PubMed · 8301479
The source did not provide an abstract. Follow the original record for more information.
Explore connections, maps & timelines
G Har-el. 1993. Persistent third branchial apparatus.. https://doi.org/10.1016/0022-3468(93)90495-7
Cite the original work for its findings. Save a collection to share your selection of sources.
KEEP EXPLORING
OBJECTIVE: Malformations of the first branchial cleft are uncommon and only sporadically reported in the literature. They may present as inflammatory openings on the neck, bland cysts or fistula associated with the external auditory canal. In this retrospective study, clinical features and anatomical relationships are described in three pediatric cases. Therapeutical guidelines for surgical management of first branchial cleft anomalies are discussed. PATIENTS: Between 1997 and 1999 three patients aged 9 months, 2 and 7 years with first branchial cleft anomalies were included in this study. All patients were treated surgically, wide exposure and superficial parotidectomy was necessary for complete removal in two of three cases. RESULTS: Exploring patients histories revealed previous infections with repeated incision and drainage procedures as well as inadequate operative resections. Clinically, purulent drainage from the ear, swelling in the parotid area and abscess formation with persistent drainage after incision in the neck or parotid area were noted. CONCLUSIONS: From our case series two of three patients underwent inadequate incision and drainage procedures to combat infection followed by scar tissue formation. Because of the variable relation to the facial nerve this led to difficulties in identifying and protecting the nerve during definite surgery. Management of first branchial cleft anomalies must include the facilities to achieve ear surgery and superficial parotidectomy including facial nerve exposure.
Oculo-auriculo-vertebral (OAV) syndrome is made up of anomalies, mainly of first and second branchial arch derivatives. Characteristic features include structural malformations of the external and middle ears, face, and jaw. It has been previously suggested that hemorrhage involving the first and second branchial arches causes hypoplasia and malformation of the face and auricle, but this theory cannot explain the multisystemic and protean manifestations of this disease. The theory set forth describes the cutaneous, facial, vertebral, and systemic anomalies in the OAV spectrum as a result of ectodermal nondisjunction early in development with subsequent mesodermal tethering. A subgroup of OAV may therefore be a disorder of ectodermal nondisjunction involving the otic placode similar to the spectrum of diseases such as occult spinal dysraphism that is associated with the same mechanism in the embryonic neuraxis. This would imply a molecular mechanism involving cell adhesion molecules that unify the two disease processes and explain the multisystem anomalies of the OAV syndrome.
This article briefly presents the embryology of the ear and discusses the external auditory canal and middle ear, including first branchial cleft anomalies, external auditory canal atresia and stenoses, congenital cholesteatoma, and congenital teratoma. Topics related to the labyrinths include aplasia, the common-chamber anomaly, the pseudo-Mondini and Mondini malformations, semicircular canal dysplasia, and the large vestibular aqueduct. Vascular malformations and variations also are presented, including the absent and aberrant internal carotid artery, the persistent stapedial artery, and high jugular bulb; cerebrospinal fluid and perilymph fistulas are the subjects of the final section.