A cephalometric inter-centre comparison of growth in children with cleft lip and palate.
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AIM: To examine whether the treatment provided by the Mount Vernon Cleft Team produces craniofacial growth outcomes comparable with that of the Oslo Team. LOCATION: Mount Vernon Hospital, Middlesex, UK. DESIGN: A retrospective cephalometric investigation. SUBJECTS: Seventy-five Mount Vernon children and 150 Oslo children with complete unilateral or bilateral clefts of the lip and palate METHOD: The subjects were matched for age, gender, and cleft type, and their radiographs were digitized. The radiographs from each site were grouped according to patient age (9-11 or 14-16) and cleft classification (bilateral/unilateral). Patients with associated craniofacial anomalies were excluded from the study. RESULTS: Of the four variables studied (SNA, SNPg, NGn, sNANsPG) significant differences in maxillary growth were noted for bilateral and unilateral cleft groups at 14-16 years of age. The soft tissue profile was significantly flatter in bilateral and unilateral Mount Vernon cases at 14-16 years. The craniofacial growth exhibited by the Mount Vernon patients demonstrated 3.9-5.1 degrees reduction in maxillary prominence with respect to the Oslo sample. The bilateral cases from Mount Vernon had greater anterior face heights at 14-16 years. CONCLUSION: The treatment provided by the Mount Vernon Cleft team leads to a reduced maxillary prominence in children aged 14-16 years compared with the Oslo sample. This reduction is statistically significant in unilateral cleft lip and palate. (+info)
Infrasellar craniopharyngioma mimicking a clival chordoma: a case report.
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An unusual case of entirely infrasellar craniopharyngioma mimicking a clival chordoma is described. Only 22 cases of craniopharyngioma with nasopharyngeal extension have been reported in the literature. Of the reported cases, most were primarily intracranial with secondary downward extension; only two were thought to originate from an infrasellar location. The present case is another example of an entirely infrasellar craniopharyngioma, with extensive clival destruction, mimicking a clival chordoma. Relevant literature on the subject is reviewed. (+info)
Chordoma in the sella turcica.
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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. (+info)
Asymptomatic spontaneous rupture of suprasellar dermoid cyst: a case report.
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Suprasellar dermoid cysts are uncommon intracranial lesions. CT and MRI findings in a rare case of asymptomatic rupture of suprasellar dermoid cyst with subarachnoid dissemination is described. (+info)
Pituitary size in patients with Laron syndrome (primary GH insensitivity).
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OBJECTIVE: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron syndrome, leads to an increase in the size of the pituitary gland. METHODS: Eleven patients (six females, five males) with Laron syndrome underwent magnetic resonance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36-68 Years and two children, a 4-Year-old boy and a 9-Year-old girl. The latter patient had been treated with IGF-I (150-180 mg/kg per day) since the age of 3 Years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and compared with reference values for age and sex. RESULTS: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary-hypothalamic region were detected. CONCLUSION: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary-hypothalamic region in Laron syndrome is normal. (+info)
Pharyngeal pituitary non-functioning adenoma with normal intra-sellar gland: massive tumor shrinkage on octreotide therapy.
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OBJECTIVE: Functioning or non-functioning ectopic tumors may develop from pharyngeal pituitary remnants. They constitute <1% of all obstructive pharyngeal masses and they have a strong tendency to bleed. We report a case of a non-functioning ectopic pituitary adenoma of the rhino-pharynx studied over a long-term somatostatin analog treatment. PATIENT AND TREATMENT: A 60-Year-old woman presented with severe posterior epistaxis. She had complained of nasal obstruction for the past 2 Years. Magnetic resonance imaging (MRI) and endoscopic examination revealed a 2 cm exophytic, bleeding mass in the cavum, which was judged inoperable, and a biopsy was performed. On immunostaining, tumor cells were positive for pancytokeratins MNF 116 and C11, epithelial membrane antigen, chromogranin and neuron-specific enolase (NSE), and negative for synaptophysin, desmin, actin, estrogen and progesterone receptors, all anterior pituitary hormones and human chorionic gonadotropin. Blood levels of the above hormones and tumor markers were normal, except for a moderate elevation of NSE (33.8 microg/l, normal value <12 microg/l). It was concluded that this was a non-functioning pituitary adenoma of the rhino-pharynx. MRI showed a normal intra-sellar pituitary gland, including the normal bright signal of the posterior lobe. Somatostatin receptor scintigraphy (SRS) disclosed intense tracer uptake in the tumor, indicating high somatostatin receptor content. There was also an intense uptake in the intra-sellar pituitary. Therapy with long-acting octreotide was started, 20 mg per Month i.m. RESULTS: The patient has been on octreotide for the last 12 Months. Nasal obstruction rapidly subsided and bleeding did not recur. Repeated endoscopic examinations showed rapid tumor reduction, the mass shrinkage being almost complete at 3 Months. This was confirmed by MRI, while SRS showed markedly decreased uptake in the residual tumor and the intra-sellar pituitary, and NSE became normal. CONCLUSION: Pharyngeal pituitary remnant adenomas are rare, but they must be considered in the differential diagnosis of bleeding or obstructive masses of the rhino-pharynx. In this case, the positive SRS influenced the choice of octreotide, as an alternative to surgery. As we show for the first time in this location, octreotide can exert prolonged and marked anti-tumoral effects in non-functioning adenoma. (+info)
Longitudinal cephalometric standards for the neurocranium in Norwegians from 6 to 21 years of age.
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The purpose of this study was to establish and describe normative cephalometric standards of the neurocranium (theca cranii and cranial base) for Norwegian males and females from 6 to 21 years of age using lateral cephalograms. The subjects included 35 males and 37 females from the Oslo University Growth Archive with lateral cephalograms taken every third year from 6 to 21 years of age. The total number of lateral cephalograms was 194 from males and 200 from females. All subjects were Caucasian, all had normal occlusion and no apparent facial disharmony, and none had undergone orthodontic therapy. Nineteen measurements and three indices of the neurocranium were analysed longitudinally. Comparisons between the various parameters in the neurocranium of males and females in each age group were performed using the Student's t-test. The size of the neurocranium of females was smaller than that of males throughout the observation period and the differences increased with age, particularly the diameter of the neurocranium (n-l), length of the neurocranium (n-opc), anterior cranial base length (n-s), and posterior cranial base length (s-ba). The cephalometric standards of the neurocranium established in this study can be used as a reference material in investigations of individuals with various craniofacial aberrations and syndromes. (+info)
Differentiation of pituitary adenomas from other sellar and parasellar tumors by 99mTc(V)-DMSA scintigraphy.
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Pentavalent technetium-99m dimercaptosuccinic acid [99mTc(V)-DMSA] scintigraphy was evaluated for the differentiation of pituitary adenomas, especially non-functioning adenomas, from other sellar and parasellar lesions. Diffuse 99mTc(V)-DMSA accumulation within the tumor was found in seven of seven non-functioning, three of four growth hormone-secreting, and seven of eight prolactin-secreting adenomas, but only partial accumulation in only two of 16 non-pituitary adenomas and normal pituitary glands. There were no significant relationship between tumor-to-background ratios and tumor size or serum hormone level. 99mTc(V)-DMSA scintigraphy showed overall sensitivity of 81% (17/21 cases) for detecting pituitary adenomas, in particular 100% for non-functioning adenomas. 99mTc(V)-DMSA may be useful for detecting pituitary adenomas, especially non-functioning adenomas, and for the differentiation of non-functioning pituitary adenomas from other sellar and parasellar lesions. (+info)