SPOTTER- 6yr old girl, presented with following findings- B/L. Diagnosis, associated syndromes/ conditions and management. What is the embryologic significance



Preauricular tag  ear malformations are associated with an increased frequency of clinically significant structural renal anomalies compared with the general population. This is due to the observation that auricular malformations often are associated with specific MCA syndromes that have high incidences of renal anomalies. These include CHARGE association, Townes-Brocks syndrome, branchio-oto-renal syndrome, Nager syndrome, Miller syndrome, and diabetic embryopathy. Patients with auricular anomalies should be assessed carefully for accompanying dysmorphic features, including facial asymmetry; colobomas of the lid, iris, and retina; choanal atresia; jaw hypoplasia; branchial cysts or sinuses; cardiac murmurs; distal limb anomalies; and imperforate or anteriorly placed anus. If any of these features are present, then a renal ultrasound is useful not only in discovering renal anomalies but also in the diagnosis and management of MCA syndromes themselves. A renal ultrasound should be performed in patients with isolated preauricular pits, cup ears, or any other ear anomaly accompanied by 1 or more of the following: other malformations or dysmorphic features, a family history of deafness, auricular and/or renal malformations, or a maternal history of gestational diabetes. In the absence of these findings, renal ultrasonography is not indicated. Simple preauricular cysts should not be confused with first branchial cleft cysts. Branchial cleft anomalies are closely associated with the external auditory canal, tympanic membrane, angle of the mandible, and/or facial nerve. Misinterpreting a first brachial abnormality for a simple sinus tract may place the unsuspecting physician at risk for damaging the facial nerve, incompletely excising the lesion, or both. Patients identified with preauricular pits or cysts should be examined for other congenital anomalies. Embryo The auricle forms during the sixth week of gestation. The first and second branchial arches give rise to a series of 6 mesenchymal proliferations known as the hillocks of His, which fuse to form the definitive auricle. The first arch gives rise to the first 3 hillocks, which form the tragus, helical crus, and the helix. The second arch gives rise to the second 3 hillocks, which form the antihelix, scapha, and the lobule. Defective or incomplete hillock fusion during auricular development is postulated as the source of the preauricular sinus. Another theory suggests that localized folding of ectoderm during auricular development is the cause of preauricular sinus formation. The first 3 hillocks are most often linked to supernumerary hillocks, leading to preauricular tag formation.
Very comprehensive and exhaustive coverage.... don't think there is much to add to the topic... Thanks sir for enlightening us...

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Accessory tragi. It is a relatively common congenital anomaly of the first branchial arch or second branchial arches. Other anomalies may be present concurrently, including cleft palate, cleft lip, or mandibular hypoplasia. There is a known association with Goldenhar syndrome (oculo-auriculo-vertebral syndrome)and with Wildervanck syndrome. Simple surgical excision is curative. The skin is peeled off the extra-auricular tissue and protruding cartilage remnants are trimmed. Truthfully, I did not know this, found it from the Net. However, did learn something. Thanks.
Pre auricular tag. Associated with 1.Goldenhers syndrome 2.Trechear Collins syndrome. 3.CHARGE association 4.Renal anomalies like horseshoe kidney, absent kidney, double pelvical system.ectopic kidney.
As per pict he does not have microtonia No ophthalmic abnormality seen so It is not goldenhet syndrome. It is my viev Othet may right
Accessory tubercules/ unfused tubercles external ear Usually developmental anomalies of first & second brachial arches
Preauricular skin tag,associated with oculoauriculovertebral dysplasia
B/L preauricular skin tags ? association with Goldenhar Syndrome
B/l accessory tragi Association Goldenhar syndrome
Preauricular appendage
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