Lesiones sincrónicas selares: adenoma hipofisario y quiste de la bolsa de Rathke / Collision sellar lesions: pituitary adenoma and Rathke cleft cyst

Resumen El quiste de la bolsa de Rathke (QBR) es una entidad benigna de crecimiento lento que proviene del remanente del ectodermo primitivo. Presenta un origen común con los adenomas hipofisarios (AH), sin embargo, la presentación sincrónica de un AH y un QBR es infrecuente. Presentamos el caso de una mujer de 41 años con enfermedad de Cushing. Se realizó resonancia magnética con el hallazgo de dos lesiones en región selar. Se hizo un abordaje transnasal endoscópico, con exéresis completa de ambas. El informe de anatomía patológica reveló un AH corticotropo y un QBR. Tuvo una remisión clínica analítica endocrinológica a los seis meses postquirúrgicos. Ante el hallazgo de una imagen quística a nivel selar concomitante con un adenoma hipofisario, debe ser considerada la posibilidad diagnóstica de un QBR.

Abstract Rathke's cleft cyst (RCC) are a slow-growing, benign, cystic lesions that arises from the remnants of the primitive ectoderm and Rathke's pouch. They present a common origin with pituitary adenomas (PA), however, the concomitant presentation of a PA and a RCC rarely occur. We present a case of a 41-year-old female with Cushing's disease. Magnetic resonance imaging (MRI) showed two synchronic lesions in the sellar region. An endoscopic transnasal approach was performed, with complete excision of both. The histological studies revealed an ACTH secreting PA and a RCC. The patient presented clinical and endocrinological remission six months after surgery. With the presence of cystic lesion at the sellar region, and the concomitant finding of a pituitary adenoma, RCC should be considered.

Transpalatine Route for the Correction of Transsphenoidal Encephalocele in a Child: A Case Report and Literature Review

Introduction Transsphenoidal encephalocele is a rare neural tube defect characterized by the herniation of meninges and eventually of parts of the brain through a bony defect in the sphenoid bone. The clinical presentation is variable, and surgical treatment is controversial. Case Report This report describes the case of an 8-month-old female child diagnosed with transsphenoidal encephalocele. The child presented with obstruction of the upper airways and was fed via a nasogastric tube but did not present changes in the hypothalamic-pituitary-axis. The patient underwent surgery with the transsphenoidal­transpalatine route, with an excellent outcome and without fistulas or infections. Conclusions Although transsphenoidal encephalocele is a rare congenital anomaly, the transsphenoidal­transpalatine route for the correction of this type of encephalocele is a safe option and produces a favorable outcome in pediatric patients.

Introdução A encefalocele transesfenoidal é um defeito do tubo neural raro, caracterizado por herniação de meninges e eventualmente partes do cérebro através de uma falha óssea no osso esfenoide. A apresentação clínica é variável e o tratamento cirúrgico é controverso. Relato de Caso No presente trabalho descrevemos o caso de uma criança de 8 meses, sexo feminino, com diagnóstico de encefalocele transesfenoidal. Apresentava-se com obstrução de vias aéreas superiores, alimentando-se por sonda nasoenteral e sem alterações do eixo hipotálamo-hipofisário. Tratada com cirurgia via transesfenoidal transpalatal, evoluiu com excelente resultado, sem fístulas e sem infecções. Conclusões Embora a encefalocele transesfonoidal seja uma anomalia congênita rara, a via transpalatina-transesfenoidal para correção deste tipo de encefalocele é uma opção segura e produz um resultado favorável no paciente pediátrico.

Transsphenoidal Approach to Pituitary Adenomas: from the Microscope to the Endoscope

Objective Report the transitioning from the usage of microscope to endoscope in the transsphenoidal approach to pituitary adenomas with a main focus on technical nuances and incorporation of new surgical instruments throughout several years. Methods Between 1993 and 2015, the Skull Base team of Hospital das Clínicas of UFMG operated on 225 pituitary adenomas through a transsphenoidal approach. The study was divided into 3 groups: the first group (from 1993 to 2001) used the microscope only; the second group (from 2002 to 2004) used both the microscope and endoscope simultaneously, and the third group (from 2005 to 2015) used the endoscope only. Results There were 120 functional adenomas and 98 non-functional pituitary tumors; 7 cases could not be classified. The first two groups consisted of 50 cases, as 175 cases were operated with the endoscope only. As the transseptal moved to the endonasal route, it allowed the procedure to be performed by three or four hands, better visualization of the lateral sella, approach to the cavernous sinus and treatment of CSF leaks with a vascularized graft from the septal mucosa. The surgical instruments were adapted as the microscopic approach was switched to the endoscopic technique. Conclusion Despite the lack of literature data showing superior magnification or lighting when comparing the endoscope to the microscope, we believe the former provides greater comfort and safety in the transsphenoidal approach for the treatment of pituitary adenomas.

Objetivo Relatar a transição do microscópio ao endoscópio no acesso transesfenoidal aos adenomas hipofisários, com ênfase nas nuances técnicas, conceitos e incorporações de instrumentos cirúrgicos ao longo dos anos. Método No período de 1993 a 2015, foram operados 225 adenomas hipofisários por via transesfenoidal pelo grupo de cirurgia de base do crânio do Hospital das Clínicas da UFMG. O estudo foi dividido em três etapas, de acordo com o dispositivo usado para iluminação e magnificação do campo operatório: primeira etapa (1993 a 2001), uso do microscópio; segunda etapa (2002 a 2004), uso simultâneo do microscópio e do endoscópio; terceira etapa (2005 a 2015), uso exclusivo do endoscópio. Resultados Adenomas funcionantes corresponderam a 120 casos, e os não funcionantes, a 98. Sete casos não puderam ser classificados. Cinquenta cirurgias corresponderam às duas primeiras etapas, e 175 foram realizadas exclusivamente com o endoscópio. O acesso migrou do transeptal para o endonasal, o que permitiu a cirurgia a três ou quatro mãos, melhor visualização das porções laterais da sela e abordagem do seio cavernoso, além de tratamento de fístulas com retalho pediculado de mucosa do septo. Os instrumentos cirúrgicos foram adaptados à medida que se trocou o microscópio pelo endoscópio. Conclusão Apesar de a literatura não demonstrar que um tipo de equipamento de iluminação e magnificação seja superior ao outro, acreditamos que o endoscópio nos propiciou maior conforto e segurança no tratamento dos adenomas hipofisários por via transesfenoidal.

Analysis of full endoscopic transnasal-transphenoidal approach for pituitary adenomas : the summary of 72 patients / 中华显微外科杂志

Objective To investigate and evaluate the clinical value of full endoscopic transnasal transphenoidal approach for the surgery of pituitary adenomas.Methods Seventy-two patients,who underwent full endoscopic transnasal transphenoidal approach for the surgery of pituitary adenomas,were selected from the Medical Center of Pituitary Adenomas of our hospital from January 2009 to March 2012.To retrospectively investigate pre- and post-operation symptoms,hormone levels,images information,operation information,complications,following-up information and so on.Results Among the 72 consecutive patients,there were 22 nonfunctioning adenomas,twenty-four prolactin secreting adenomas,seven somatotropin secreting adenomas,five adrenocorticotropic hormone secreting adenomas,one thyrotropin secreting adenomas,and 13 multi-secreting adenomas.The tumor removal was total in 56(77.8％),subtotal 13(18.0％),and partial 3(4.2％).Five cases had CSF leaks,and 6 diabetes insipidus.After 3-24 months of follow-up,the levels of increasing-hormone declined to normal levels in most patients.Conclusion Full endoscopic transnasal transphenoidal approach for the surgery of pituitary adenomas is a kind of technique which is safe,minimally invasive,having less complications and fast recovery.However,it is necessary for surgeons to accept systematic and specialized training,and own advanced equipments.

Evolución del tratamiento neuroquirúrgico de la patología hipofisaria: Experiencia de 800 casos

Los tumores de hipófisis son las neoplasias intracraneales más frecuentes (10 %-15 %). El propósito del trabajo es presentar la experiencia del autor en el manejo neuroquirúrgico de 800 casos de tumores pituitarios, basado en el protocolo de estudio aplicado en las Unidades de Hipófisis de los Hospitales “Miguel Pérez Carreño” y Universitario de Caracas, y en el ejercicio privado del autor. Utilizando inicialmente abordaje trans-craneano y luego abordaje trans-esfenoidal en la mayoría de los casos.Se presentan los hallazgos más frecuentes del material de casos tratados y los procedimientos diagnósticos utilizados, resaltando el uso de resonancia magnética y angiografía cerebral. Se analizan los abordajes trans-craneano y trans-esfenoidal. Se enfatiza la ventaja de la microcirugía trans-esfenoidal y las mejoras introducidas en la técnica quirúrgica y el uso de neuro-endoscopia. Se argumenta la importancia del manejo inter-disciplinario. Estas experiencias permitieron reducir la morbi-mortalidad al acortar el tiempo quirúrgico y hacer el posoperatorio más benigno. Esto se tradujo en una reincorporación más temprana del paciente a su vida normal, mejor calidad de vida y mejor pronóstico.

Pituitary tumors are among the most common intracranial neoplasias (10 % to 15 %). The purpose of this study is to present the author’s experience in the surgical management of 800 pituitary tumors based on the study protocols applied in the Pituitary Units of the Miguel Pérez Carreño Hospital and University Hospital, and in the author’s private practice. The most frecuent findings and diagnostic techniques are presented, highlighting the role of magnetic resonance and cerebral angiography. Trans-sphenoidal and trans-cranial surgical techniques are analyzed. The advantage of the trans-sphenoidal technique, the improvements made to the surgical technique, and the use of neuro-endoscopy are presented. The importance of a multi-specialty team is stressed. This experience allowed to reduce the associated morbidity and mortality by shortening surgical time and improving recovery, quality of life, and prognosis.

Tumores de hipófise: considerações sobre 120 casos e revisão de literatura / Pituitary tumors: considerations about 120 cases and literature review

Objetivo: Estudo retrospectivo dos pacientes com adenoma hipofisário atendidos no Serviço de Neurocirurgia do Conjunto Hospitalar do Mandaqui de São Paulo, SP. Pacientes e métodos: Foram estudados, retrospectivamente, 120 casos de tumores de hipófise atendidos entre janeiro de 1993 e maio de 2006. Os dados analisados foram idade, sexo, queixa principal, apresentação clínica, classificação radiológica, tipo histológico e complicações. Resultados: A idade dos pacientes variou de 5 a 69 anos (média de 40,2 anos). Foram 89 pacientes do sexo feminino e 31 do sexo masculino. O déficit visual foi a queixa principal e presente em 52% dos pacientes. Com relação à classificação radiológica: 5,4%foram classificados como Hardy II; 8,1% como Hardy III; 86,5% como Hardy IV. A via de acesso foi transesfenoidal sublabial em 113 pacientes. Apenas três pacientes necessitaram reabordagem do tumor por craniotomia e sete pacientes foram submetidos à craniotomia sem tentativa transesfenoidal.Os tumores foram diagnosticados como não-secretores em 21,6% dos pacientes, prolactinomas em70,2% dos pacientes, secretor de GH em 5,4% e de ACTH e LH em 2,7% cada um. As principais complicações do tratamento cirúrgico foram diabetes insípido e fístula liquórica. Houve quatro óbitos no pós-operatório. Conclusões: A média etária foi de 40,2 anos; o sexo feminino foi mais acometido;a perda visual foi a principal queixa; a maioria dos pacientes se apresentou em grau IV da classificação de Hardy; o principal tipo histológico foi o prolactinoma; a via transesfenoidal sublabial foi suficiente para a maioria dos tumores.

Objective: Retrospective study of 120 cases of pituitary adenomas treated at Serviço de Neurocirurgiado Conjunto Hospitalar do Mandaqui de São Paulo, SP, Brazil. Patients and methods: 120 cases ofpituitary tumors treated between January, 1993 and May, 2006 were studied retrospectively. The data analyzed were: age, sex, main complaint, clinical presentation, radiological classification, type of hormone and complications. Results: The age of the patients varied between five to sixty-nine years (average of 40.2 years). The female/male ratio was 89:31. The visual deficit was the main complaint and present in 52% of the patients. According to Hardy’s classification, 5.4% were classified as Hardy II; 8.1% as Hardy III and 86.5% as Hardy IV. The surgical access was the transphenoidal in 113 patients. Three patients needed a second approach by craniotomy and seven patients were submitted to craniotomy as the first choice. The tumors were diagnosed as not secretor in 21.6% patients, prolactinomas in 70.2% patients, secretor of GH in 5.4% and ACTH and LH in 2.7% each one. The main complications of the surgical treatment were diabetes insipidus and cerebrospinal fluid leak. There were four deaths in the postoperative period. Conclusions: In this series the average age at presentations was 40.2 years;females were predominant; the visual loss was the main complaint; the majority of the patients were classified as Hardy IV; prolactinoma predominated; the transphenoidal surgical access was enough for the majority of the tumors.