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1.
J. afr. imag. méd ; 13(1): 31-35, 2021. Tables, figures
Article in English | AIM | ID: biblio-1342864

ABSTRACT

Objectif :Décrire le profil épidémiologique et remnologique des pathologies retrouvées chez les patients adressés pour IRM hypophysaire dans le service d'imagerie médicale du CHU-B. Patients et Méthode :il s'agissait d'une étude transversale descriptive, réalisée au service d'imagerie médicale du CHU de Brazzaville de janvier 2014 à décembre 2018. Tous les patients adressés pour IRM hypophysaire étaient inclus. Les examens ont été réalisés sur une IRM de haut champ de 1,5 Tesla sans et avec injection de produit de contraste. Les données épidémiologiques, l'indication et les résultats retrouvés ont été traitées avec le logiciel Microsoft Excel 2013. Résultats :Cinquante-huit IRM étaient retenues durant la période d'étude. L'âge moyen des patients était de 38,24 ± 11,52 ans avec des extrêmes allant de 12 à 74 ans et un âge médian de 36 ans. Le sexe ratio (F/H) était de 4,8 avec 48 femmes (82,76%). La galactorrhée était le motif de consultation le plus fréquemment retrouvé (21,88% des cas), suivie des aménorrhées (18,75%). L'adénome hypophysaire était la pathologie la plus fréquente devant les selles turciques vides et les kystes de la poche de Rathke. Conclusion :La pathologie hypophysaire est l'apanage du sujet jeune de sexe féminin. Son mode de révélation le plus fréquent est celui de troubles gynécologiques et/ou endocriniens. Les adénomes constituent la quasi-totalité de la pathologie hypophysaire avec une nette prédominance de macroadénome. L'IRM apporte des informations essentielles au diagnostic de cespathologies malgré son accessibilité et sa disponibilité qui restent encore limité.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Pituitary Neoplasms , Magnetic Resonance Imaging , Central Nervous System Cysts , Galactorrhea , Pituitary Gland , Congo
2.
Repert. med. cir ; 29(2): 127-130, 2020. ilus., tab.
Article in English, Spanish | COLNAL, LILACS | ID: biblio-1147880

ABSTRACT

Es una lesión quística que surge del remanente epitelial de la bolsa de Rathke, casi siempre su diagnóstico es un hallazgo incidental ya que en la mayoría de los casos es asintomático. Cuando se manifiesta se debe a que ha aumentado lo suficiente su volumen hasta comprimir estructuras vecinas causando cefalea, alteraciones visuales y disfunción pituitaria. En su mayoría ocurre en adultos entre la cuarta y quinta década de vida. Presentamos el caso de una paciente femenina de 9 años de edad que consultó por talla baja al servicio de endocrinología, por lo cual se inició tratamiento con hormona de crecimiento y se solicitó una resonancia magnética nuclear (RMN) la cual reportó quiste de la bolsa de Rathke versus adenoma hipofisario.


Rathke pouch cysts are epithelium-lined cysts arising from the embryological remnants of Rathke ́s pouch. They are usually incidentally identified since the majority are asymptomatic. They become symptomatic when they enlarge enough to compress neighbor structures causing headache, visual disturbances and pituitary dysfunction. They occur mostly in adults in the fourth to fifth decades of life. A case is presented in a 9-year-old female patient who consulted for growth retardation to the endocrinology service. She was treated with growth hormone and a magnetic resonance imaging (MRI) scan reported Rathke ́s pouch cyst versus pituitary adenoma.


Subject(s)
Humans , Female , Child , Pituitary Diseases , Headache , Optic Chiasm , Central Nervous System Cysts , Failure to Thrive
3.
Repert. med. cir ; 29(2): 127-130, 2020. Ilus., tab.
Article in English, Spanish | LILACS, COLNAL | ID: biblio-1222615

ABSTRACT

Es una lesión quística que surge del remanente epitelial de la bolsa de Rathke, casi siempre su diagnóstico es un hallazgo incidental ya que en la mayoría de los casos es asintomático. Cuando se manifiesta se debe a que ha aumentado lo suficiente su volumen hasta comprimir estructuras vecinas causando cefalea, alteraciones visuales y disfunción pituitaria. En su mayoría ocurre en adultos entre la cuarta y quinta década de vida. Presentamos el caso de una paciente femenina de 9 años de edad que consultó por talla baja al servicio de endocrinología, por lo cual se inició tratamiento con hormona de crecimiento y se solicitó una resonancia magnética nuclear (RMN) la cual reportó quiste de la bolsa de Rathke versus adenoma hipofisario.


Rathke pouch cysts are epithelium-lined cysts arising from the embryological remnants of Rathke ́s pouch. They are usually incidentally identified since the majority are asymptomatic. They become symptomatic when they enlarge enough to compress neighbor structures causing headache, visual disturbances and pituitary dysfunction. They occur mostly in adults in the fourth to fifth decades of life. A case is presented in a 9-year-old female patient who consulted for growth retardation to the endocrinology service. She was treated with growth hormone and a magnetic resonance imaging (MRI) scan reported Rathke ́s pouch cyst versus pituitary adenoma.


Subject(s)
Female , Child , Pituitary Diseases , Headache , Optic Chiasm , Central Nervous System Cysts , Failure to Thrive
4.
Rev. chil. endocrinol. diabetes ; 13(2): 61-63, 2020. ilus
Article in Spanish | LILACS | ID: biblio-1095286

ABSTRACT

El quiste de la bolsa de Rathke es una lesión epitelial benigna de la región selar, formada a partir de remanentes embrionarios. La mayoría de los casos son asintomáticos, aunque pudiera presentarse con cefalea, disfunción hipofisaria y trastornos visuales, muy infrecuentemente como apoplejía hipofisaria. Se presenta el caso de una paciente que, habiendo presentado amenorrea primaria, se le realiza el diagnóstico de quiste de la bolsa de Rathke con hiperprolactinemia, logrando menarquia luego del tratamiento con cabergolina.


Rathke's cyst is a benign epithelial lesion of the sellar region, formed from embryonic remnants. Most cases are asymptomatic although it could present with headache, pituitary dysfunction and visual disorders, very infrequently as pituitary stroke. We present the case of a patient who, having presented primary amenorrhea, is diagnosed with Rathke's cyst with hyperprolactinemia, achieving menarche after treatment with cabergoline.


Subject(s)
Humans , Female , Adolescent , Hyperprolactinemia/complications , Central Nervous System Cysts/complications , Amenorrhea/etiology , Prolactin/therapeutic use , Hyperprolactinemia/diagnosis , Hyperprolactinemia/drug therapy , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Central Nervous System Cysts/diagnosis , Central Nervous System Cysts/drug therapy , Cabergoline/therapeutic use
5.
Arq. bras. neurocir ; 38(3): 210-214, 15/09/2019.
Article in English | LILACS | ID: biblio-1362594

ABSTRACT

Epidermoid cysts (ECs) of the central nervous system (CNS) constitute benign circumscribed lesions that aremore common in lateral than in midline sites. Epidermoid cysts of the CNS arise more frequently in the cerebellopontine angle, around the pons, near the sella, within the temporal lobe, in the diploe, and in the spinal canal. Most common tumoral lesion of sellar region is pituitary adenoma, and sellar cystic epithelial masses may be difficult to differentiate based only on clinical and imaging findings. Epidermoid cysts are covered by keratinized squamous epithelium and are usually filled with keratin lamellae. The process is, for the most part, maldevelopmental in origin, presumably arising from trapped surface ectodermal elements in association with the developing CNS during the closure of the neural groove or formation of the secondary cerebral vesicles. In the present study, the authors describe a case of sellar epidermoid cyst producing endocrine alterations and visual disturbance in a 35 years woman, and review the physiopathological and diagnostic criteria of this lesion.


Subject(s)
Humans , Female , Adult , Sella Turcica/abnormalities , Epidermal Cyst/surgery , Epidermal Cyst/physiopathology , Epidermal Cyst/diagnostic imaging , Central Nervous System Cysts
6.
Journal of Korean Neurosurgical Society ; : 723-726, 2019.
Article in English | WPRIM | ID: wpr-788814

ABSTRACT

We report two rare cases of spontaneously regressed Rathke's cleft cyst (RCC). A 52-year-old woman presented with headache. A pituitary hormone study was normal. Brain magnetic resonance imaging (MRI) showed a 0.45-cm³ cystic sellar lesion. The cyst was hyperintense on T1-weighed imaging and hypointense on T2-weighted imaging without rim enhancement, comparable to a RCC. Six months later, brain MRI showed no change in the cyst size. Without any medical treatments, brain MRI 1 year later revealed a spontaneous decrease in cyst volume to 0.05 cm³. A 34-year-old woman presented with headache and galactorrhea lasting 1 week. At the time of the visit, the patient's headache had disappeared. Her initial serum prolactin level was 81.1 ng/mL, and after 1 week without the cold medicine, the serum prolactin level normalized to 11.28 ng/mL. Brain MRI showed a RCC measuring 0.71 cm³. Without further treatments, brain computed tomography 6 months later showed a spontaneous decrease in cyst volume to 0.07 cm³. Another 6 months later, brain MRI revealed that the cyst had remained the same size. Neither patient experienced neurological symptoms, such as headache or visual disturbance, during the period of cyst reduction. The RCCs in both patients underwent spontaneous regression without any medical treatment during a period of 6 months to 1 year. Although spontaneous regression of a RCC is rare, it is still possible and a sufficient follow-up period should be considered.


Subject(s)
Adult , Female , Humans , Middle Aged , Pregnancy , Brain , Central Nervous System Cysts , Follow-Up Studies , Galactorrhea , Headache , Magnetic Resonance Imaging , Neoplasm Regression, Spontaneous , Prolactin
7.
Journal of Korean Neurosurgical Society ; : 723-726, 2019.
Article in English | WPRIM | ID: wpr-765388

ABSTRACT

We report two rare cases of spontaneously regressed Rathke's cleft cyst (RCC). A 52-year-old woman presented with headache. A pituitary hormone study was normal. Brain magnetic resonance imaging (MRI) showed a 0.45-cm³ cystic sellar lesion. The cyst was hyperintense on T1-weighed imaging and hypointense on T2-weighted imaging without rim enhancement, comparable to a RCC. Six months later, brain MRI showed no change in the cyst size. Without any medical treatments, brain MRI 1 year later revealed a spontaneous decrease in cyst volume to 0.05 cm³. A 34-year-old woman presented with headache and galactorrhea lasting 1 week. At the time of the visit, the patient's headache had disappeared. Her initial serum prolactin level was 81.1 ng/mL, and after 1 week without the cold medicine, the serum prolactin level normalized to 11.28 ng/mL. Brain MRI showed a RCC measuring 0.71 cm³. Without further treatments, brain computed tomography 6 months later showed a spontaneous decrease in cyst volume to 0.07 cm³. Another 6 months later, brain MRI revealed that the cyst had remained the same size. Neither patient experienced neurological symptoms, such as headache or visual disturbance, during the period of cyst reduction. The RCCs in both patients underwent spontaneous regression without any medical treatment during a period of 6 months to 1 year. Although spontaneous regression of a RCC is rare, it is still possible and a sufficient follow-up period should be considered.


Subject(s)
Adult , Female , Humans , Middle Aged , Pregnancy , Brain , Central Nervous System Cysts , Follow-Up Studies , Galactorrhea , Headache , Magnetic Resonance Imaging , Neoplasm Regression, Spontaneous , Prolactin
8.
Coluna/Columna ; 17(1): 63-65, Jan.-Mar. 2018. graf
Article in English | LILACS | ID: biblio-890932

ABSTRACT

ABSTRACT Introduction: Among the primary lesions occupying the spinal space, only 1% corresponds to the epidural arachnoid cyst (EAC). This condition is usually asymptomatic, and identified accidentally in imaging tests. In symptomatic cases, total surgical resection is recommended. Objective: To describe a case of EAC refractory to clinical treatment. Methods: A 45-year-old woman had lumbar pain for six years and increased pain in the last months, with irradiation to the left lower limb (corresponding to L1). No other alterations found in the physical examination. Magnetic resonance imaging (MRI) of the spine revealed an intravertebral cystic lesion at T12-L1 level, in the left posterolateral position, causing enlargement of the foramen, and suggesting an epidural arachnoid cyst. Results: Due to failure of the initial clinical treatment, the patient underwent left T12-L1 hemilaminectomy, resection of the cyst and correction of dural failure. The patient progressed with effective pain control and MRI confirmed absence of residual lesion. Conclusion: EAC is more common in men (4:1) and may be congenital or acquired. The most common topography is thoracic (65%). Its clinical presentation is low back pain, lower limb pain and paresthesia. MRI is the method of choice for diagnosis and surgical intervention is restricted to cases that are symptomatic or refractory to clinical treatment, and the prognosis tends to be excellent. We conclude that, in addition to being a rare and commonly asymptomatic condition, an adequate therapeutic approach is essential for complete cure, avoiding intense pain and manifestations that bring about a drastic reduction of functional capacity. Level of evidence: IV. Type of study: Case series.


RESUMO Introdução: dentre as lesões primárias que ocupam o espaço espinhal, apenas 1% corresponde ao cisto epidural aracnoideo (CEA). Esta patologia costuma ser assintomática, identificada acidentalmente em exames de imagem. Já em casos sintomáticos, a ressecção cirúrgica total é recomendada. Objetivos: descrever um caso de CEA refratário a tratamento clínico. Metódos: mulher, 45 anos, lombalgia há seis anos, com piora nos últimos meses e irradiação para membro inferior esquerdo (correspondente a L1). Sem demais alterações ao exame físico. A Ressonância magnética (RM) de coluna vertebral evidenciou lesão cística intravertebral a nível de T12 - L1, em situação póstero-lateral esquerda, provocando alargamento do forame, sugestivo de cisto epiduralaracnóideo. Resultados: Devido a falha do tratamento clínico inicial, a paciente foi submetida a hemilaminectomia de T12 - L1 à esquerda, ressecção do cisto e correção da falha dural. Evoluiu com controle álgico efetivo e RM de controle confirmou ausência de lesão residual. Conclusão: O CEA é mais comum em homens (4:1) e pode ser congênito ou adquirido. A topografia mais comum é a nível torácico (65%). Apresenta-se clinicamente com lombalgia, dor em membros inferiores e parestesias. A RM é o método diagnóstico de escolha e a intervenção cirúrgica é restrita aos casos sintomáticos ou refratários ao tratamento clínico e o prognóstico tende a ser excelente. Concluímos que, além de ser uma patologia rara e comumente assintomática, é essencial a adequada abordagem terapêutica para que ocorra cura completa, evitando quadros álgicos intensos e manifestações que cursem com drástica redução da capacidade funcional. Nível de evidência: IV. Tipo de Estudo: Série de casos.


RESUMEN Introducción: Entre las lesiones primarias que ocupan el espacio espinal, solo el 1% corresponde al quiste aracnoideo epidural (QAE). Esta patología generalmente es asintomática e identificada accidentalmente en pruebas de imagen. En casos sintomáticos, se recomienda la resección quirúrgica total. Objetivo: Describir un caso de QAE refractario al tratamiento clínico. Métodos: Mujer de 45 años tuvo dolor lumbar durante seis años y un aumento del dolor en los últimos meses, con irradiación a la extremidad inferior izquierda (que corresponde a L1). No se encontraron otras alteraciones en el examen físico. La resonancia magnética (RM) de la columna vertebral reveló una lesión quística intravertebral en el nivel T12-L1, en la posición posterolateral izquierda, causando agrandamiento del foramen y sugiriendo un quiste aracnoideo epidural. Resultados: Debido a la falla del tratamiento clínico inicial, la paciente fue sometida a hemilaminectomía T12-L1 izquierda, resección del quiste y corrección de la falla dural. Ella progresó con un control eficaz del dolor y la RM confirmó la ausencia de lesión residual. Conclusión: EL QAE es más común en hombres (4:1) y puede ser congénito o adquirido. La topografía más común es torácica (65%). Su presentación clínica es lumbalgia, dolor en las extremidades inferiores y parestesia. La RM es el método de elección para el diagnóstico y la intervención quirúrgica se limita a los casos que son sintomáticos o refractarios al tratamiento clínico, y el pronóstico tiende a ser excelente. Concluimos que, además de ser una condición rara y comúnmente asintomática, un abordaje terapéutico adecuado es esencial para una curación completa, evitando el dolor intenso y las manifestaciones que provocan una reducción drástica de la capacidad funcional. Nivel de evidencia: IV. Serie de casos.


Subject(s)
Humans , Female , Middle Aged , Arachnoid Cysts , Spinal Cord Injuries , Low Back Pain , Central Nervous System Cysts
9.
Brain Tumor Research and Treatment ; : 82-85, 2018.
Article in English | WPRIM | ID: wpr-717591

ABSTRACT

Extremely massive sellar xanthogranuloma (XG) are rare, and the surgical outcome and prognosis are not well known. XG remain unknown whether they are derived from Rathke's cleft cysts (RCCs) or craniopharyngiomas (CPs) following extensive inflammation and metaplasia, to the point that no epithelium is readily identifiable. These lesions usually tend to occur in younger patients (mean 28.3 years), have a smaller diameter, and remain primarily intrasellar region with infrequent calcification. This 36-year-old man presented our hospital with visual deterioration. At the time of visit, there were no neurological problems other than visual field defect and hormonal disorder. He visited our hospital in 2007 due to headache and decreased vision, and underwent transphenoid surgery for pituitary RCC. Since then, he has received treatment at our hospital for postoperative hormonal disorders. Through preoperative imaging study, the author suspected CP and underwent surgery. During the operation, the adhesion of the tumor to the surrounding major neurovascular structures was severe in the naked eyes, but the tumor could be removed more easily than expected. The postoperative histological findings were confirmed as XG. The postoperative course was uneventful. Compared to the previous literature, this case is a case where the size of XG is very large in a sellar region and it can be proved that it originated from the RCC. And regular follow-up is necessary to confirm the prognosis after surgery.


Subject(s)
Adult , Humans , Central Nervous System Cysts , Craniopharyngioma , Epithelium , Follow-Up Studies , Headache , Inflammation , Metaplasia , Prognosis , Visual Fields
10.
Journal of Genetic Medicine ; : 67-70, 2017.
Article in English | WPRIM | ID: wpr-179818

ABSTRACT

Down syndrome (DS, trisomy 21) is associated with neuroanatomical abnormalities, including choroid plexus cysts and various types of brain tumors. Trisomy 21 is associated with oncogenic factor, especially in brain tumor. The brain of DS patients had a smaller volume of gray and white matter and an unbalanced cerebellum volume, indicating a smaller volume overall than normal. We report a case of a DS male patient who had an incidentally discovered neuroglial cyst in left cerebellar vermis. He visited our hospital with gait disturbance and fatigue. But, the neurologic exam was normal. To the best of our knowledge, this is the first reported case of a neuroglial cyst in a trisomy 21 patient. As the developmental mechanisms of a cyst and the choroid plexus are related, more research is needed.


Subject(s)
Humans , Male , Brain , Brain Neoplasms , Central Nervous System Cysts , Cerebellar Vermis , Cerebellum , Choroid Plexus , Down Syndrome , Fatigue , Gait , Neuroglia , Trisomy , White Matter
11.
Annals of Pediatric Endocrinology & Metabolism ; : 164-169, 2017.
Article in English | WPRIM | ID: wpr-99771

ABSTRACT

PURPOSE: Rathke’s cleft cyst (RCC) is an asymptomatic benign lesion. With increased interest in pediatric endocrinology, the prevalence of RCCs in children is also increasing. However, the clinical relevance and proper management of RCC is not well defined in children. Therefore, we investigated the clinical manifestations and radiologic features of RCC in children and adolescents, as well as the natural progression of RCC. METHODS: We retrospectively reviewed the medical records of 91 children and adolescents with RCC diagnosed with magnetic resonance imaging (MRI) in Severance Children’s Hospital from January 2006 to December 2015. The clinical, hormonal, and imaging findings were analyzed in patient groups classified according to age. The size of each cyst was assessed in sixty patients who underwent follow-up MRI during the 2 years. RESULTS: Female patients were predominant (64 vs. 27). The common clinical features at presentation were endocrine dysfunction (59.3%), headache (23.0%), and dizziness (4.4%). Symptoms related to endocrine disorders were more frequent in younger patients. In 7 patients managed surgically, the cysts were significantly larger and more frequently located in the suprasellar region. Of 60 nonsurgical patients with a follow-up MRI performed within 2 years after the diagnosis, the RCC size increased in about 26.7% (n=16). CONCLUSION: Although 94.4% of the patients with RCC had clinical symptoms, surgery was performed in only about 7.5% of patients. RCC is associated with pituitary insufficiency, thus, baseline and follow-up endocrine function tests are required. Additionally, regular MRI follow-up is required in long-term period to monitor change in size.


Subject(s)
Adolescent , Child , Female , Humans , Central Nervous System Cysts , Diagnosis , Dizziness , Endocrine System Diseases , Endocrinology , Follow-Up Studies , Headache , Hypopituitarism , Magnetic Resonance Imaging , Medical Records , Natural History , Prevalence , Retrospective Studies
12.
Brain Tumor Research and Treatment ; : 58-62, 2016.
Article in English | WPRIM | ID: wpr-205889

ABSTRACT

BACKGROUND: There have been various reports in the literature regarding the conservative management of pituitary apoplexy, pituitary incidentalomas and Rathke cleft cysts (RCCs). However, to the best of our knowledge, spontaneous involution of cystic sellar mass has rarely been reported. We report 14 cases of cystic sellar masses with spontaneous involution. METHODS: A total of 14 patients with spontaneous regression of cystic sellar masses in our hospital were included. The median age was 35 years (range, 5–67), and 8 patients were male. Clinical symptoms, hormone study and MRI were evaluated for all patients. The initial MRI showed all 14 patients with RCCs. Eight patients were presented with sudden onset of headache, and 1 patient with dizziness. Another patient, a 5-year-old child, was presented with delayed growth. Three patients had no symptoms via regular medical work up. All 14 patients had no visual symptoms. The follow-up period ranged from 5.7 to 42.8 months, with the mean of 17.3 months. RESULTS: The mean initial tumor size was 1.29 cm³ (range, 0.05 to 3.23). After involution, the tumor size decreased to 0.23 cm³ (range, 0 to 0.68) without any treatments. Repeated MRI showed a spontaneous decrease in tumor volume by 78% (range, 34 to 99). The initial MRI showed that the tumor was in contact with the optic chiasm in 7 patients, while compressing on the optic chiasm in 3 patients. Five patients were initially treated with hormone replacement therapy due to hormone abnormality. After the follow-up period, only 2 patients needed a long-term hormone replacement therapy. CONCLUSION: The spontaneous involution of RCCs is not well quantified before. Their incidence has not been well demonstrated, but this phenomenon might be underreported. Conservative management can be a treatment option in some RCCs without visual symptoms, even in those that are large in size and in contact with the optic nerve via imaging study.


Subject(s)
Child , Child, Preschool , Humans , Male , Central Nervous System Cysts , Dizziness , Follow-Up Studies , Headache , Hormone Replacement Therapy , Incidence , Magnetic Resonance Imaging , Optic Chiasm , Optic Nerve , Pituitary Apoplexy , Tumor Burden
13.
Ultrasonography ; : 353-358, 2016.
Article in English | WPRIM | ID: wpr-731221

ABSTRACT

We report the case of a giant hypothalamic hamartoma with a large intracranial cyst in a neonate. On ultrasonography, the lesion presented as a lobulated, mass-like lesion with similar echogenicity to the adjacent brain parenchyma, located anterior to the underdeveloped and compressed left temporal lobe, and presenting as an intracranial cyst in the left cerebral convexity without definite internal echogenicity or septa. The presence of a hypothalamic hamartoma and intracranial neurenteric cyst were confirmed by surgical biopsy. The association of a giant hypothalamic hamartoma and a neurenteric cyst is rare. Due to the rarity of this association, the large size of the intracranial cyst, and the resulting distortion in the regional anatomy, the diagnosis of the solid mass was not made correctly on prenatal high-resolution ultrasonography.


Subject(s)
Humans , Infant, Newborn , Anatomy, Regional , Biopsy , Brain , Central Nervous System Cysts , Diagnosis , Hamartoma , Magnetic Resonance Imaging , Neural Tube Defects , Temporal Lobe , Ultrasonography
15.
Biomédica (Bogotá) ; 34(2): 166-170, abr.-jun. 2014. ilus, tab
Article in Spanish | LILACS | ID: lil-712400

ABSTRACT

La microangiopatía cerebral retiniana con calcificaciones y quistes es una enfermedad poco frecuente, caracterizada por alteraciones cerebrales, retinianas y óseas, así como por predisposición al sangrado gastrointestinal. Existen pocos reportes de casos de esta condición, especialmente en adultos, en quienes la incidencia es baja. Los hallazgos por medio de neuroimágenes son característicos, con calcificaciones bilaterales y múltiples formaciones quísticas. El propósito de este artículo fue hacer una revisión bibliográfica e ilustrar dos casos cuyo diagnóstico fue posible con la ayuda de neuroimágenes.


Cerebroretinal microangiopathy with calcifications and cysts is a rare condition characterized by brain, retinal and bone anomalies, as well as a predisposition to gastrointestinal bleeding. There are few reported cases of this condition in adults, among whom the incidence is low. Neuroimaging findings are characteristic, with bilateral calcifications, leukoencephalopathy and intracranial cysts. The purpose of this article was to do a literature survey and illustrate two cases diagnosed with the aid of neuroimaging.


Subject(s)
Adolescent , Adult , Female , Humans , Ataxia/pathology , Brain Neoplasms/pathology , Brain/pathology , Calcinosis/pathology , Central Nervous System Cysts/pathology , Cerebral Small Vessel Diseases/pathology , Leukoencephalopathies/pathology , Magnetic Resonance Imaging , Muscle Spasticity/pathology , Neuroimaging/methods , Retinal Diseases/pathology , Seizures/pathology , Ataxia/diagnosis , Brain Neoplasms/diagnosis , Calcinosis/diagnosis , Central Nervous System Cysts/diagnosis , Cerebral Small Vessel Diseases/diagnosis , Diagnosis, Differential , Hair Color , Hypopigmentation/etiology , Intellectual Disability/etiology , Leukoencephalopathies/diagnosis , Muscle Spasticity/diagnosis , Quadriplegia/etiology , Retinal Diseases/diagnosis , Seizures/diagnosis , Trochlear Nerve Diseases/etiology
16.
Keimyung Medical Journal ; : 159-163, 2014.
Article in Korean | WPRIM | ID: wpr-24559

ABSTRACT

Rathke cleft cysts (RCCs) are benign epithelial lesions of the sellar and suprasellar region. Most RCCs remain clinically silent throughout an individual's life. Symptomatic patients with RCCs manifest headaches, endocrinopathies, and visual disturbances secondary to parasellar extension. Rarely, RCCs can present in a manner similar to pituitary tumor apoplexy. One such case is reported herein. This 53-year-old man presented with acute headache, vomiting, and partial hypopituitarism. Surgical drainage of the cyst contents via a transnasal route confirmed the diagnosis of RCC and pituitary infarction.


Subject(s)
Humans , Middle Aged , Central Nervous System Cysts , Diagnosis , Drainage , Headache , Hypopituitarism , Infarction , Pituitary Apoplexy , Pituitary Neoplasms , Stroke , Vomiting
17.
Annals of Pediatric Endocrinology & Metabolism ; : 20-26, 2014.
Article in English | WPRIM | ID: wpr-158574

ABSTRACT

PURPOSE: In the pediatric population, Rathke's cleft cysts (RCCs) are known to be an infrequent cause of headaches, visual disturbances, and pituitary dysfunction. We investigated the clinical characteristics of children in whom RCCs were incidentally discovered and evaluated whether RCCs influence the treatment response of patients with proven endocrinopathy. METHODS: A retrospective analysis was conducted in 34 patients with RCCs who were diagnosed between 2006 and 2013 at Hallym University Medical Center. Their clinical, hormonal, and imaging findings were reviewed. We evaluated the clinical outcomes of the patients with concomitant RCCs and endocrinopathy compared to matched controls. RESULTS: Twenty-six of 34 patients with radiologically proven RCCs had endocrine disorders. They were 9 boys and 17 girls, with ages ranging from 4.8 to 17.4 years at the time of the diagnosis. Of these, 7 (27%) had idiopathic short stature, 7 (27%) had growth hormone deficiency (GHD), and 12 (46%) had central precocious puberty (CPP). Nineteen of 26 patients (73.1%) showed low signal intensities on T1-weighted images (T1WI) and high signal intensities on T2-weighted images. The incidence of hypointensity on T1WI was higher in the patients with RCCs accompanied by endocrinopathy than in those without endocrinopathy (P=0.033). The treatment outcomes of the patients with CPP and GHD with and without RCCs were similar. CONCLUSION: CPP and GHD patients with a small RCC (less than 20 mm) expressing cystic magnetic resonance intensity can be managed with medical treatment, although the RCCs need to be closely monitored in radiological studies to observe their growth.


Subject(s)
Adolescent , Child , Female , Humans , Academic Medical Centers , Central Nervous System Cysts , Diagnosis , Dwarfism, Pituitary , Growth Hormone , Headache , Incidence , Puberty, Precocious , Retrospective Studies
18.
Journal of Korean Medical Science ; : 405-410, 2014.
Article in English | WPRIM | ID: wpr-112005

ABSTRACT

Pituitary adenoma (PA) is a common benign neuroendocrine tumor; however, the incidence and proportion of hormone-producing PAs in Korean patients remain unknown. Authors analyzed 506 surgically resected and pathologically proven pituitary lesions of the Seoul National University Hospital from 2006 to 2011. The lesions were categorized as: PAs (n = 422, 83.4%), Rathke's cleft cysts (RCCs) (n = 54, 10.6%), inflammatory lesions (n = 8, 1.6%), meningiomas (n = 4), craniopharyngiomas (n = 4), granular cell tumors (n = 1), metastatic renal cell carcinomas (n = 2), germinomas (n = 1), ependymomas (n = 1), and unsatisfactory specimens (n = 9, 1.8%). PAs were slightly more prevalent in women (M: F = 1:1.17) with a mean age of 48.8 yr (9-80 yr). Immunohistochemical analysis revealed that prolactin-producing PAs (16.6%) and growth hormone-producing adenomas (9.2%) were the most common functional PAs. Plurihormonal PAs and nonfunctioning (null cell) adenomas were found in 14.9% and 42.4% of patients with PAs, respectively. The recurrence rate of PAs was 11.1%, but nearly 0% for the remaining benign lesions such as RCCs. 25.4% of patients with PAs were treated by gamma-knife after surgery due to residual tumors or regrowth of residual tumor. In conclusion, the pituitary lesions and the proportions of hormone-producing PAs in Korean patients are similar to those of previous reports except nonfunctioning (null cell) PAs, which are unusually frequent.


Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Humans , Male , Middle Aged , Young Adult , Adenoma/pathology , Age Factors , Central Nervous System Cysts/pathology , Growth Hormone/metabolism , Immunohistochemistry , Pituitary Neoplasms/pathology , Prolactin/metabolism , Recurrence , Sex Factors
19.
Chinese Journal of Otorhinolaryngology Head and Neck Surgery ; (12): 209-212, 2013.
Article in Chinese | WPRIM | ID: wpr-315775

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the effectiveness of transsphenoidal endoscopic endonasal approach for the resection of Rathke cleft cysts (RCC).</p><p><b>METHODS</b>Thirty-five pathologically diagnosed RCCs were resected throuth transsphenoidal endoscopic endonasal approach at the HuanHu hospital between January 2000 and December 2011. The clinical data were retrospectively analysed. There were 12 male and 23 female. The average age was 45.7 years and the average disease course was 4.6 years. The typical clinical manifestations included headache (26 cases), visual interference (11 cases) and pituitary dysfunction (8 cases). The postoperative symptoms and follow-up results were recorded.</p><p><b>RESULTS</b>All of the patients underwent complete removal of the cyst contents, complete removal of the cyst wall in 20, fenestration and aspiration of the RCC with partial excision of the cyst wall in 15. The mean cyst volume was (1145.3 ± 12.1) mm(3) [range (121 - 4586) mm(3)]. One patient had cerebrospinal fluid leak, which was repaired using artificial dura mate of brain and gelatin sponge intraoperatively. No postoperative complications were found. Follow-up time ranged from half a year to 11 years. Headache resolved in 100% (26/26), visual interference resolved in 90.9% (10/11), pituitary dysfunction resolved in 62.5% (5/8). No patient recurred.</p><p><b>CONCLUSION</b>It is concluded that the transsphenoidal endoscopic endonasal approach is microinvasive, safe and effective in the treatment of RCC.</p>


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Central Nervous System Cysts , General Surgery , Endoscopy , Methods , Retrospective Studies
20.
Brain Tumor Research and Treatment ; : 50-53, 2013.
Article in English | WPRIM | ID: wpr-8237

ABSTRACT

We report here the case of a suprasellar cystic germ cell tumor (GCT) initially diagnosed as an arachnoid cyst. A 10-year-old boy experienced headache, dizziness, and diplopia, and was shown to have an approximately 2 cm suprasellar cyst. Two months after endoscopic third ventriculostomy was performed, a 5-6 cm cystic mass with an internal enhancing component was observed in the suprasellar cistern. Serum human chorionic gonadotropin levels were slightly increased in the serum and cerebrospinal fluid (55 and 162 IU/L, respectively) but were strikingly elevated in the cystic fluid (14,040 IU/L). The patient showed complete remission, with only a very small cystic lesion remaining after surgery, chemotherapy, and radiation treatment for a suprasellar mixed GCT. However, follow-up after treatment was complicated by moyamoya syndrome and cerebral infarction. GCT can be considered as a rare differential diagnosis in the case of a suprasellar cystic mass. Evaluation of tumor markers and close follow-up will be necessary.


Subject(s)
Child , Humans , Male , Arachnoid , Central Nervous System Cysts , Cerebral Infarction , Cerebrospinal Fluid , Chorionic Gonadotropin , Diagnosis, Differential , Diplopia , Dizziness , Drug Therapy , Follow-Up Studies , Germ Cells , Headache , Moyamoya Disease , Neoplasms, Germ Cell and Embryonal , Biomarkers, Tumor , Ventriculostomy
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