Orthostatic headache from spontaneous spinal cerebrospinal fluid leakage; diagnosed by heavily T2-weighted magnetic resonance myelography.

Orthostatic headache is derived from low cerebrospinal fluid (CSF) pressure as evidenced by cranial magnetic resonance myelography (MRM). This reports three cases of patients coming with orthostatic headache without previous obvious spine trauma. The first two cases had headache with radiating neck pain while the third case had headache with radiating pain to the eye sockets or occasional nausea. The third case was diagnosed from cranial MR imaging. The three cases were not done for CSF opening pressure measuring or criterion's method myelography, but had done T2-weighted MR. All of three cases had spinal epidural collections. The second case had meningeal diverticula. The present report found a possible site of leak in all cases. In the present report, T2-weighted MR myelography could avoid dural puncture. It was used as a non-radiation exposure investigating technique. This technique can be used as the first line of investigation prior to CTM, guiding a radiologist to seek the most likely site of leak during CTM study.

Sphenoid Sinus Cholesteatoma-Complications and Skull Base Osteomyelitis: Case Report and Review of Literature.

INTRODUCTION: Cholesteatoma of the paranasal sinuses is uncommon. Its clinical characteristics are an expanding growth of the affected paranasal sinuses consisting of keratinizing squamous epithelium with bony wall destruction. Among involved paranasal sinuses, sphenoid sinus cholesteatoma is the least common. CASE PRESENTATION: An 82-year-old female diabetic patient presented with subacute onset of fever after experiencing chronic progressive headaches for more than 20 years. Nasal endoscopy found purulent discharge from left sphenoethmoidal recess. Computed tomography (CT) scan of the paranasal sinus showed soft tissue lesions that totally filled the left sphenoid sinus with posterior and inferior wall destruction. There was no evidence of connection to the left mastoid cavity. MANAGEMENT AND OUTCOME: Left sphenoidotomy was performed. Histopathology revealed cholesteatoma. Two months after surgery, she became worse and CT showed extensive skull base destruction. The patient underwent bilateral sphenoidectomy and craniotomy with surgical debridement of osteomyelitis of the skull base. She received long-term intravenous ertapenam and sitafloxacin for treating drug-resistant Klebsiella infection. The osteomyelitis could not be controlled, and she died. DISCUSSION: Progressive headache can be caused by an uncommon disease such as sphenoid sinus cholesteatoma, which is a surgical condition. Complicating osteomyelitis of the skull base requires extensive debridement surgery and should be anticipated.

Heterozygous nonsense mutation SATB2 associated with cleft palate, osteoporosis, and cognitive defects.

Studies of human chromosomal aberrations and knockout (KO) mice have suggested SATB2 as a candidate gene for a human malformation syndrome of craniofacial patterning and brain development. Of 59 unrelated patients with craniofacial dysmorphism, with or without mental retardation, one 36-year-old man had a nonsynonymous mutation in SATB2. The affected individual exhibited craniofacial dysmorphisms including cleft palate, generalized osteoporosis, profound mental retardation, epilepsy and a jovial personality. He carries a de novo germline nonsense mutation (c.715C>T, p.R239X) in the exon 6 of SATB2. Expression studies showed that the mutant RNA was stable, expected to produce a truncated protein predicted to retain its dimerization domain and exert a dominant negative effect. This new syndrome is the first determined to result from mutation of a gene within the family that encodes nuclear matrix-attachment region (MAR) proteins.

Radiographic manifestations of pulmonary cryptococcosis.

Pulmonary cryptococcosis may occur in both immunocompromised and immunocompetent hosts. The purpose of the present study was to review the radiologic findings of pulmonary cryptococcosis in King Chulalongkorn Memorial Hospital. The radiographs and computed tomography of the chests of the patients who had a diagnosis of pulmonary cryptococcosis, between 1998-2001, were retrospectively reviewed. Seven patients were included. Five were HIV infected, three had diffuse reticulonodular opacities, two (of the three) patients also had accompanying cavities; two had solely pleural effusion. Two patients were immunocompetent; one had a pulmonary nodule and another one had an endobronchial lesion and multiple pulmonary masses in the collapsed lung seen on CT scan, which were consistent with cryptococcoma. None had adenopathy. There was a difference in the radiologic manifestations between immunocompromised and immunocompetent hosts. Knowledge in radiographic features in pulmonary cryptococcosis should help radiologists to early recognize the disease and may improve the treatment outcome.

The orbital floor is a surgical landmark for the Asian anterior skull base.

BACKGROUND: Surgical landmarks for defining the level of the skull base during endoscopic sinus and skull base surgery (ESBS), e.g., the middle turbinate, may be distorted by tumor or previous surgery. The orbital floor is a valid fixed anatomic landmark for the white population, but it is not known if its use is useful for an Asian population. OBJECTIVES: To define fixed anatomic landmarks for ESBS in an Asian population and to compare the level of the skull base and its relation with anatomic landmarks between Asian and white populations. METHODS: Computed tomographies performed on paranasal sinuses of Thai patients were retrospectively assessed. The distance between the nasal floor to four structures, the orbital floor, sphenoid planum, cribriform, and ethmoid roof, were measured. The level of skull base related to the orbital floor of an Asian population was compared with data of a white population from a previously published study. RESULTS: A total of 150 Thai patients (300 paranasal sinus systems) were assessed and compared with 150 white patients. The orbital floor was always below the skull base (600 sides [100%]). When compared with white patients, the Asian patients had significantly higher mean (standard deviation [SD]) values: orbital floor (35.2 ± 3.4 mm versus 33.9 ± 3.0 mm; p < 0.001), ethmoid roof (49.3 ± 3.8 mm versus 48.4 ± 4.5 mm; p = 0.01), cribriform (46.4 ± 3.6 mm versus 44.0 ± 3.7 mm; p < 0.001), and sphenoid roof (45.7 ± 3.7 mm versus 44.9 ± 3.7 mm; p = 0.01). The Asian population had a mean (SD) longer distance from the orbital floor to the cribiform (11.2 ± 2.5 mm versus 10.1 ± 2.7 mm; p < 0.001), a shorter distance to the sphenoid roof (10.5 ± 3.3 mm versus 11.0 ± 2.9 mm; p = 0.03), and a similar distance to the ethmoid roof (14.1 ± 3.1 mm versus 14.5 ± 3.5 mm; p = 0.09). CONCLUSION: Although a statistical difference exists between racial groups, clinically, the orbital floor is a useful fixed anatomic landmark for ESBS for both Asian and white populations.

MR imaging of CNS leiomyosarcoma in AIDS patients.

Leiomyosarcomas of the central nervous system are extremely rare; however they are becoming more prevalent in immunocompromised patients. The authors present MRI (Magnetic Resonance Imaging) of six cases of pathological proved leiomyosarcomas of the central nervous system in patients infected with human immunodeficiency virus. MR images of 4 cases of intraspinal leiomyosarcoma showed lobulated masses expanding multilevel of neural foramina with extradural and intradural extension, giving dumbbell appearance which mimic neurofibroma. Two cases of intracranial leiomyosarcoma revealed a mass at the left cavernous sinus involving prepontine cistern in one case and two lesions in the other case showing masses with dural based appearance at the region of the planum sphenoidale and the posterior aspect of the falx cerebri which mimiced a meningioma. The leiomyosarcoma should be included in the differential diagnosis of extra-axial CNS lesions in HIV-infected patients.

Does nasal irrigation enter paranasal sinuses in chronic rhinosinusitis?

BACKGROUND: Nasal irrigation is widely used in treating sinonasal diseases. Not only does it remove static secretions and promote mucociliary clearance, but, in chronic rhinosinusitis, nasal flush is also a potential route for topical drug administration into paranasal sinuses. A clinical study was conducted to investigate how well nasal irrigation could reach paranasal sinuses with the ostiomeatal units blocked in chronic rhinosinusitis. This study was performed to (1) assess the ability of a nasal douche and spray to deliver a solution into the paranasal sinuses in chronic rhinosinusitis and (2) compare the performance of the two techniques. METHODS: Fourteen patients, with bilateral chronic rhinosinusitis, underwent nasal irrigation with 140 mg/mL of iodinated contrast solution by 40 mL of douching using an irrigation syringe in one side, and 10 mL of spraying in the other side. A computed tomography scan was undertaken for each patient to determine the volume and the distribution of staining in the nose and paranasal sinuses. RESULTS: Only two patients had any staining, with a small amount present in a total of three maxillary sinuses (0.10 mL, 0.04 mL, and 0.13 mL). The mean volumes of paranasal sinus staining by nasal douche and nasal spray were 0.0093 and 0.01 mL, respectively. We found that the two techniques had a similar performance. Both of them delivered only a small amount of the solution, if any, into the sinuses (with a mean difference of -0.0007 mL; 95% CI, -0.02-0.02 mL; p = 0.94). CONCLUSION: Nasal douche and spray is not effective in delivering a nasal irrigation solution into paranasal sinuses in chronic rhinosinusitis.