Embolia de líquido amniótico asociada a paro cardiorrespiratorio recuperado y síndrome de Sheehan / Amniotic fluid embolism associated with recovered cardiac arrest and postpartum Sheehan's syndrome

Resumen La embolia de líquido amniótico es una condición catastrófica propia del embarazo que ocurre típicamente durante el parto o justo posterior a este, cuyo sustrato fisiopatológico no ha sido aclarado por completo. Se ha estimado, según cifras de los Estados Unidos, que su incidencia rondaría 1 por cada 12.953 partos, y en el Reino Unido 1 por cada 50.000 partos; sin embargo, estas cifras pueden ser imprecisas debido a que no existen una referencia ni un consenso respecto a los criterios diagnósticos, además de que el cuadro clínico se puede confundir con otras emergencias obstétricas. Se presenta el caso de una paciente sin antecedentes mórbidos que presenta un cuadro de embolia de líquido amniótico no fatal, caracterizado por un estado fetal no tranquilizador durante la inducción del trabajo de parto, seguido de un paro cardiorrespiratorio durante la cesárea de urgencia y la rápida y catastrófica aparición de signos clínicos de una coagulopatía de consumo grave. Se describen además las complicaciones posoperatorias y su manejo, entre ellas un síndrome de Sheehan y la aparición de convulsiones tónico-clónicas generalizadas con alteración de neuroimágenes.

Abstract Amniotic fluid embolism is a catastrophic pregnancy condition that typically occurs during or inmediately after delivery, and whose pathophysiological background has not been fully clarified. According to US records the incidence of amniotic fluid embolism could been around 1 for every 12,953 births and in the United Kingdom 1 for every 50,000 births, however these numbers may be imprecise because there is no gold standard as well as no consensus regarding the diagnostic criteria, in addition that the clinical presentation can be misdiagnosis with other obstetric emergencies. We present the clinical case of a patient without a morbid history who presents with a non-fatal amniotic fluid embolism, characterized by an non-reassuring fetal status during labor induction, followed by cardiorespiratory arrest during emergency cesarean section and the rapid and catastrophic appearance of clinical signs of a severe consumptive coagulopathy. Postoperative complications and their management are also described, including Sheehans syndrome and the appearance of generalized tonic-clonic seizures with impaired neuroimaging.

Management of hypopituitarism: a perspective from the Brazilian Society of Endocrinology and Metabolism

ABSTRACT Hypopituitarism is a disorder characterized by insufficient secretion of one or more pituitary hormones. New etiologies of hypopituitarism have been recently described, including head trauma, cerebral hemorrhage, and drug-induced hypophysitis. The investigation of patients with these new disorders, in addition to advances in diagnosis and treatment of hypopituitarism, has increased the prevalence of this condition. Pituitary hormone deficiencies can induce significant clinical changes with consequent increased morbidity and mortality rates, while hormone replacement based on current guidelines protects these patients. In this review, we will first discuss the different etiologies of hypopituitarism and then address one by one the clinical aspects, diagnostic evaluation, and therapeutic options for deficiencies of TSH, ACTH, gonadotropin, and GH. Finally, we will detail the hormonal interactions that occur during replacement of pituitary hormones.

Hipopituitarismo postraumatismo encefalocraneano: revisión de la literatura y algoritmo de estudio y abordaje terapéutico

Hypopituitarism after moderate or severe traumatic brain injury (TBI) is usually underdiagnosed and therefore undertreated. Its course can be divided in an acute phase during the first 14 days after TBI with 50 to 80% risk of hypopituitarism, and a chronic phase, beginning three months after the event, with a prevalence of hypopituitarism that ranges from 2 to 70%. Its pathophysiology has been addressed in several studies, suggesting that a vascular injury to the pituitary tissue is the most important mechanism during the acute phase, and an autoimmune one during chronic stages. In the acute phase, there are difficulties to correctly interpret pituitary axes. Hence, we propose a simple and cost-effective algorithm to detect and treat a potential hypothalamic-pituitary-adrenal axis impairment and alterations of sodium homeostasis, both of which can be life-threatening. In the chronic phase, post-concussion syndrome is the most important differential diagnosis. Given the high prevalence of hypopituitarism, we suggest that all pituitary axes should be assessed in all patients with moderate to severe TBI, between 3 to 6 months after the event, and then repeated at 12 months after trauma by a specialized team in pituitary disease.

Non-functioning pituitary adenomas and pregnancy: one-center experience and review of the literature

SUMMARY The usual clinical presentation of non-functioning pituitary adenoma (NFPA) consists of symptoms of mass effect and hypopituitarism. NFPA is a rare condition in young women and an uncommon complication during pregnancy. We present the outcome of three patients with NFPA during pregnancy. Case 1: a 38-year-old woman was referred at 32nd week of spontaneous pregnancy because of diagnosis of a pituitary macroadenoma discovered in the context of progressive visual loss. Hormonal deficiency and hypersecretion were ruled out. Prolactin levels were high as expected. She developed diplopia and severe headache despite the use of dopamine agonists and corticosteroids, so pregnancy was interrupted at 34th week. After an uncomplicated delivery of a healthy newborn, transsphenoidal surgery was performed. The pathology was consistent with a gonadotroph adenoma. She recovered visual field, and remained with normal pituitary function. Postsurgical tumor remnant increased in size during the follow-up. Case 2: a 34-year-old woman was referred due to secondary amenorrhea and galactorrhea. A macroadenoma with suprasellar extension was discovered. Transsphenoidal surgery confirmed a gonadotroph adenoma. Two years after surgery she had a normal pregnancy. Six years after surgery a small tumor recurrence occurred. Case 3: a 23-year-old woman was referred due to a microincidental pituitary adenoma. Laboratory testing was normal. No findings on physical examination. A wait and see approach was decided. Two years after diagnosis, the patient got pregnant without complications. Image remained stable. This article may contribute new cases and provides an extensive review of NFPA during pregnancy.

Panhipopituitarismo y escotoma perimacular como presentación de enfermedad relacionada a IgG4 / IgG4 related disease presenting as panhypopituitarism and perimacular scotoma

La enfermedad relacionada a IgG4 (IgG4-RD) constituye una entidad sistémica recientemente descrita, de causa desconocida. Afecta predominantemente a hombres mayores y presenta características histopatológicas distintivas, como fibrosis estoriforme, flebitis obliterante y denso infiltrado linfoplasmocitario con inmunomarcación para IgG4, pudiendo estar asociada a elevación sérica de dicha inmunoglobulina. Si bien cualquier órgano puede estar afectado, el compromiso de la hipófisis es infrecuente. Describimos el caso de un hombre de 36 años que se presentó con cefaleas, alteración del campo visual, panhipopituitarismo, diabetes insípida y una imagen que mostraba una lesión infiltrativa infundíbulo-panhipofisaria extendida. Arribamos al diagnóstico de IgG4-RD a través de biopsia hipofisaria. La respuesta al tratamiento con dosis inmunosupresoras de corticoides fue exitosa.

IgG4-related disease (IgG4-RD) is a recently described systemic entity of unknown origin. It predominantly affects older men and has distinctive histopathologic features as storiform fibrosis, obliterative phlebitis, dense lymphoplasmacytic infiltrate with immunostaining for IgG4, and it may be associated with elevated serum levels of IgG4. Although any organ can be affected, pituitary gland is rarely involved. We describe the case of a 36-year-old man who presented with headaches, impaired vision, panhypopituitarism with diabetes insipidus and an infiltrative lesion mainly of infundibulum and pituitary. We arrived at diagnosis of IgG4-RD by pituitary biopsy. A successful response to treatment with immunosuppressive doses of corticosteroids was achieved.

Spontaneous remission of acromegaly: apoplexy mimicking meningitis or meningitis as a cause of apoplexy? / Remissão espontânea da acromegalia: meningite simulando apoplexia ou meningite como causa da apoplexia?

Pituitary apoplexy is a rare but potentially life-threatening clinical syndrome characterized by ischemic infarction or hemorrhage into a pituitary tumor. The diagnosis of pituitary tumor apoplexy is frequently complicated because of the nonspecific nature of its signs and symptoms, which can mimic different neurological processes, including meningitis. Several factors have been associated with apoplexy, such as dopamine agonists, radiotherapy, or head trauma, but meningitis is a rarely reported cause. We describe the case of a 51-year-old woman with acromegaly due to a pituitary macroadenoma. Before surgical treatment, she arrived at Emergency with fever, nausea, vomiting and meningismus. Symptoms and laboratory tests suggested bacterial meningitis, and antibiotic therapy was initiated, with quick improvement. A computerized tomography (CT) scan at admission did not reveal any change in pituitary adenoma, but a few weeks later, magnetic resonance imaging (MRI) showed data of pituitary apoplexy with complete disappearance of the adenoma. Currently, her acromegaly is cured, but she developed hypopituitarism and diabetes insipidus following apoplexy. We question whether she really experienced meningitis leading to apoplexy or whether apoplexy was misinterpreted as meningitis. In conclusion, the relationship between meningitis and pituitary apoplexy may be bidirectional. Apoplexy can mimic viral or bacterial meningitis, but meningitis might cause apoplexy, as well. This fact highlights the importance of differential diagnosis when evaluating patients with pituitary adenomas and acute neurological symptoms.

A apoplexia é uma síndrome clínica rara, mas potencialmente fatal, caracterizada por infarto isquêmico ou hemorragia em um tumor pituitário. O diagnóstico de apoplexia de tumor pituitário é frequentemente complicado pela natureza inespecífica dos seus sinais e sintomas, que podem simular diferentes processos neurológicos, incluindo a meningite. Vários fatores estão associados com a apoplexia, como o uso de agonistas dopaminérgicos, radioterapia ou trauma da cabeça, mas a meningite foi raramente relatada. Descrevemos o caso de uma mulher de 51 anos de idade com acromegalia por um macroadenoma pituitário. Antes do tratamento cirúrgico, ela foi trazida ao pronto-socorro com febre, náusea, vômitos e meningismo. Os sintomas e análises laboratoriais sugeriram meningite bacteriana e o tratamento com antibióticos foi iniciado, com melhora rápida dos sintomas. Uma tomografia computadorizada (CT) na admissão ao hospital não revelou nenhuma alteração no adenoma pituitário, mas algumas semanas depois uma ressonância magnética (MRI) mostrou informações de apoplexia pituitária, com desaparecimento completo do adenoma. Atualmente, a acromegalia está curada, mas ela desenvolveu hipopituitarismo e diabetes insipidus depois da apoplexia. Questionamo-nos se a paciente realmente apresentou meningite que levou à apoplexia ou se a apoplexia foi mal interpretada como sendo meningite. A relação entre a meningite e a apoplexia pode ser bidirecional. A apoplexia pode simular a meningite viral ou bacteriana, mas a meningite também pode causar apoplexia. Esse fato enfatiza a importância do diagnóstico diferencial ao se avaliar pacientes com adenomas pituitários e sintomas neurológicos.

An intrasellar germinoma with normal tumor marker concentrations mimicking primary lymphocytic hypophysitis / Germinoma intrasselar com marcadores tumorais normais mimetizando hipofisite linfocítica primária

Intracranial germinomas (GE) are malignant neoplasms most commonly found in the suprasellar region, which may cause anterior and particularly posterior pituitary hormone deficits with central diabetes insipidus (DI). Differential diagnosis of pituitary stalk thickening includes granulomatous, inflammatory, infectious, and neoplastic lesions. Although careful analysis of clinical, laboratory, and imaging findings may facilitate the diagnosis, transsphenoidal biopsy is indicated to confirm the disease, as the correct diagnosis directs the appropriate treatment.

Germinomas intracranianos (GE) são neoplasias malignas comumente na região suprasselar, podendo causar deficiência hormonal da hipófise anterior, em particular da hipófise posterior, com diabetes insípido central (DI). Entre os diagnósticos diferenciais do espessamento de haste hipofisária, incluem-se doenças granulomatosas, inflamatórias, infecciosas e neoplásicas. Embora as avaliações clínica, laboratorial e a ressonância magnética selar sugiram o diagnóstico, a biópsia transesfenoidal está indicada para confirmação, visto que o diagnóstico correto direciona o tratamento.

Pseudo-tumor pituitario e hipopituitarismo secundario a un papiloma invertido del seno esfenoidal / Pseudo-pituitary tumor and hypopituitarism secondary to a sphenoid sinus inverted papilloma

El papiloma invertido (PI) es un tumor epitelial benigno, poco frecuente, que se origina mayormente de la pared nasal lateral. A pesar de ser benigno, constituye una lesión altamente invasiva de tejidos vecinos y puede sufrir una transformación maligna. El PI primario del seno esfenoidal con extensión intracraneana e invasión dural, aun sin evidencia histológica de malignidad, ha sido excepcionalmente descrito. Describimos el caso de una mujer de 59 años de edad que fue evaluada por cefaleas intensas de 5 años de evolución y anormalidades del campo visual. Una resonancia magnética nuclear (RMN) mostró una masa selar heterogénea de 1.4 por 2 cm con extensión supraselar y al seno esfenoidal, con erosión del piso selar y compresión del quiasma óptico. Recibió 16 mg/día de prednisona durante aproximadamente 3 meses con una regresión casi total de la masa en la RMN. En la evaluación hormonal se halló insuficiencia gonadal, tiroidea y adrenal central. En una nueva RMN se observó crecimiento del tumor con compromiso total del seno esfenoidal. Una biopsia endoscópica confirmó el diagnóstico de PI. Se realizó una cirugía sinusal transnasal endoscópica con una resección completa evidenciada en una RMN un año más tarde.

nverted papilloma (IP) is a benign uncommon epithelial tumor, arising mostly from the lateral nasal wall. Though benign, this lesion is highly invasive into surrounding tissues and malignant transformation may occur. Primary IP of the sphenoid sinus and intracranial extension with dural invasion, even without histological evidence of malignancy, has only rarely been described. Hypopituitarism as a complication of this lesion has never been reported. We describe the case of a 59-year-old woman who was evaluated because of a 5-year-history of severe headaches and abnormalities in the visual field. Magnetic resonance imaging (MRI) showed a 1.4 per 2.0 cm heterogeneous sellar lesion with suprasellar and sphenoid sinus extension, eroding the sellar floor with optic chiasm compression. Otolaryngologists gave her 16 mg/day of prednisone during approximately 3 months with a near total regression of the mass on MRI. The endocrine biochemical evaluation showed pituitary gonadal, thyroid and adrenal insufficiency. A new MRI showed growth of the tumor with obliteration of the sphenoid sinus. An endoscopic sinus biopsy revealed an IP, so a transnasal endoscopic sinus surgery was performed with complete resection evidenced by MRI a year later.

Pituitary deficiency after aneurysmal subarachnoid hemorrhage

OBJECTIVE: Aneurysmal subarachnoid hemorrhage puts patients at high risk for the development of pituitary insufficiency. We evaluated the incidence of pituitary dysfunction in these patients and its correlation with clinical outcome. METHODS: Pituitary function was tested in 66 consecutive patients in the first 15 days after aneurysmal subarachnoid hemorrhage. The following were measured in all patients: thyroid-stimulating hormone, free thyroxine, triiodothyronine, luteinizing hormone, follicle-stimulating hormone, total testosterone (in males), estradiol (in females), prolactin, serum cortisol, plasma adrenocorticotropic hormone, growth hormone and insulin growth factor. RESULTS: The endocrine assessment was made at a mean of 7.4 days (standard deviation ±6.6) after subarachnoid hemorrhage. Forty-four (66.7%) female and 22 (33.3%) male patients were evaluated. Thirty-nine patients (59.1%) had some type of pituitary dysfunction. Follicle-stimulating hormone/luteinizing hormone deficiency was the most frequent disorder (34.8%), followed by growth hormone/insulin growth factor (28.7%), adrenocorticotropic hormone (18.1%) and thyroid-stimulating hormone (9%). Seventeen (25.7%) patients showed deficiencies in more than one axis. A greater incidence of hormone deficiency was observed in patients with a Glasgow Coma Scale score ≤13 (t test, p = 0.008), Hunt-Hess grade ≥4 (t test, p<0.001), or Fisher grade 4 (t test, p = 0.039). Hormone deficiency was not significantly associated (p>0.05) with increased hospitalization or clinical outcome. CONCLUSION: Pituitary dysfunction was identified in a substantial portion of patients with previous aneurysmal subarachnoid hemorrhage, but no association was found between this dysfunction and poor clinical outcome. .

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.

Panhypopituitarism due to Wegener's granulomatosis / Pan-hipopituitarismo causado pela granulomatose de Wegener

Wegener's granulomatosis (WG) is a multi-system necrotizing granulomatous vasculitis which classically affects the upper respiratory tract, lungs and kidneys. Pituitary participation has been described in 24 patients in the literature to date. The aim of this article is to report a case of pituitary involvement in WG, and to present a literature review on this association. We present a female patient with WG who evolved with central diabetes insipidus (CDI), panhypopituitarism, and mild hyperprolactinemia. MRI showed an infiltrative pattern. Pituitary involvement has been reported in around 1 percent of patients with WG, mostly in women. It is represented by CDI and hypopituitarism. MRI generally shows pituitary enlargement, stalk thickening and loss of hyperintensity of the neurohypophysis. Permanent endocrine therapy is generally needed. WG should be considered in cases of CDI and hypopituitarism, essentially if a vasculitis is suspected and more common sellar disorders have been ruled out.

A granulomatose de Wegener (GW) é uma vasculite necrotizante multissistêmica que afeta classicamente o trato respiratório superior, pulmões e rins. O envolvimento da hipófise foi descrita em 24 pacientes na literatura, até hoje. O objetivo deste artigo é relatar um caso de GW com envolvimento pituitário, e apresentar uma revisão da literatura sobre esta associação. Apresentamos uma paciente com GW que evoluiu para diabetes insipidus central (DIC), panhipopituitarismo e leve hiperprolactinemia. A RM mostrou um padrão infiltrativo. O envolvimento da hipófise foi relatado em cerca de 1 por cento dos pacientes cm GW, na sua maioria mulheres. A desordem é representada por DIC e hipopituitarismo. A RM geralmente mostra o aumento da hipófise, aumento da espessura da haste, e perda da hiperintensidade da neurohipofise. Normalmente, é necessária terapia endócrina permanente. A GW é geralmente considerada nos casos de DIC e hipopituitarismo, essencialmente se há suspeita de vasculite e quando desordens selares mais comuns foram descartadas.

Septo-optic dysplasia / Displasia septo-óptica

Septo-optic dysplasia (SOD), also referred to as de Morsier syndrome, is a rare congenital condition, characterized by two of the classic triad features: midline brain abnormalities, optic nerve hypoplasia (ONH) and pituitary endocrine dysfunction. We report 5 children with SOD, originally referred to be evaluated due to short stature, who also presented bilateral optic nerve hypoplasia, nystagmus and development delay. In 4 of the patients, we identified neuroimaging abnormalities of the hypothalamo-pituitary axis such as anterior pituitary hypoplasia (3/5), ectopic posterior pituitary (4/5), thin or absent stalk (3/5) and empty sella (1/5). We also encountered diverse pituitary deficiencies: growth hormone (3/5), adrenocorticotropic hormone (3/5), thyroid-stimulating hormone (2/5) and antidiuretic hormone (1/5). Only one child presented intact pituitary function and anatomy. Although rare, SOD is an important cause of congenital hypopituitarism and it should be considered in children with optic nerve hypoplasia or midline brain abnormalities for early diagnosis and treatment.

A displasia septo-óptica (DSO, síndrome de Morsier) é uma condição congênita rara definida por dois critérios da tríade: defeitos de linha média, hipoplasia de nervo óptico e insuficiência hipotálamo-hipofisária. Descrevemos 5 casos de DSO, encaminhados por baixa estatura, com hipoplasia dos nervos ópticos, nistagmo e atraso global do desenvolvimento. Destes, 4 possuem alteração na ressonância magnética de sela túrcica e insuficiência hipotálamo-hipofisária, tendo sido observada adeno-hipófise hipoplásica (3/5), neuro-hipófise ectópica (4/5), haste afilada/ausente (3/5) e sela vazia (1/5), além de déficit dos eixos somatotrófico (3/5), adrenocorticotrófico (3/5), tireotrófico (2/5) e do hormônio antidiurético (1/5). Apenas um paciente não apresenta alteração anatômica ou funcional do eixo hipotálamo-hipofisário. Embora rara, a DSO é causa importante de hipopituitarismo congênito, devendo ser considerada em crianças com hipoplasia de nervo óptico ou defeito de linha média para seu diagnóstico e tratamento precoces, evitando seqüelas graves.

Non-functioning pituitary adenomas: clinical feature, laboratorial and imaging assessment, therapeutic management and outcome / Adenomas hipofisários não-secretores: avaliações clínica, laboratorial, neuro-radiológica e evolução após tratamento

OBJECTIVE: This study is an updated review of a Southeast Brazilian experience NFPA, emphasizing clinical features, laboratorial and imaging assessment, therapeutic management and outcome. DESIGN AND METHODS: Retrospective study, in which 104 patients with NFPA were evaluated by the same team of endocrinologists and neurosurgeon. Patients underwent biochemical evaluation, radiological studies and visual field assessment. RESULTS: Hypopituitarism and neuro-ophthalmological defects were observed in 89 percent. We observed GH deficiency (81.4 percent), hypogonadism (63.3 percent), adrenal hypofunction (59.5 percent), hypothyroidism (20.4 percent), high (38.5 percent) and low (16.7 percent) prolactin levels. Preoperative imaging classified 93 percent of the tumors as macroadenomas. Extra-sellar expansion was observed in 83.8 percent. Varying degrees of visual disturbance were observed in 74 percent. Primary treatment was transsphenoidal surgery (75 percent). Clinical control was achieved with one surgery in 37.5 percent of patients. The majority of patients needed a second therapeutic approach, radiotherapy or other surgeries. Immunohistochemistry resulted negative for pituitary hormones in 43 percent. Improvement of neuro-ophthalmological symptoms was observed in 61 percent of the patients after treatment. CONCLUSIONS: Our data confirm elevated prevalence of mass effect and hypopituitarism in patients harboring NFPA. Recurrence due to invasion or incomplete resection of the tumor is quite common, which frequently leads to a second therapeutic option.

OBJETIVOS: Esse estudo é uma revisão atualizada em adenomas hipofisários não-secretores (AHNS), enfatizando características clínicas, avaliações laboratorial e neuro-radiológica, manejo terapêutico e desfecho. MÉTODOS: Estudo retrospectivo, em que 104 pacientes com AHNS foram avaliados pela mesma equipe de endocrinologistas e neurocirurgião. Os pacientes foram submetidos à avaliação bioquímica, a estudos radiológicos e à avaliação de campo visual. RESULTADOS: Hipopituitarismo e distúrbios neuro-oftalmológicos foram observados em 89 por cento. Observou-se deficiência de GH (81,4 por cento), hipogonadismo (63,3 por cento), hipofunção adrenal (59,5 por cento), hipotireoidismo (20,4 por cento), concentrações elevadas (38,5 por cento) e baixas (16,7 por cento) de prolactina. Imagens pré-operatórias classificaram os tumores em sua maioria como macroadenomas (93 por cento). Expansão extra-selar foi observada em 83,8 por cento. Graus variados de distúrbios visuais foram observados em 74 por cento. O tratamento de escolha foi a cirurgia transesfenoidal (75 por cento). O controle clínico foi alcançado com cirurgia em 37,5 por cento dos pacientes. A maioria dos pacientes precisou de segunda abordagem terapêutica, radioterapia ou outras cirurgias. A imunoistoquímica resultou negativa para hormônios hipofisários em 43 por cento. Melhora de sintomas neuro-oftalmológicos foi observada em 61 por cento dos pacientes após o tratamento. CONCLISÕES: Os dados do estudo confirmam elevada prevalência de efeitos de massa e hipopituitarismo em pacientes com AHNS. Recorrência causada por invasão ou ressecção incompleta do tumor é comum, levando frequentemente à segunda opção terapêutica.

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.

Aneurisma gigante de carótida interna simulando macroadenoma de hipófise / Giant internal carotid artery aneurism simulating pituitary adenoma

O aneurisma gigante com extensão selar é uma causa rara de hipopituitarismo, e está freqüentemente associado com aterosclerose, displasia fibromuscular ou radioterapia hipofisária. O presente caso é de uma paciente de 78 anos com aneurisma gigante de carótida interna esquerda, somente diagnosticado devido a quadro de pan-hipopituitarismo e síndrome de nervos cranianos (compressão dos nervos abducente e ópticos). A etiologia foi definida após tomografia computadorizada, ressonância nuclear magnética e arteriografia cerebral compatíveis com aneurisma. Ficou demonstrada associação com aterosclerose e displasia fibromuscular pela arteriografia. Não foi realizado tratamento cirúrgico após avaliação de risco/benefício. Obteve-se melhora clínica significativa após instituição de reposição hormonal com glicocorticóide e levotiroxina.

Abordaje transcalloso de craneofaringioma supraselar / Carniopharyngioma suprasellar transcallosal approach

El craniofaringioma es una neplasia epitelial benigna usualmente de origen supraselar, considerado un tumor hipofisiario de crecimiento lento, puede causar varios grados de hipopituitarismo. En este artículo se describe un caso manejado en el Hospital Dr. Patrocinio Peñuela Ruíz (IVSS) y se realiza una revisión bibliográfica acerca de la presentación, patología y tratamiento. Escolar masculino de 8 años, inicia enfermedad de 1 mes de evolución caracterizada por cefalea frontal de moderada intensidad, disminución de la agudeza visual, movimientos reiterativos a predominio de hemicuerpo derecho. En una primera intervención se coloca válvula de derivación ventrículo peritoneal por hidrocefalia obstructiva: al mes y medio se realizó exéresis completa del tumor mediante abordaje transcalloso. La evolución postoperatoria fue de difícil manejo por presentar alteraciones metabólicas e hidroelectrolíticas. En RMN se evidencia ausencia total del tumor. El trastorno hidroelectrol¡tico se mantuvo a pesar del tratamiento médico: 24 días después del acto quirúrgico, fallece por hipernatremia incohercible. La lesión apareció en nuestro paciente a los 8 años, como una tumoración de 6 por 4 cm. La clínica se presenta 1 mes antes de su ingreso, caracterizada por cefalea, trastornos visuales, movimientos repetitivos a predominio de hemicuerpo derecho sin alteraciones endocrinas al momento del diagnóstico. Mediante abordaje quirúrgico transcalloso se pudo resecar totalmente una tumoración que histopatológicamente determinó un adamantinoma. Sin embargo, el manejo multidisciplinario es condición sine cua non para terminar de lograr el éxito quirúrgico en cirugías tan d¡fíciles como la del paciente.

Pregnancy in a patient with hypopituitarism following surgery and radiation for a pituitary adenoma.

This is a case of partial hypopituitarism resulting from surgery and radiation for a non- functioning pituitary macroadenoma. The patient had amenorrhea which was secondary to hypogonadotrophic hypogonadism and had been on L-thyroxine for central hypothyroidism. For pregnancy, ovulation was induced by gonadotrophins and this was followed by an intrauterine insemination. The antenatal period was uneventful and a Caesarean section was done at 33 weeks when the patient presented with preterm labour. Both infant and mother are well, eight months after delivery.