoma as a feature of Goldenhar Syndrome. Method: A retrospective case series was conducted with patients seen at the UNC Craniofacial Center in Chapel Hill, NC. Inclusion criteria included patients with a diagnosis of Goldenhar Syndrome also found to have congenital cholesteatoma necessitating surgery. A total of three patients were identified who met inclusion criteria. Results: Three patients with Goldenhar Syndrome were identified who also had congenital external auditory canal cholesteatoma. All three patients underwent surgical management of their disease. Conclusion: Congenital external auditory canal cholesteatoma appears to be a feature of Goldenhar Syndrome not widely described in current literature. Though rare, it is a relevant disease process with significant clinical implications for both hearing as well as speech and language development. Awareness of this condition can thus help guide practitioners in the care of these patients.

Anterior segment dysgenesis and secondary glaucoma in Goldenhar syndrome

A 3-year-old girl presented with a history of watering, haze and increase in the size of the right eye for two months. The child had bilateral preauricular skin tags, limbal dermoid and dermolipoma, consistant with the diagnosis of Goldenhad syndrome. In addition, her right eye manifested enlarged cornea, flat anterior chamber, atrophic iris and elevated intraocular pressure. This case report highlights a possible association of anterior segment dysgenesis and glaucoma with Goldenhar syndrome.

Accessory Auricle Deformity of Eustachian Tube

Accessory Auricle Deformity of Eustachian Tube

Goldenhar Syndrome with Occipital Meningoencephalocele: A Rare Case

A 7-year-old male patient reported in our neurosurgery OPD with chief complaints of swelling in occipital region. The swelling was gradually increasing in size as per history given by patient’s attendant. Antenatal and birth history was uneventful. Developmental milestones were normal. Ear examination showed left sided pre auricular tag. Hearing was normal. Eye examination showed right eye limbal dermoid. Limbs examination showed syndactyly of 5th finger. Cardiovascular, respiratory and oral examination was normal. His height and weight were within normal limit. Chest X-ray was normal. Skeletal survey did not show any vertebral deformities. The karyotype was normal. The MRI brain showed a defect of size 1.3 cm in the occipital region through which cerebellum was seen herniating. The mass was surrounded by hypodense collection with multiple thickened septae within suggestive of CSF collection with thickened meninges. Patient underwent surgery and cystic portion was removed, with preservation of the occipital and cerebellar parenchyma and closure of defect was done. Patient responded well that there were no post operative complications and child was discharged on the 10th postoperative day. Hence, based on clinical and radiographic findings, a diagnosis of Goldenhar syndrome was made.

GOLDENHAR SYNDROME: A CASE REPORT

Goldenhar syndrome is a birth defect resulting from the maldevelopment of the first two branchial arches with incomplete development of the ear, nose, soft palate, lip and mandible. The phenotype is highly variable. Goldenhar syndrome is one of the variants of craniofacial anomalies. It is unilateral in 70-80% of the cases. It is known as oculoauriculo vertebral (OAV) dysplasia. The syndrome complex includes limbaldermoid or lipodermoid, pre-auricular tags, hemifacial asymmetry and vertebral anomalies. These are the common anomalies of the condition.This work reports a case 14 year old male in ophthalmology department of MMIMSR who presented with limbaldermoid and on evaluation was found to have the classical signs of this syndrome. Also included is the current protocol for treatment of this syndrome.

The oculoauriculofrontonasal syndrome: Further clinical characterization and additional evidence suggesting a nontraditional mode of inheritance.

The oculoauriculofrontonasal syndrome (OAFNS) is a rare disorder characterized by the association of frontonasal dysplasia (widely spaced eyes, facial cleft, and nose abnormalities) and oculo-auriculo-vertebral spectrum (OAVS)-associated features, such as preauricular ear tags, ear dysplasia, mandibular asymmetry, epibulbar dermoids, eyelid coloboma, and costovertebral anomalies. The etiology is unknown so far. This work aimed to identify molecular bases for the OAFNS. Among a cohort of 130 patients with frontonasal dysplasia, accurate phenotyping identified 18 individuals with OAFNS. We describe their clinical spectrum, including the report of new features (micro/anophtalmia, cataract, thyroid agenesis, polymicrogyria, olfactory bulb hypoplasia, and mandibular cleft), and emphasize the high frequency of nasal polyps in OAFNS (56%). We report the negative results of ALX1, ALX3, and ALX4 genes sequencing and next-generation sequencing strategy performed on blood-derived DNA from respectively, four and four individuals. Exome sequencing was performed in four individuals, genome sequencing in one patient with negative exome sequencing result. Based on the data from this series and the literature, diverse hypotheses can be raised regarding the etiology of OAFNS: mosaic mutation, epigenetic anomaly, oligogenism, or nongenetic cause. In conclusion, this series represents further clinical delineation work of the rare OAFNS, and paves the way toward the identification of the causing mechanism.

Accessory auricle: Classification according to location, protrusion pattern and body shape.

BackgroundAccessory auricles (AAs) are common congenital anomalies. We present a new classification according to location and shape, and propose a system for coding the classifications.MethodsThis study was conducted by reviewing the records of 502 patients who underwent surgery for AA. AAs were classified into three anatomical types: intraauricular, preauricular, and buccal. Intraauricular AAs were divided into three subtypes: intracrural, intratragal, and intralobal. Preauricular AAs were divided into five subtypes: precrural, superior pretragal, middle pretragal, inferior pretragal, and prelobal. Buccal AAs were divided into two subtypes: anterior buccal and posterior buccal. AAs were also classified according to their protrusion pattern above the surrounding surface: pedunculated, sessile, areolar, remnant, and depressed.Pedunculated and sessile AAs were subclassified as spherical, ovoid, lobed, and nodular, according to their body shape. Cartilage root presence and family history of AA were reviewed. A coding system for these classifications was also proposed.ResultsThe total number of AAs in the 502 patients was 1,003. Among the locations, the superior pretragal subtype (27.6%) was the most common. Among the protrusion patterns and shapes, pedunculated ovoid AAs were the most common in the preauricular (27.8%) and buccal areas (28.0%), and sessile lobed AAs were the most common in the intraauricular area (48.7%). The proportion of AAs with a cartilage root was 78.4%, and 11% of patients had a family history. The most common type of preauricular AA was the superior pretragal pedunculated ovoid AA (13.2%) with a cartilage root.ConclusionsThis new system will serve as a guideline for classifying and coding AAs.

Investigation of copy number variations on chromosome 21 detected by comparative genomic hybridization (CGH) microarray in patients with congenital anomalies

BackgroundThe clinical features of Down syndrome vary among individuals, with those most common being congenital heart disease, intellectual disability, developmental abnormity and dysmorphic features. Complex combination of Down syndrome phenotype could be produced by partially copy number variations (CNVs) on chromosome 21 as well. By comparing individual with partial CNVs of chromosome 21 with other patients of known CNVs and clinical phenotypes, we hope to provide a better understanding of the genotype-phenotype correlation of chromosome 21.MethodsA total of 2768 pediatric patients sample collected at the Genetics Laboratory at Oklahoma University Health Science Center were screened using CGH Microarray for CNVs on chromosome 21.ResultsWe report comprehensive clinical and molecular descriptions of six patients with microduplication and seven patients with microdeletion on the long arm of chromosome 21. Patients with microduplication have varied clinical features including developmental delay, microcephaly, facial dysmorphic features, pulmonary stenosis, autism, preauricular skin tag, eye pterygium, speech delay and pain insensitivity. We found that patients with microdeletion presented with developmental delay, microcephaly, intrauterine fetal demise, epilepsia partialis continua, congenital coronary anomaly and seizures.ConclusionThree patients from our study combine with four patients in public database suggests an association between 21q21.1 microduplication of CXADR gene and patients with developmental delay. One patient with 21q22.13 microdeletion of DYRK1A shows association with microcephaly and scoliosis. Our findings helped pinpoint critical genes in the genotype-phenotype association with a high resolution of 0.1 Mb and expanded the clinical features observed in patients with CNVs on the long arm of chromosome 21.

Presentation and Surgical Management of Epibulbar (Limbal) Dermoids

Purpose: To report clinical characteristics and surgical outcomes of limbal dermoids excision in Pakistani patients. Study Design: Retrospective, Descriptive. Place and Duration of Study: Mughal Eye Hospital Lahore from 1st June 2016 to 30 Dec 2017. Material and Methods: Records of 15 epibulbar dermoids were reviewed. Site, colour, presence of pigmentation and presence of ocular and systemic associations of the lesions were studied. Lesions were photographed and then surgically managed. Excision was done with blade and scissors. Postoperative follow up included management of complications and photograph on each visit. Results: Age ranged from 4 to 60 years (Mean 18 ± 13.48). Male to female ratio was 6:9. All the patients had unilateral epibulbar dermoids, which were present in inferotemporal quadrant of the limbus. Most lesions (9 cases - 60%) involved cornea and sclera equally while a few extended more on the scleral (3 cases - 20%) or corneal side (3 cases - 20%). Most (12 cases - 80%) were round and a few (3 cases- 20%) tapering. 5 (33%) had Goldenhar Syndrome. 4 (27%) patients had preauricular tags. One (7%) patient had maxillary hypoplasia and divergent squint. Postoperatively one patient (7%) had corneal thinning and one patient (7%) had extensive formation of granulation tissue. There was pigmentation of the lesion in 12 cases (80%). Three (20%) patients had microtia. Conclusion: Limbal dermoids in Pakistani patients have characteristics resembling those described in other parts of the world. Treatment with excision and superficial sclerokeratectomy without graft gives satisfactory results. Keywords: Epibulbar Dermoids, Limbal Dermoids, Goldenhar syndrome.

Three cases of cervical accessory auricle

Three cases of cervical accessory auricle

Accessory Auricles: Systematic Review of Definition, Associated Conditions, and Recommendations for Clinical Practice.

Accessory auricles are rare congenital abnormalities embryologically derived from the first branchial arch. Presentation may be variable with locations grouped into anatomical zones based on the frequency of location found in the literature. This study reviewed the papers between 1981 and 2017. Findings included an association with syndromes including Goldenhar, VACTERL, Treacher-Collins, Townes-Brocks, and Wolf-Hirschhorn. Based on histological and embryological evidence, the term "accessory auricle" is best used as an umbrella term to define this difference. Finally, indications are provided for imaging and laboratory investigations to guide the physician treating patients with accessory auricles.