Transclival cerebrospinal fluid fistula in a patient with Marfan's syndrome.

Marfan's syndrome is a disease associated with reduced structural integrity of connective tissues. We report a 36-year-old patient with Marfan's syndrome who presented with rhinorrhoea, occipital headache and vomiting. Physical examination revealed typical Marfan's syndrome features including dolicocephalous, mandibular micrognathia, tall stature, disproportionately long limbs and digits, and hypermobility of the joints. A high-resolution CT scan demonstrated pneumoencephalous, cerebrospinal fluid (CSF) filling the sphenoidal sinus, and a small bone defect of the clivus. Surgery performed through a transsphenoidal approach revealed the sphenoid sinus to be filled with CSF and a small fenestration in the clivus. The arachnoid diverticulum and the fenestration were repaired and covered with a graft of abdominal fat. In this patient, a deficiency in bone development associated with Marfan's syndrome gave rise to a clival fenestration and a transclival CSF fistula. Although abnormalities of the spinal meningeal membranes have been reported in Marfan's syndrome, to our knowledge, this is the first report of a fistula located in the cranial base in this condition.

Tratamiento quirúrgico: técnicas, indicaciones, resultados y complicaciones / Surgical treatment: techniques, indications, results and complications

En el momento actual, el tratamiento electivo de la acromegalia consiste en la resección quirúrgica del adenoma productor de hormona del crecimiento con conservación de la función hipofisaria. La mayoría de los casos son intervenidos mediante un abordaje transesfenoidal, que puede ser sublabial o intranasal. El endoscopio puede mejorar la visión de algunos aspectos de la resección quirúrgica realizada con un microscopio y es útil como adyuvante de la microcirugía. La resección quirúrgica de un adenoma con técnica endoscópica exclusiva puede presentar serias limitaciones en los campos quirúrgicos con hemorragia, lo que añade riesgo y en ningún caso mejora los resultados endocrinológicos que ofrece la microcirugía. Estos resultados guardan relación con el tamaño del tumor y los criterios de curación utilizados, así como con el tiempo de evolución postoperatoria. En general se puede esperar una remisión de la enfermedad en más del 90% de los casos intervenidos por tumores < 1 cm de diámetro, pero también se puede obtener la remisión de la enfermedad en al menos un 15% de los casos con adenomas invasivos. El porcentaje de complicaciones es bajo, con insuficiencia de la función hipofisara secundaria al acto quirúrgico en alrededor del 1% de los casos (AU)

Transsphenoidal microsurgery remains the most valid and widely used method of treating acromegaly. This procedure that has been proved relatively safe, achieves selective adenomectomy in a great number of cases and a successful outcome in about 90% of patients with microadenomas. Pituitary insuficiency surgically realated in less than 1% of the cases. 90% of all pituitary adenomas can be treated via the transphenoidal approach using different entry sites: sublabial, pernasal, endonasal. The use of endoscopes is very helpful for better visualization within the sphenoid sinus, especially within the sella, but does not seem to improve the final endocrinological results. Acurate endocrinological diagnosis and early surgical treatment lead to prevent tumor growth and possible focal or general invasion. An other surgical point, is the the prevention of the possibility of pituitary apoplexy rare but life threatening (AU)

Varying additive effects of bromocriptine with two somatostatin analogs in cultures of GH-secreting adenomas.

In this study, we have investigated the effect of combined treatment using two somatostatin analogs, lanreotide or octreotide, with bromocriptine on GH release in cultures of GH-secreting pituitary tumors. Sixteen acromegalic patients were included in the study. All patients had been treated with lanreotide prior to the surgery. Five patients (31.2 %) reached GH levels below 2.0 microg/l and normal IGF-I levels according to age and sex after lanreotide treatment. A positive correlation was observed between the lanreotide-induced inhibition of GH release in vitro and serum GH decrease after lanreotide treatment (r = 0.52; p = 0.03). Combined treatment significantly inhibited GH release in vitro in 8 of the 16 tumors (50 %). However, only 5 (31.2 %) of the respective patients had been resistant to presurgical treatment with lanreotide. Three of these 5 patients (18.7 %) responded to a BC concentration similar to that achieved with therapeutic doses, and in 2 patients only when a pharmacological dose of BC was used in the combined treatment. The additive effect was observed with the combination of lanreotide and BC in 6 tumors and with octreotide and BC in 3. Only one tumor showed simultaneous response to both types of combination. These results suggest that the additive effect under the combined treatment might be found between 18 and 30 % of patients who are resistant to these drugs, and that different combinations of somatostatin analogs and dopamine agonists should be tested in resistant patients.

The complete normalization of the adrenocortical function as the criterion of cure after transsphenoidal surgery for Cushing's disease.

Transsphenoidal microsurgery is the standard treatment for patients with Cushing's disease. However, there is general lack of agreement regarding the definition of cure. We studied 58 patients with corrected hypercortisolism after transsphenoidal surgery for Cushing's disease. Plasma and urinary cortisol levels were measured after surgery. After the postsurgical hypocortisolism stage (or periodically in patients without hypocortisolism), urinary free cortisol, plasma cortisol at 0800 h and 2300 h, morning cortisol after 1 mg dexamethasone, and cortisol response to insulin-induced hypoglycemia were performed. Patients were classified in 3 groups: group I, patients with transient hypocortisolism and normal hypothalamus-pituitary-adrenal axis afterwards; group II, patients with transient hypocortisolism and abnormalities in the circadian rhythm or the stress response afterwards; and group III, patients without postoperative hypocortisolism. Thirty-three patients were included in group I, 8 in group II, and 17 in group III. Groups I and II were similar in postsurgical plasma cortisol (46.9 +/- 30.3 vs. 60.7 +/- 38.6 nM) and mean follow-up (69.8 vs. 68.8 months) but were significantly different in their recurrence rate (3.4% vs. 50%, P < 0.001). Patients in group III had normal postsurgical plasma and urinary cortisol but persistent abnormalities in circadian rhythm and stress response. After a mean follow-up of 39.1 months, their recurrence rate was similar to that of group II (64.7% vs. 50%). The complete normalization of the adrenocortical function, which is always preceded by postsurgical hypocortisolism, is associated with a very low recurrence risk and should be considered, in our opinion, the main criterion of surgical cure in Cushing's disease.

Spontaneous cerebrospinal fluid fistulae associated with empty sellae: surgical treatment and long-term results.

OBJECTIVE: To evaluate the accuracy of different diagnostic tools for cerebrospinal fluid fistulae (CFFs) associated with empty sellae and to assess the long-term results after surgical treatment. METHODS: Records were retrospectively reviewed for 38 of 44 patients who were treated for CFFs associated with empty sellae between 1972 and 1996. Glucose and radioisotope analyses of nasal fluids were used to establish the CFF diagnosis. Computed tomography, magnetic resonance imaging, and cisternography were used to localize the sites of the CFFs. Treatment was performed using three different approaches, i.e., subfrontal approach, transsphenoidal approach, and lumboperitoneal shunt placement. RESULTS: Glucose and radioisotope analyses of nasal fluids confirmed CFFs in 97 and 100% of the cases, respectively. For localization of the sites, a leak in the sella was suggested in 32% of the cases using gammagraphy, in 50% using computed tomography or magnetic resonance imaging, and in 75% using computed tomographic contrast cisternography. After surgery, rhinorrhea ceased for 34 patients (89%), but 14 (41%) patients experienced recurrences of the rhinorrhea 6 months to 20 years after surgery (mean, 4.2 yr). Cure rates for the subfrontal approach, transsphenoidal approach, and shunt placement were 94, 85, and 86%, and the recurrence rates were 43, 33, and 50%, with mean times of 7.8, 4.2, and 0.8 years, respectively. CONCLUSION: In this series, confirmation of CFFs was easy, although localization of the sites remained difficult. Recurrences were more numerous and occurred sooner after treatment with shunts; packing of the sella through a craniotomy achieved better and more stable results, although differences were not statistically significant.

Hypothalamic-pituitary dysfunction in patients with craniopharyngioma.

OBJECTIVE: Previous studies of preoperative pituitary function in patients with craniopharyngioma have been limited in scope and have focused on children. We have evaluated the impact of craniopharyngiomas and their surgical treatment on pituitary function in a large group of mostly adult patients. DESIGN: We performed a retrospective study of patients treated at our centre between 1980 and 1992. PATIENTS: Twenty-two men and 13 women, most of them adults, treated surgically for craniopharyngioma during the above period. MEASUREMENTS: Serum glucose, GH, LH, FSH, TSH and cortisol were measured both before and after a combined insulin induced hypoglycaemia, GnRH and TRH test. Basal concentrations of thyroid hormones, PRL and gonadal steroids were also measured. Preoperative computed tomographic scan was performed in all patients, and a detailed study of visual function before and after surgery was available for 32 of them. Endocrine function was reevaluated post-operatively. RESULTS: In preoperative studies, 29 patients had some anterior pituitary deficit and 13 had diabetes insipidus. The most common abnormality was gonadotrophin deficiency, followed by GH deficiency. Dynamic studies suggested a hypothalamic origin for these deficits. In post-surgical evaluation, impaired pituitary function was observed in most patients. Panhypopituitarism was present in 28 cases and diabetes insipidus in 24. CONCLUSIONS: Our report illustrates the high incidence of endocrine deficits in patients with craniopharyngioma. Additional hypothalamic-pituitary dysfunction usually occurs following surgical treatment of these tumours.

Recovery of visual and endocrine function following transsphenoidal surgery of large nonfunctioning pituitary adenomas.

A series of 35 patients with non-functioning pituitary adenomas undergoing transsphenoidal surgery is presented. In most cases, the presenting symptoms were related to the mass effect of the tumor. There was no operative mortality. Before surgery, visual field defects were documented in 21 patients (60%). After surgery, excluding 3 patients with preoperative blindness, 28% regained normal vision and 67% showed variable improvement. Preoperatively, 24 patients (69%) had abnormal pituitary function, 24 (69%) had hypogonadism, 7 (20%) adrenal insufficiency, 8 (23%) hypothyroidism and 2 (6%) panhypopituitarism. After pituitary surgery, all but one patient with normal preoperative function retained it. Of the patients with hypopituitarism, 11 (46%) had variable improvement and 13 (54%) had persistent deficits. After surgery, 4 patients (57%) with adrenal insufficiency recovered normal adrenal function, 7 patients (29%) with hypogonadism recovered gonadal function and 1 patient (13%) with hypothyroidism recovered thyroid function. Prior to surgery, the presence of a normal or slightly elevated PRL and a rise in TSH after TRH and in LH after GnRH stimulation were of value in predicting possible recovery of pituitary function after surgery. These observations suggest the presence of viable pituitary tissue in these cases and point out that, in some instances, the mechanism of hypopituitarism may be compression of the portal circulation, rather than destruction of the normal pituitary gland.

Treatment of invasive growth hormone pituitary adenomas with long-acting somatostatin analog SMS 201-995 before transsphenoidal surgery.

The purpose of this study was to determine whether the long-acting somatostatin analog SMS 201-995 (octreotide) shrinks growth hormone (GH)-secreting adenomas and improves the results of subsequent transsphenoidal surgery. Ten previously untreated active acromegalic patients (nine women and one man) with invasive tumors were treated with SMS 201-995 (100 micrograms subcutaneously every 8 hours) for 6 weeks prior to transsphenoidal surgery. The clinical activity, mean GH secretion, insulin-like growth factor (IGF)-I concentration, and tumor volume were measured under basal conditions and on Days 14, 28, and 42 of treatment. The SMS 201-995 improved the symptoms of acromegaly in all patients. Mean levels of both GH and IGF-I (+/- standard deviation) were significantly decreased by Day 14 (from 92.9 +/- 30.5 to 44.9 +/- 20.3 micrograms/liter and from 10.6 +/- 7.4 to 5.9 +/- 2.6 U/ml, respectively), after which there were only slight further decreases. Six (60%) of the 10 patients experienced tumor shrinkage ranging from 9% to 78% (mean 30%). When it occurred, tumor shrinkage was significant by Day 14 (7.9 +/- 6.3 to 6.5 +/- 5.1 cu cm) and no further shrinkage was achieved by longer administration. Transsphenoidal surgery reduced postoperative GH levels to less than 2 micrograms/liter and IGF-I to less than 1.5 U/ml in six patients (60%). This percentage of cure is higher than expected from the literature and the authors' previous experience. However, an investigation of the influence of this drug on several parameters, such as reduction of tumor size or GH and IGF-I concentrations, has failed to prove any relationship. Only pretreatment size of the tumor was of predictive value with respect to the surgical outcome.

Lymphocytic hypophysitis causing hypopituitarism and diabetes insipidus, and associated with autoimmune thyroiditis, in a non-pregnant woman.

A 25 year old non-pregnant woman presented with a one-year history of amenorrhoea and polyuria. Three months before her admission, she had suffered lymphocytic meningitis. Hormonal studies revealed hypopituitarism and central diabetes insipidus, with associated primary autoimmune hypothyroidism. Computed tomographic scan and magnetic resonance imaging showed a pituitary mass with suprasellar extension and thickened stalk. Transsphenoidal surgery was performed and the histological study revealed fibrosis and diffuse lymphocytic infiltration with predominance of CD4 lymphocytes. This further case of lymphocytic hypophysitis was not related to pregnancy and produced diabetes insipidus, two uncommon associations. We discuss the features that can lead to a preoperative suspicion of this rare disorder.

Experimental neurogenic pulmonary edema. Part 1: The role of systemic hypertension.

Neurogenic pulmonary edema (NPE) was produced consistently in normal cats by increasing intracranial pressure with an intraventricular infusion of mock cerebrospinal fluid. The usual elevation of systemic arterial pressure (SAP) that follows severe intracranial hypertension (the "Cushing response") was controlled by blood withdrawal at variable rates to achieve and maintain constant cerebral perfusion pressure (CPP) in three groups of cats of 50, 20, and 0 mm Hg, respectively, for 30 minutes. In this model, NPE occurs in the absence of increased SAP and in the presence of decreasing CPP. These results indicate that systemic arterial hypertension is not an essential stimulus for the development of NPE, and suggest that the lungs are directly affected by the intense sympathetic discharge evoked by severe intracranial hypertension.

Experimental neurogenic pulmonary edema Part 2: The role of cardiopulmonary pressure change.

Pressure changes in the aorta, left atrium, and main pulmonary artery were measured before, during, and after inducing increased intracranial pressure in cats. By selectively controlling each of the three pressures, it was concluded that pulmonary arterial hypertension is the single most important precursor of experimental neurogenic pulmonary edema. An earlier observation that neurogenic pulmonary edema may develop in the absence of systemic arterial hypertension was confirmed.

Syringomyelia: long-term results after posterior fossa decompression.

A retrospective study of the surgical results in 31 adult patients with syringomyelia associated with Arnold-Chiari malformation was carried out. The standard surgical procedure consisted of a posterior fossa craniectomy. The vallecula was opened in 28 patients, with plugging of the central canal in 19. In three patients, severe arachnoidal scarring was present, and dissection was not attempted. The postoperative follow-up period ranged from 5 to 10 years. More than half of the patients considered themselves improved by surgery; however, only five cases showed objective improvement. Despite the operation, six patients experienced progressive neurological deterioration.

Effect of cerebellar lesions on monoamine levels in various brain areas of the cat.

Modifications in thee content of monoamines after different lesions of the cerebellar cortex were investigated in eight prosencephalic structures of cat's brain. Apart from other minor changes, lesions of the posterior vermis induced significant changes in the thalamus (decrease of DA and increase of 5-HT). Lesions of the cortex of a cerebellar hemisphere, on the other hand, produced an increase of 5-HT in the caudate nucleus and an increase of DA in the hippocampus in addition to a generalized increase of 5-HT in all the prosencephalic structures studied. These findings are discussed in relation to the anatomical connections of the lesioned areas and their expected role in the sleep-wakefulness cycle.

Subdural haematoma as a complication in the surgical removal of intracranial hydatidosis.

A patient with multiple cerebral hydatidosis was operated on through a left frontal craniotomy. The occurrence of a subdural haematoma over the right cerebral convexity, secondary to cerebral decompression during the operation, proved fatal. The authors wish to draw attention to the possibility of this type of complication in surgical procedures likely to produce cerebral collapse.

Epicranial plexiform neurofibroma.

Epicranial plexiform neurofibroma is a rare presentation of neurofibromatosis. Two such cases are presented in which epicranial tumors constituted isolated manifestations of the disease except for skin pigmentation. Tumor development began at two and three months of age respectively; one case showed a calvarian bony defect associated with the tumor.

Empty sella and rhinorrhea. A report of eight treated cases.

The association of an empty sella with rhinorrhea is an unusual finding. Of 29 cases of empty sella observed by the authors, eight underwent surgery for rhinorrhea. None of the patients showed clinical evidence of increased intracranial pressure or hydrocephalus. Seven were cases of primary empty sella, the eighth was an acromegalic patient, who had received radiotherapy 4 years before. Except for this patient, the others showed no clinical signs of a previous tumor. In the surgical treatment of these patients, the authors used both the transfrontal and transsphenoidal approaches. In spite of generally accepted good results following surgical closure of this type of fistula, four patients needed more than one operation. The fistula closed in only three of them; in the last patient rhinorrhea persisted after three operations.

Primary carcinoma of the choroid plexus with metastatic dissemination within the central nervous system.

The authors present a case of primary carcinoma of the choroid plexus in a 26-years-old female, localized in the left lateral ventricle. Four years after total removal plus local radiotherapy, the patient presented with an intraparenchymatous metastasis in the right cerebellar hemisphere and intraspinal metastatic seeding. With this kind of malignant growth, a therapeutic approach consisting of radical removal of the tumour plus radiotherapy to the whole neuraxis is proposed.