Goldenhar syndrome: a cause of secondary immunodeficiency?

<p>Abstract</p> <p>Goldenhar syndrome (GS) results from an aberrant development of the 1^st and 2^nd branchial arches. There is a wide range of clinical manifestations, the most common being microtia, hemifacial microsomia, epibulbar dermoids and vertebral malformations. We present two cases of GS and secondary immunodeficiency due to anatomical defects characteristic of this disorder. Case 1 (3-year-old female) averaged 6 episodes of sinusitis and otitis media per year. Case 2 (7-year-old female) also had recurrent otitis media, an episode of bacterial pneumonia, and 2 episodes of bacterial meningitis. Their immune evaluation included a complete blood count with differential, serum immunoglobulin levels and specific antibody concentrations, lymphocyte phenotyping, and mitogen and antigen responses, the results of which were all within normal ranges. Both children demonstrated major structural abnormalities of the inner and middle ear structures, retention of fluid in mastoid air cells, and chronic sinusitis by computed tomography. These two cases illustrate how a genetically-associated deviation of the middle ear cleft can cause recurrent infections and chronic inflammation of the middle ear and adjacent sinuses, even meninges, leading to a greatly reduced quality of life for the child and parents.</p

Split temporalis muscle flap anatomy, vascularization and clinical applications

Introduction : For more than a century, the temporalis muscle has been used for facial reconstructions. More recently, a split temporalis muscle flap elevated on the superficial temporal pedicle has been described, for which the resulting gain of length makes crossing of the midline possible, as well as reconstruction of substance losses exceeding the midline. Materials and methods : Fourteen fresh cadaveric dissections were performed to study the different techniques for splitting the temporalis muscle. Dissections with catheterization and injection of radio-opaque contrasting agent in the external carotid artery were then performed to specify the vascularization of the flap split on the superficial temporal pedicle. Results : The duplication of the superficial temporal pedicle grants greater length compared to that of the deep pedicles, 57 mm versus 40 (p = 0.036). The middle temporal artery is capable of ensuring the vascularization, and therefore the viability, of the split flap. From these results, we spoke about the limitations of this study and we have inferred the main indications