Elena Gómez García

Atypical case of congenital maxillomandibular fusion with duplication of the craniofacial midline.

We report the first case of syngnathia with hypophyseal duplication and describe the central nervous system (CNS) and craniofacial anomalies associated with hypophyseal duplication in the reported autopsy case. We studied clinical reports, scanner images, and autopsy results of a 2-months-old female baby. The propositus had frontonasal dysmorphism, retrognathia, and bifid tongue. She also presented maxillomandibular bony fusion (syngnathia) and an intraoral hairy polyp. In the cranium, the sella turcica was broadened, with two complete hypophyses and two infundibulums. The CNS had both olfactory bulbs and corpus callosum agenesis. There are 27 previous cases of maxillomandibular fusion and seven previous autopsy cases of hypophyseal duplication associated with other frontonasal malformations. As far as the authors know, this is the first case reported in the literature that associates syngnathia with duplication of the craniofacial midline including hypophyseal duplication.

Early Diagnosis of Necrotizing Fasciitis of Unknown Origin: A Challenge to Prevent the Delay of Surgical Treatment

Cervical Necrotizing Fasciitis is an uncommon infection. It is characterised by a rapidly progressive polymicrobial infection that spreads along the deep fascial planes of the neck. Its fulminant course and high morality makes this disease a diagnostic challenge for the maxillofacial surgeon. Therefore, when clear clinical signs and suspicious images are available, the patient must be taken to the operating room for surgical treatment. Although it is considered a rare entity, misdiagnosis is not acceptable due to its fatal outcome. Close physical examination with a thorough medical history may set the alarm. Further medical test will help to achieve the diagnosis, but never should delay the start of the appropriate treatment. We present a case of cervical necrotizing fasciitis of unknown origin successfully managed in our department. Odontogenic infection and less frequently tonsilar infections are the origin of this infection. Nevertheless, just an auricular wound could be identified as a possible source of this cervical necrotizing fasciitis. We believe that the aggressive surgical treatment combined with broad-spectrum antibiotic and intensive medical care was the key of the early recovery and few after-effects. The aim of this article is to insist in the weigh of the maxillofacial surgeon on the early surgical treatment of the cervical necrotizing fasciitis.