Nariz en silla de montar poscirugía transesfenoidal de hipófisis. Reconstrucción con cartílago costal / Saddle nose after transsphenoidal pituitary surgery. Reconstruction with rib cartilage

Durante los últimos años, la cirugía endoscópica transesfenoidal se ha convertido en el procedimiento quirúrgico de elección para el tratamiento de tumores hipofisarios. La técnica de abordaje actual es el resultado de una evolución histórica de vía por craneotomía a vía endonasal con ingreso a través del seno esfenoidal. Aunque la cirugía de tumores hipofisarios vía transesfenoidal endoscópica ha permitido disminuir las complicaciones graves asociadas a los abordajes externos, no está exenta de complicaciones, como la fístula de líquido cefalorraquídeo. A nivel del abordaje nasal, hay escasas descripciones de complicaciones y el compromiso del cartílago septal con deformidad en silla de montar no es una complicación documentada. Se presenta un caso de rinodeformidad en silla de montar poscirugía transesfenoidal de hipófisis en una paciente de 32 años a quien se le practicó una reconstrucción nasal con cartílago costal autólogo. En nuestra búsqueda bibliográfica es el primer caso registrado en Iberolatinoamérica

In recent years, transsphenoidal endoscopic surgery has become the surgical procedure of choice for the treatment of pituitary tumors. The current approach technique is the result of an historical evolution from craniotomy surgery to an endonasal procedure with entry through the sphenoid sinus. Although endoscopic transsphenoidal surgery has made it possible to reduce serious complications associated with external approaches, it is not without complications, such as cerebrospinal fluid fistula. At the level of the nasal approach, there are few descriptions of complications and involvement of the septal cartilage with saddle deformity is not a documented complication. We present a case of saddle rhinodeformity after transsphenoidal pituitary surgery in a 32-year-old patient who underwent nasal reconstruction with autologous costal cartilage. In our bibliographic search, it is the first case registered in IberoLatin America.

Síndromes de neoplasia endocrina múltiple: revisión radiológica / Multiple Endocrine Neoplasia Syndromes: Radiological Review

Los síndromes de neoplasia endocrina múltiple (MEN), incluyen una serie de enfermedades con alteraciones genéticas que se caracterizan por la presencia de tumores que afectan a dos o más glándulas endocrinas. Son síndromes con una herencia autosómica dominante e incluyen tres patrones: MEN 1 (síndrome de Wermer), MEN 2 (que incluye MEN 2A o síndrome de Sipple y MEN 2B o síndrome de Wagenmann-Froboese) y MEN 4. Los adenomas paratiroideos y el carcinoma medular tiroideo, son los tumores más frecuentes del MEN tipo 1 y 2 respectivamente. Esos síndromes son más comunes en pacientes jóvenes, con patología de afectación bilateral, múltiple o multifocal y, sobre todo, en pacientes con antecedentes familiares. Es necesario el trabajo en equipo de endocrinólogos, cirujanos, oncólogos y radiólogos para optimizar el tratamiento de esos pacientes.

Multiple endocrine neoplasia (MEN) encompasses a serial of familial genetically disorders in wich tumors simultaneusly occur in two or more endocrine organs. MEN síndromes are autosomal-dominant disorders categorized into three main patterns: MEN 1 (Wermer syndrome), MEN 2 (includes MEN 2A o Sipple syndrome and MEN 2B o Wagenmann-Froboese syndrome) and MEN 4. Parathyroid adenomas and medullary thyroid carcinoma are the most frecuent tumors in MEN 1 and MEN 2 respectively. These entities will be suspected in younger patients, bilateral, multiple or multifocal disease and, specially, in patients with family background. Cooperation between endocrinologist, surgeons, oncologists and radiologists is pivotal for optimizing patient treatment.

Infundibuloneurohipofisitis: caso clínico y revisión de la literatura / Infundibuloneurohypophysitis: clinical report and literature review

Infundibuloneurohypophysitis is a rare condition, which is part of the group of hypophysitis, of relatively recent description (1993). The main clinical manifestation is diabetes insipidus, whose natural evolution is towards chronicity. The differential diagnosis with other thickening of the hypophysial stem is very important, where the clinic, imaging, laboratory and eventually biopsy are a main support for a correct diagnosis. We present a clinical case that shows the usual picture of infundibuloneurohypophysitis, and illustrates the imaging evolution in a female patient, with diabetes insipidus as the main clinical manifestation

A Case of Idiopathic Granulomatous Hypophysitis

Granulomatous hypophysitis is a rare pituitary condition that commonly presents with enlargement of the pituitary gland. A 31-year-old woman was admitted to the hospital with a severe headache and bitemporal hemianopsia. Magnetic resonance imaging (MRI) showed an 18 x 10-mm sellar mass with suprasellar extension and compression of the optic chiasm. Interestingly, brain MRI had shown no abnormal finding 4 months previously. On hormonal examination, hypopituitarism with mild hyperprolactinemia was noted. The biopsy revealed granulomatous changes with multinucleated giant cells. We herein report this rare case and discuss the relevant literature.

Panhipopituitarismo y diabetes insípida secundarios a hipofisitis linfocitaria en una paciente con trisomia 12p / Panhypopituitarism and diabetes insipidus secondary to a lymphocytic hypophysitis in one patient with trisomy 12p

Lymphocytic hypophysitis (LH) is an uncommon inflammatory disease of the hypophysis. It's female to male ratio of appearance is 9:1. Pregnant women are more affected during the third trimester of pregnancy or postpartum. Clinical and radiological presentation can simulate a hypophyseal adenoma. We report a nonpregnant 13 years old adolescent, with a trisomy 12p, with panhypopituitarism, diabetes insipidus and a selar tumor. It was necessary to differentiate between a germinoma and a LH. The latter was confirmed with the hypophyseal biopsy.

Lymphocytic Hypophysitis with Diabetes Insipidus: Improvement by Methylprednisolone Pulse Therapy

Lymphocytic hypophysitis is a rare inflammatory disorder in the pituitary gland. The lesion is usually confined to the adenohypophysis. Although the involvement of the posterior pituitary gland or the stalk is rare, such patients with diabetes insipidus have been reported. Surgery has been used to make the definitive diagnosis. Recent studies suggest, however, that the pathologic diagnosis may not be necessary always. We reported a case of Lymphocytic hypophysitis managed by methylprednisolone pulse therapy. A 50-year-old premenopausal woman with Lymphocytic hypophysitis and diabetes insipidus was treated with methylprednisolone pulse therapy. Her adenopituitary lesion disappeared and the diabetes insipidus resolved. The optimal management for patients with lymphocytic hypophysitis may be the high index of the suspicion prior to the extensive surgical resection. In addition, methylprednisolone pulse therapy may improve the clinical and MRI findings.

Disseminated Cryptococcosis in a Patient with Pituitary Cushing's Disease

Disseminated cryptococcosis mainly occurs in patients with cell-mediated immunity disorders. A case of disseminated cryptococcosis, in a patient with pituitary Cushing's disease, is reported. Cultures of blood, cerebrospinal fluid (CSF) and aspirates of a skin lesion all grew Cryptococcus neoformans. Despite antifungal treatment, with amphotericin-B, the patient died within 3 weeks.

Intrasellar tuberculoma presenting as pituitary apoplexy.

The combination of apoplectic symptoms and a sellar mass most often points to a diagnosis of a pituitary adenoma. Sellar tuberculomas are not considered as a cause of 'pituitary apoplexy' and there has been no radiological documentation of haemorrhage associated with them. We report a 27 years old man who presented with 3 previous episodes of pituitary apoplexy. CT scan showed evidence of a sellar mass with haemorrhage. Transsphenoidal biopsy of the intrasellar mass was reported as 'tuberculoma'. The patient had marked reduction in the size of the lesion following antituberculous therapy with no recurrence of symptoms. Intrasellar tuberculomas must be considered as one of the differential diagnosis when patients present with a pituitary apoplexy.

Meningite consequente a macroadenoma hipofisário: relato de caso / Meningitis as a complication of pituitary macroadenoma

Os autores relatam um caso de macroprolactinoma que evoluiu com meningite e pneumoencéfalo espontâneo. Discutem-se os possíveis mecanismos dessa evolução.

Hyperprolactinemia associated to calcification of the pituitary stalk: case report

In this work, the authors report the case of a female patient with 24 years of age with hyperprolactinemia, who presented a pituitary stalk calcification as seen by CT scan. Once other possible etiologies were excluded, we concluded that the calcification was probably related to hyperprolactinemia caused by interruption of the input of dopamine to the pituitary gland.