Endoscopic transsphenoidal treatment of empty sella turcica syndrome using a silastic coil.

An empty sella turcica is due to the presence of an arachnoid diverticulum with its fluid content in the sella turcica, exerting pressure on the pituitary gland. In most cases this condition has an asymptomatic course, and is discovered by accident. Some patients, however, develop empty sella turcica syndrome with headaches, mild dishormonose, dysopsia and, rarely, spontaneous rhinorrhoea. Surgical treatment of empty sella turcica consists of filling the sella, through the transsphenoid route, with tissues collected from the patient or with artificial material. The aim of this report is to present our own experience of endoscopic extradural sella elevation using a silicone spiral, in 4 patients with primary empty sella turcica syndrome. The main indication for surgery was progressing dysopsia. The microinvasive endoscopic transsphenoidal method has been used, based upon the Jho technique with our own modifications. For the elevation of the sella, we used a coiled section of a Pudenz valve intraventricular silicone drain, adjusting its size to the dimensions of the operated sella. Both the implantation of the helix, and the postoperative course were uncomplicated for all surgically treated patients. The follow-up of several months confirmed improvement of the dysopsia in all surgically treated patients. MR examinations confirmed the correct location of the silicone spiral placed in the sella. It seems that the good results achieved are due to a correct indication for surgical treatment. The follow-up period ranges from 12 to 30 months and, so far, the clinical improvement is stable and satisfying both for the patients who underwent treatment and for the neurosurgeons.     

Serum prolactin and radiological skull examinations in infertile men with azoospermia or severe oligozoospermia

Measurements of serum prolactin, FSH, LH, testosterone, and oestradiol and radiological examinations of the skull were carried out in 142 infertile men with sperm counts below 10 millions/ml. The prolactin levels were compared to those observed in 27 fertile men. Six of the infertile men showed high levels of prolactin. Two of these men had earlier been hypophysectomized, and the sella turcica was found to be abnormal on the roentgenograms. In the remaining four men the sella turcica was normal, and serum gonadotrophins, testosterone and oestradiol were within normal range, except for one man who displayed a very high FSH. The testis biopsies from these four men showed severe hypoplasia, signalling that treatment of the infertility with bromocriptine may be of limited value.     

Serum Prolactin and Radiological Skull Examinations in Infertile Men with Azoospermia or Severe Oligozoospermia

Measurements of serum prolactin, FSH, LH, testosterone, and oestradiol and radiological examinations of the skull were carried out in 142 infertile men with sperm counts below 10 millions/ml. The prolactin levels were compared to those observed in 27 fertile men.
Six of the infertile men showed high levels of prolactin. Two of these men had earlier been hypophysectomized, and the sella turcica was found to be abnormal on the roentgenograms. In the remaining four men the sella turcica was normal, and serum gonadotrophins, testosterone and oestradiol were within normal range except for one man who displayed a very high FSH. The testis biopsies from these four men showed severe hypoplasia, signalling that treatment of the infertility with bromocriptine may be of limited value.     

Empty sella syndrome: does it exist in children?

OBJECT: The empty sella syndrome (ESS) is well documented in adults, and although the same phenomenon of herniation of the arachnoid space into the enlarged sella turcica has been noted in children, it is not widely known that children suffer from this syndrome. Therefore, the aims of this paper are to increase neurosurgeons' awareness of the existence of this phenomenon in children and to add to the scant body of literature on the subject. METHODS: The authors treated 12 children, ranging in age between 2 and 8 years, in whom neuroradiological studies demonstrated an enlarged sella turcica filled with cerebrospinal fluid and herniation of suprasellar and arachnoid spaces. The causes of ESS in these children were high intracranial pressure, neglected or improperly treated hydrocephalus, and suprasellar arachnoid cyst. Primary ESS was found as well. Most of the children presented with headache, abnormal body weight (the majority being underweight), and short stature. The results of hormone assays were normal in all children. CONCLUSIONS: If undiagnosed and untreated, ESS in children may lead to serious consequences, including impairment of pituitary and hypothalamic function and damage to the optic chiasm. It is important to raise awareness in the neurosurgical community about the existence of ESS in children so that it can be diagnosed and treated at an early stage. A classification system for the diaphragma sellae is recapitulated.     

Systemic non-Hodgkin lymphoma initially presented with visual disturbance due to intrasellar lymphoma--a case report

A case with systemic non-Hodgkin lymphoma involving the sella turcica and kidney is reported. A 69-year-old man presented with a progressive two month history of visual disturbance and headache. Neurological examination revealed bilateral visual disturbance and right optic atrophy. MRI showed a contrast-enhancing mass in the sella turcica. The tumor extended to the right optic nerve. Without extensive studies for systemic disease, the patient immediately underwent transsphenoidal surgery. The slightly firm, fibrous and vascular-rich tumor was subtotally removed. The histopathological examination revealed a malignant lymphoma, diffuse-large-cell type with B-cell phenotype. The postoperative course was uneventful and the patient's symptoms subsided gradually. The patient received radiation therapy and the tumor disappeared. Postoperative CT examinations of the abdomen and pelvis revealed a large mass at the upper portion of the left kidney. Ga-scan also suggested the mass to be consistent with the abdominal CT. However, the patient suddenly died of acute heart failure with unknown cause just before starting chemotherapy for systemic lymphoma. Patients presenting primary central nervous system lymphoma (PCNSL) may have systemic non-Hodgkin lymphoma. To exclude systemic non-Hodgkin lymphoma, systemic investigation is essential for the initial management of patients presenting PCNSL.     

Primary pituitary abscess

This report details the management of a patient with a primary pituitary abscess. The patient presented with unilateral ophthalmoplegia and obtundation progressing to stupor. The diagnosis was established by utilizing plain x-ray films of the skull, computed tomography scans, and angiography. After a transsphenoidal decompression of the sphenoid sinus and the sella turcica, with accompanying antibiotic therapy, the patient enjoyed a progressive and nearly complete recovery. In view of the patient's clinical presentation and radiographic findings, we were able to make the diagnosis of pituitary abscess pre-operatively.     

Primary intrasellar microgerminoma detected by magnetic resonance imaging: case report

A case of primary intrasellar germinoma in a 24-year-old woman is presented. Her initial symptoms were diabetes insipidus and headaches. The neurological examination found no abnormalities, but the endocrinological examination disclosed a deficiency in growth hormone secretion. Her skull x-ray revealed a normal sella turcica, and a computed tomographic scan revealed no abnormal mass in the pituitary fossa, but a slightly enlarged stalk. Magnetic resonance imaging revealed a small tumor in the posterior lobe. Transsphenoidal exploration revealed a germinoma in the posterior pituitary lobe. Six cases of primary intrasellar germinoma, including our case, were reviewed. Our case is considered to be the smallest intrasellar germinoma yet reported.     

Chordoma in the sella turcica

A 75-year-old man presented with a rare case of chordoma in the sella turcica of the skull base. He had been treated for hypertension and chronic renal failure since 1990. Computed tomography detected a tumor in the sella turcica in 1994, but the patient had no clinical complaints and the serum pituitary hormone levels were normal. He died of disseminated intravascular coagulation, myocardial infarction, pulmonary infection, and multiple cerebral infarctions in 2000. At autopsy, the tumor in the sella turcica was 3.1 cm in greatest diameter and had compressed the pituitary gland posteriorly. Histological examination found oval cells and vacuolated short spindle-shaped cells which showed morphological changes similar to myxoma cells. The tumor was lobulated by narrow connective tissues. The tumor did not contain any cartilaginous tissue components, and was stained positively for epithelial membrane antigen but negatively for S-100 protein. The final diagnosis was chordoma. There was no association between the tumor and the cause of death.     

A case of cavernous angioma of the middle cranial fossa originating in the cavernous sinus with hyperprolactinemia

A 43-year-old female was admitted showing abnormal feeling in the left eye region. Left facial hypesthesia and quadrantic hemianopsia of left eye were the detectable neurological findings. A plain skull x-ray film revealed a double floor and dilatation of the sella turcica. The tomogram of the sella turcica demonstrated a dilatation of superior orbital fissure. Caudocranial projection revealed a dilatation of foramen ovale. A computerized tomography scan revealed a slightly high density tumor which was enhanced slightly with a contrast medium in the parasellar region and left middle fossa. Carotid angiogram demonstrated the displacement of the left carotid fork to the upper and lateral direction. However, no other sign was found. The sinogram of cavernous sinus clearly demonstrated an indentation in the left cavernous sinus. The endocrinological tests were almost normal except for hyperprolactinemia. A trigeminal neurinoma and a pituitary tumor were suspected. The tumor was totally removed and was histologically diagnosed as a cavernous angioma. At operation it seems to be extracerebral in the middle fossa and originate in the cavernous sinus. Diagnostic problems endocrine findings and the origin are discussed.     

经唇下-鼻中隔-蝶窦入路鞍区手术的非X线定位探讨

应用非X线综合测量定位法对经唇下鼻中隔-蝶窦入路鞍区手术(Sublabi0-septo-sphenoidaL APPROACH,SSSA)进行精确定位，以缩短时间、减少出血、提高手术成功率。对122例鞍区肿瘤患者在木中综合采用非X线定位法包括眉弓中点、前鼻棘-蝶鞍、前鼻棘走角定位法，上切牙尖、前鼻棘-蝶鞍、前鼻棘走角定位法，解剖学标志定位法(鼻中隔及犁状骨确定中线，蝶窦开口与犁状骨件为蝶窦前壁的标志，找寻蝶窦中隔矫正鞍底．鞍底的形态提供鞍底中点的标志，鞍区骨质破坏位置提示入路方向)．并采用X线片定位法矫正其精确度，结果为40例患者应用非X线综合测量定位鞍底位置深度后，再以X线片佐证，提示测量准确，手术时间平均在2.2h.82例患者直接采用非X线片定位法案合测量鞍底．手术顺利，手术时间平均在15h．平均输血量为200ml。无死亡及严重并发症。故非X线综合测量定位法可直接应用于经唇下一鼻中隔-蝶窦入路鞍区手术的精确定位。     

A successfully treated case of cerebrospinal fluid fistula caused by fracture of the sella turcica

Fracture of the sella turcica is rare and is associated with many complications. We successfully treated a cerebrospinal fluid fistula caused by a fracture of the sella turcica. A 66-year-old male in a motor vehicle accident was admitted to an outside hospital with disturbance of consciousness. A computed tomography (CT) scan of the head revealed a subarachnoid hemorrhage and pneumocephalus. Cerebrospinal fluid rhinorrhea developed after admission. Repair of the fistula was attempted without success, and the patient was transferred to our hospital for further examination and treatment. A fracture of the sella turcica was clearly visualized on coronal and sagittal head CT and on a three-dimensional reconstructed CT (3D-CT) image. The source of the CSF fistula was thought to be the anterior wall of the sella turcica. Through a bifrontal interhemispheric approach, the cerebrospinal fluid fistula was repaired microscopically with the assistance of endoscopy. Postoperatively, the fistula stopped completely. Coronal and sagittal head CT and 3D-CT images are useful for making a diagnosis of CSF leakage. Endoscopic images can assist in observation of the dead angle of the microscope.     

Visual improvement after chiasmapexy for primary empty sella turcica.

Postoperative visual improvement is described in a patient found to have primary empty sella turcica on evaluation of her sole symptom, progressive painless visual impariment. Chiasmal elevation by filling the sella turcia with muscle was found to be an alternative to opening the lamina terminalis and may be preferred in cases of empty sella which lack dense adhesions of the chiasm to the sella floor.     

Size-adjustable titanium plate for reconstruction of the sella turcica. Technical note

A size-adjustable plate constructed of pure titanium is proposed for use in the reconstruction of the sella turcica. The plate is composed of two semicircular pieces that are connected by a hinge located at the top of the plate. Using an applicator, the plate is inserted into the sella turcica in a closed position. The same applicator is then used to open and secure the plate. The titanium causes minimal ferromagnetic artifacts on postoperative magnetic resonance imaging. Preliminary findings indicate a possible clinical use for this plate in the reconstruction of the sella turcica when no suitable piece of bone is available.     

Persisting embryonal infundibular recess

Persisting embryonal infundibular recess (PEIR) is a rare anomaly of the third ventricular floor that has an unclear pathogenesis. In all 7 previously described cases, PEIR was present in adult patients and was invariably associated with hydrocephalus and, in 4 reported cases, with an empty sella. These associated findings led to speculations about the role of increased intraventricular pressure in the development of PEIR. In the present case, PEIR was found in a 24-year-old man without the presence of hydrocephalus or empty sella. Disorders of pituitary function had been present since childhood. Magnetic resonance imaging revealed a cystic expansion in an enlarged sella turcica. A communication between the third ventricle and the sellar cyst was suspected but not apparent. During transcranial surgery, the connection was confirmed. Later, higher-quality MR imaging investigations clearly showed a communication between the third ventricle and the sellar cyst through a channel in the tubular pituitary stalk. This observation and knowledge about the embryology of this region suggests that PEIR may be a developmental anomaly caused by failure of obliteration of the distal part of primary embryonal diencephalic evagination. Thus, PEIR is an extension of the third ventricular cavity into the sella. Although PEIR is a rare anomaly, it is important to identify when planning a procedure on cystic lesions of the sella. Because attempts at removal using the transsphenoidal approach would lead to a communication between the third ventricle and the nasal cavity, a watertight reconstruction of the sellar floor is necessary.     

Proposal for bipolar coagulation of the sella turcica wall during pituitary tumor operation

Convoluted morphology, limited visualization, and the lack of appropriate instruments limit the surgical approach to the walls of the sella turcica. They are, however, a site of local invasion and retained tumor fragments in cases of pituitary lesions. Effective bipolar coagulation within the sella turcica could reduce recurrence from local invasion, but it is made difficult because rigid bipolar forceps cannot fit effectively or safely against the complex contours of the walls there. Effective coagulation and thorough tumor removal are further frustrated by the lack of direct visualization of either the sella walls or the part of the floor adjacent to the surgical opening. It would be desirable, however, to coagulate the dura mater and retained tumor fragments. As an aid in this problem, we are reporting the use of a flexible bipolar coagulation system in two forms and of a mirror system for visualization in the sella without requiring the removal of one hand from surgical maneuvers. A pair of parallel flexible wire extensions to the standard bipolar forceps can be shaped by the surgeon into a contour that will coagulate the specific local terrain. A web of wire woven into one surface of a cotton patty, which is a bipolar coagulation system, supplements the wire loops. This allows the walls of the sella turcica to be coagulated more effectively where the contours are difficult to gauge and not well visualized. For visualization of the sella turcica walls and floor, we have used a small front surface mirror 1.0 mm thick.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ectopic pituitary adenoma in the suprasellar cistern: case report

The case of a 56-year-old man with an ectopic pituitary adenoma is reported. Neurological examinations, neurodiagnostic imaging, surgical observation, endocrinological evaluation, histological examination, and immunohistological study demonstrated evidence of ectopic prolactinoma in the suprasellar cistern and the presence of a normal pituitary in the sella turcica. The patient underwent total removal of the suprasellar mass by a pterional approach, leading to a surgical and endocrinological cure.     

ACTH dependent Cushing's syndrome with an empty sella turcica: report of three cases with emphasis on diagnostic value of selective venous sampling

Three cases of ACTH dependent Cushing's syndrome are reported with emphasis on diagnostic value of selective venous sampling. Case 1. A 44-year-old female was admitted to our hospital with clinical diagnosis of Cushing's disease. Endocrinological examination revealed typical data of Cushing's disease. High resolution CT scan showed an empty sella turcica, and a chest film showed multi-cystic lesion in the left lower lung field. In the first trial of selective venous sampling, central to peripheral ACTH ratio (C/P ratio) was high at the superior vena cava. So, an ectopic ACTH producing lung tumor was strongly suspected. Further examinations for lung tumor were performed, and finally showed lung cryptococcosis. Therefore, selective venous sampling was performed again, and pituitary ACTH dependency was diagnosed. An eccentric pituitary microadenoma was successfully removed by transsphenoidal surgery. Case 2. A 54-year-old female was admitted to our hospital with clinical diagnosis of Cushing's disease. In the endocrinological examinations plasma ACTH was not respond to provocation of LVP or CRF. In selective venous sampling, C/P ratios of ACTH were not greater than 2.0 at any sampling site. Further examinations showed lung tumor in the lower lobe of left lung. This tumor was surgically proved to be an ectopic ACTH producing lung carcinoid. Case 3. A 24-year-old female was admitted to our hospital for the purpose of further examination of Cushing's disease. Three years previously exploratory transsphenoidal surgery demonstrated an empty sella without adenoma. She received postoperative radiation therapy with a dose of 5000 rad. Endocrinological examination showed typical data due to Cushing's disease.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pituitary adenoma composed of two compartments each secreting prolactin or growth hormone

We report a case of pituitary adenoma with two compartments, i.e. a part within the sella turcica and a part infiltrating the sphenoid bone beneath the sella, producing growth hormone (GH) and prolactin. The patient had the amenorrhea-galactorrhea syndrome, but not acromegalic symptoms. Although the two compartments were united through a small dural perforation, immunohistochemical studies demonstrated that GH and prolactin were separately secreted from the intrasellar and extrasellar components, respectively. Somatotropic adenomas frequently produce prolactin, but it is unusual for these hormones to be secreted from distinctly different parts of the same lesion.     

Craniofacial morphology in untreated and shunt-treated hydrocephalic children

Craniofacial morphology was studied in 45 shunt-treated hydrocephalic children and 7 untreated hydrocephalic patients. A sample of 74 normal children from northern Finland were used as controls. Following shunt treatment the sella turcica became shallow and J-shaped. The cranial base angles changed markedly during shunt treatment. The cranial base angles were more obtuse in untreated patients than in control subjects, whereas the opposite was the case in shunt treated patients. The Nasion-Sella-Basion angle was 143.4 degrees in untreated hydrocephalic patients, 132.6 degrees in normal subjects and 127.9 in shunt-treated hydrocephalics. The changes in cranial base angles appeared to be progressive during a two-year follow-up period.     

Delayed cerebrospinal fluid rhinorrhea seven months after transsphenoidal surgery for pituitary adenoma--case report

A 51-year-old female had undergone transsphenoidal surgery for pituitary adenoma producing growth hormone. Cerebrospinal fluid (CSF) leakage occurred during surgery. The sella turcica and sphenoid sinus were packed with abdominal fat and fibrin glue, buttressing the closure with a fragment of sphenoid bone. No CSF rhinorrhea occurred postoperatively. Severe meningitis developed 7 months later. CSF rhinorrhea occurred 10 days after readmission. Exploration through the transsphenoidal approach identified a small hole at the floor of the sella and CSF leaking into the sphenoid sinus through the hole. The CSF leakage stopped after the second surgery. Delayed CSF rhinorrhea without bromocriptine administration is very rare. The cause of delayed CSF rhinorrhea remains unclear. CSF rhinorrhea should be suspected if meningitis develops even months after transsphenoidal surgery.