RESUMEN
BACKGROUND Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5 cases per million children ages 0-15 years old. The most common places for this tumor are the jaw, vertebra, pelvis, and the extremities. The disease with multisystem involvement can present a mortality rate of 20% and one-third of children have multisystem involvement. We present a case with unusual bone involvement of the anterior cranial base with a challenging diagnosis and a complex surgical approach. CASE REPORT We report the case of a 6-year-old boy who manifested the disease with daily holocranial headache, worse in the frontal region and refractory to analgesia for 10 days, strabismus homonymous, diplopia, and right palpebral ptosis. The tumor affected the sphenoid sinus, internal carotid artery, and sella turcica, and made contact with the pituitary gland. A joint surgery with Otorhinolaryngology and Neurosurgery was performed by nasal endoscopic access to the skull base by means of the right medial turbinectomy (for the access) and right sphenoid opening, septectomy and opening of the left sphenoid to work with 4 hands and, after resection of lesion, inside the sphenoid. CONCLUSIONS This patient had rare bone involvement from LCH and atypical clinical presentation next to the important and delicate structures of the anterior skull base, but had a satisfactory outcome.
Asunto(s)
Histiocitosis de Células de Langerhans , Seno Esfenoidal , Humanos , Histiocitosis de Células de Langerhans/diagnóstico , Histiocitosis de Células de Langerhans/complicaciones , Histiocitosis de Células de Langerhans/cirugía , Masculino , NiñoRESUMEN
BACKGROUND Periorbital abscesses are uncommon complications of acute bacterial rhinosinusitis; with the evolution of diagnostic and therapeutic methods, it is rare that the patient progresses to irreversible blindness. Central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) rarely occur simultaneously and the factors that influence this occurrence are not well understood. CASE REPORT This is a case report of an immunocompetent healthy adolescent girl, who developed irreversible blindness caused by a periorbital abscess secondary to acute bacterial rhinosinusitis due to CRAO and CRVO. Despite 6 days of clinical treatment, including intravenous antibiotics (vancomycin-associated piperacillin with tazobactam), she had a large periorbital abscess and could not open her left eye. Therefore, she was transferred to a tertiary hospital; 1 day after her admission, she underwent surgical treatment to drain the abscess through external and endoscopic access. In addition, she received broad-spectrum antibiotics (meropenem with vancomycin) for 3 weeks. She was no longer able to perceive light with the left eye, despite her clinical improvement. This case report discusses the factors that could have contributed to this poor outcome, despite clinical and surgical treatment. CONCLUSIONS We conclude that there are several mechanisms that can lead to the loss of vision and when the indicated surgical intervention is delayed, it can increase the risk of visual sequelae.