RESUMO
Autoimmune polyglandular syndromes are rare disorders characterized by failure of several endocrine glands, as well as non-endocrine organs, associated with immune-mediated tissue destruction. We report a rare case of polyglandular syndrome type II in a patient who presented with premature ovarian failure, Hashimoto's thyroiditis and empty sella associated with a diagnosis of differentiated thyroid carcinoma. This case probably represents the first report on this tumor in a patient with polyglandular disorder.
Assuntos
Carcinoma/complicações , Poliendocrinopatias Autoimunes/complicações , Poliendocrinopatias Autoimunes/fisiopatologia , Neoplasias da Glândula Tireoide/complicações , Adulto , Carcinoma/cirurgia , Síndrome da Sela Vazia/etiologia , Feminino , Doença de Hashimoto/etiologia , Terapia de Reposição Hormonal , Humanos , Poliendocrinopatias Autoimunes/tratamento farmacológico , Insuficiência Ovariana Primária/etiologia , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia/efeitos adversos , Resultado do TratamentoRESUMO
BACKGROUND: The surgical management of anterior skull base cerebrospinal fluid (CSF) fistulas has evolved throughout the last decade. Endonasal endoscopic surgery of the anterior skull base has become the standard procedure for the repair of most these fistulas. OBJECTIVES: To describe the presenting symptoms, etiology, treatment, and long-term outcomes of patients with anterior skull base CSF fistulas treated endoscopically at our institution. MATERIAL/ METHODS: Retrospective analysis of 25 patients with CSF fistulas treated endoscopically at the University of Puerto Rico Hospital from November 2004 to August 2008. RESULT: The etiology was spontaneous leak in 10 patients, menigoencephalocele in 7 patients, previous sinus surgery in one patient, and trauma in one patient. The most common location of leak was the cribiform plate, followed by the ethmoid roof. An overlay technique was used for repair in 61% of the procedures versus 39% for the underlay technique. The mean follow up was 23 months. Our overall initial rate of closure was 94% with 100% after a second procedure. CONCLUSIONS: Endoscopic repair of anterior skull base CSF fistulas has a high success rate and lower morbidity and mortality when compared with open approaches.
Assuntos
Rinorreia de Líquido Cefalorraquidiano/cirurgia , Endoscopia , Procedimentos Cirúrgicos Otorrinolaringológicos , Base do Crânio/cirurgia , Adolescente , Adulto , Idoso , Rinorreia de Líquido Cefalorraquidiano/diagnóstico , Rinorreia de Líquido Cefalorraquidiano/etiologia , Síndrome da Sela Vazia/etiologia , Encefalocele/complicações , Osso Etmoide/patologia , Osso Etmoide/cirurgia , Seio Etmoidal/patologia , Seio Etmoidal/cirurgia , Feminino , Cefaleia/etiologia , Humanos , Masculino , Meningite/etiologia , Meningocele/complicações , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Base do Crânio/patologia , Seio Esfenoidal/patologia , Seio Esfenoidal/cirurgia , Transplante Autólogo , Resultado do TratamentoRESUMO
An ectopic pituitary adenoma, exclusively involving the sphenoid sinus, associated with a normal size pituitary gland and an empty sella is a rare clinical entity. Only four cases have been found in the literature. We describe the case of a 47-year-old woman with clinical and biochemical acromegaly. Computed tomography (CT) appeared to show expansion of the sellar floor inferiorly into the sphenoid sinus, and magnetic resonance imaging (MRI) demonstrated an empty sella and a mass lesion confined to the sphenoid sinus. The tumor was totally resected by taking a transnasal endoscopic approach and the dura mater of the base of the pituitary fossa was confirmed intact.
Assuntos
Acromegalia/etiologia , Adenoma/complicações , Coristoma/complicações , Síndrome da Sela Vazia/etiologia , Hormônio do Crescimento Humano/metabolismo , Neoplasias dos Seios Paranasais/complicações , Neoplasias Hipofisárias/complicações , Seio Esfenoidal , Adenoma/diagnóstico , Coristoma/diagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Neoplasias dos Seios Paranasais/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Tomografia Computadorizada por Raios XRESUMO
Turner's syndrome (TS) is associated with a wide spectrum of clinical features, such as short stature and gonadal dysgenesis. While it is a common chromosomal abnormality, the association of Turner's syndrome and hypopituitarism is an uncommon finding. We describe here a girl with concomitant pituitary insufficiency and gonadal dysgenesis. When she was 7 years old, her mother reported that she suffered from frontal headache, asthenia and delayed growth. Basal laboratory thyroid evaluation suggested hypothyroidism, with no evidence of autoimmune disease association. She began taking L-thyroxine. At age 11 years, short stature and complaints of frontal headache still persisted. She was still prepubertal and her bone age was delayed by 2.2 years. Her karyotype was compatible with 45,X/46,XX (100 cells analyzed by FISH) and a CT scan showed empty sella. At 12 years of age, an anterior pituitary stimulation test with insulin, gonadotropin-releasing hormone (GnRH) and thyrotropin-releasing hormone (TRH) showed gonadotropin, thyrotropin (TSH) and growth hormone (GH) deficiency. Replacement therapy with GH was begun and she grew 12 cm during the first year of treatment. This report illustrates that, despite the high incidence of sinusitis, short stature and primary hypothyroidism in TS, we should consider the presence of hypopituitarism when the patient presents low levels of TSH with negative thyroid antibodies and inappropriately low levels of gonadotropins for patients with gonadal dysgenesis.