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.

Rhinoorbitocerebral mucormycosis: a case report and literature review.

Mucormycosis is a rare oportunistic infection typically described in diabetic patients with a ketoacidotic status, as well as neutropenic patients. The infection is caused by a group of saprophytic fungi of the class Phycomicetes, being the most frequent ones the Rhizomucor, Rhizopus and Mucor. Its hystological findings include vascular trombosis and tissue necrosis, predominantly in the rino-orbito-cerebral area. Even though the frequency of presentation is very low, given its rapid evolution and severe consequences which include a high mortality rate, it is very important to be aware of the main features of the disease and treat it promptly. Although the diagnosis is based on the high clinical suspect, the computed tomography (CT) and the magnetic resonance image (MRI) plays an important role in determining the extension. The patients should receive treatment in a reference hospital so that a multidisciplinary approach is ensured. In this sense, we present a case of rhino-orbito-cerebralmucormycosis in a diabetic patient, recently treated in our Department. A comprehensive review of the literature has been performed to update the physiopathology and diagnosis. Finally, we describe the different treatment options focusing in the surgical approach, as well as the medical treatment with amphotericine and posaconzole.