Spontaneous Transethmoidal Meningoceles in Adults: Case Series with Emphasis on Surgical Management.

BACKGROUND: Spontaneous onset transethmoidal meningocele is a rare entity among the adult population. METHODS: A retrospective chart review was performed and cases of adults diagnosed with spontaneous transethmoidal meningoceles from November 2000 till February 2014 were reported. Data collected included demographics, clinical presentation, diagnostic modalities, and results. Intraoperative findings, the type of surgical reconstruction performed, and the percentage of recurrence, if present, were also reported. RESULTS: Ten cases of spontaneous transethmoidal meningoceles in adults were diagnosed. Eight were females and two males with a mean age of 47.5 years. All patients presented with CSF leakage with or without meningitis. They underwent a reconstruction of the base of skull defect using the temporalis fascia graft in addition to fibrin glue (Tissucol) and Surgicel (Ethicon). In two cases with a larger defect, a piece of septal bone and turbinate mucosa were applied achieving a watertight seal in all cases. CONCLUSION: Spontaneous transethmoidal meningocele in adults is a rare condition. It usually presents with clear rhinorrhea with or without meningitis and an endoscopic multilayer reconstruction is advocated for treatment of such conditions.

Autosomal dominant hyaline body myopathy: clinical variability and pathologic findings.

OBJECTIVE: To report clinical, morphologic, and immunohistochemical studies on autosomal dominant, clinically nonprogressive, and not previously described progressive forms of hyaline body (HB) myopathy (HBM) in a Saudi Arabian kindred. RESULTS: Muscle biopsies from four patients showed HB in type 1 fibers; they were positive for ATPase at pH 4.3/4.6 and for heavy chain slow myosin (HCSM); some HB were HCSM negative. HB were nonreactive for alphaB-crystallin, ubiquitin, tropomyosin, actins, desmin, and components of sarcolemma. Ultrastructurally, HB were granular and filamentous or amorphous, often with fragments of sarcomeres, and surrounded by a zone of sarcomeric disorganization. All biopsies showed "myopathic" changes, angulated neurogenic fibers, and fiber type grouping. There was no correlation between HB and course of disease; the progressive cases displayed more severe myopathic features. CONCLUSIONS: Formation of hyaline bodies in hyaline body myopathy is associated with either myolysis or defective incorporation of heavy chain slow myosin into the cytoskeleton. Hyaline bodies very likely contain additional unidentified proteins. Neurogenic factors are also involved in the hyaline body myopathy pathogenesis.

Laryngotracheoplasty using the Medpor implant: an animal model.

OBJECTIVE: The Medpor implant (Porex Surgical, College Park, GA) is a porous polyethylene that has been used safely in areas in the head and neck. Medpor allows the in-growth of capillaries and soft tissue and reduces the risk of infection and extrusion. This study was undertaken to evaluate the use of the Medpor implant in laryngotracheal reconstruction and to compare epithelialization of the trachea following two different methods at various intervals. METHODS: Eight beagles were divided into two groups. The first group underwent laryngotracheoplasty using the Medpor implant only, whereas the second group had the Medpor implant lined with a strap muscle flap. One animal from each group was sacrificed at 5-, 7-, 9-, and 12-month intervals. The degree of stenosis, inflammation, and epithelialization were compared. RESULTS: The Medpor implant can safely be used to reconstruct the trachea in this animal model. There is less stenosis and better epithelialization when the Medpor implant is lined with a strap muscle flap. CONCLUSION: The Medpor implant can safely be used in laryngotracheal reconstruction in beagles.

Localization of connective tissue growth factor in human uterine tissues.

Connective tissue growth factor (CTGF) is a recently described heparin-binding mitogen for fibroblasts and smooth muscle cells. The aim of this study was to investigate the production of CTGF by human uterine tissues using immunohistochemical and Northern blotting analyses. For immunohistochemistry, formalin-fixed human proliferative (n = 5), early secretory (n = 5; days 15-19), mid-secretory (n = 5; days 20-23), late secretory (n = 5; days 24-28) endometrial, and decidual (n = 5) tissues were stained using a highly specific affinity-purified polyclonal antibody raised against residues 81-94 of human CTGF. Myometrial (n = 5) and leiomyoma (n = 5) tissues were also used for CTGF immunochemistry. In proliferative endometrium, epithelial and vascular endothelial cells showed strong CTGF immunoreactivity, whereas stromal cells were negative or only weakly positive for the CTGF protein. Throughout the entire secretory stage, CTGF was detected in epithelial and endothelial cells of endometrium. Stromal cells showed strong immunoreactivity to CTGF only in oedematous areas for early and mid-secretory endometrium, and in decidualized regions of late secretory endometrium. During pregnancy, the decidual, epithelial and endothelial cells of the endometrium were all immunoreactive to CTGF. In myometrial and leiomyoma samples, CTGF immunoreactivity was found only in the endothelial cells. Northern blotting of mRNA from normal uterus (n = 2) or leiomyoma (n = 6) using a 320 bp human CTGF cDNA probe revealed a single 2.4 kb transcript. This study is the first to demonstrate CTGF gene expression and localization of its encoded protein in human uterine tissues. The cell- and cycle-specific localization of CTGF support a role for this molecule in regulating aspects of uterine cell growth, migration, and/or matrix production during the menstrual cycle and pregnancy.

Gaucher disease with oculomotor apraxia and cardiovascular calcification (Gaucher type IIIC).

The authors describe four siblings from consanguineous parents who presented with oculomotor deficit in early childhood characterized by impaired volitional horizontal saccades, compensatory lateral head thrust, and preservation of vertical movement. When about 10 years of age, heavily calcified aortic and mitral valves required surgery. Fibroblast beta-glucocerebrosidase activity was markedly reduced. Genotype analysis indicated that the two patients who were tested were homozygous for the D409H (1342G-->C) mutation. Relating this rare phenotype of Gaucher disease to D409H mutation will facilitate management of the disease and counseling of families.

Pseudoxanthoma elasticum of the internal mammary artery.

A case of pseudoxanthoma elasticum of the left internal mammary artery from a 61-year-old male who underwent coronary artery bypass grafting is reported. Intraoperative evaluation of the left internal mammary artery revealed partial stenosis. Histologically, the stenotic portion showed pseudoxanthoma elasticum. This represents the first reported case of pseudoxanthoma elasticum in the internal mammary artery.

Non-neoplastic cystic lesions of the sellar region presentation, diagnosis and management of eight cases and review of the literature.

The differential diagnosis of a sellar lesion includes pituitary adenoma, cranio-pharyngioma, tumour cyst, benign cyst, and other less common lesions such as aneurysm, squamous cell carcinoma and metastases. Pure cystic lesions within the sella turcica are not uncommon and may appear clinically and radiologically as pituitary adenoma. Intrasellar cysts are broadly classified as neoplastic or non-neoplastic; the latter may be primary lesions of the pituitary fossa or they may arise from the parasellar region and invade into the sella. The lesions occupy space and may cause disturbance of pituitary function; if they extend into the suprasellar region they can affect visual fields and acuity. Non-neoplastic cystic lesions usually appear well defined radiologically and have distinct pathological features. Modern imaging modalities, such as computed axial tomography and magnetic resonance imaging, are important complementary tools of investigation for reaching a differential diagnosis, and for surgical planning. Most non-neoplastic sellar cysts can be approached through the trans-sphenoidal route, but large lesions with suprasellar extension may require transcranial surgery. We report the presentation and management of eight cases (four males, four females, all adult) of non-neoplastic cyst involving the sella turcica and the parasellar region. Investigations included computed tomography scan of the head for all and magnetic resonance imaging for six (75%) patients. Work-up included endocrinological and opthalmological evaluations. All presented with headache; six (75%) had visual acuity change, two (25%) had evidence of visual field defects, four (50%) had optic atrophy on fundoscopy, three (37.5%) had endocrine symptoms and hormone reduction was found in four (50%).

Neurosurgical aspects of sphenoid sinus mucocele.

The aetiological spectrum of sphenoid sinus mucocele includes congenital anomaly, trauma, infection, allergy and surgery of the sphenoid sinus. Enlargement of the mucocele, even with a short history, can result in progressive expansion of the sinus and extension of the lesion into the pituitary fossa, the suprasellar region, nasopharynx, orbits, clivus or ethmoid air cells. It is a benign cystic lesion with an excellent prognosis when treated appropriately. Generally, these lesions are managed by an ear, nose and throat surgeon, but when there is extension into the sellar and parasellar (especially suprasellar) regions they are managed by the neurological surgeon. Sphenoid sinus mucocele should be considered in the differential diagnosis when there is suspicion of a cystic lesion in these regions. Three cases of large sphenoid sinus mucocele are presented, with discussion on their neurosurgical management and a review of the literature.

Neuromuscular and microvascular changes associated with chronic administration of an extract of Teucrium stocksianum in mice.

This work examines the effect on the weights of vital body organs, on blood biochemical variables, on neuromuscular coordination and on cerebral microcirculation of aqueous extracts of Teucrium stocksianum, given to mice in drinking water at concentrations of 2 and 4% for 56 days. The treatment caused progressive impairment of neuromuscular coordination, as evidenced by the time spent on the rota-rod. After photochemical challenge, the time for first observable platelet aggregation in arterioles was shorter than for the control group by 22 and 45% in the 2 and 4% T. stocksianum-treated groups, respectively. Platelet aggregation on the venular side was not affected by the treatment nor were microvascular diameters. Treatment with the plant extract produced no statistically significant effect on the plasma biochemical variables that are considered indices of liver and kidney function. Histologically, brains obtained from mice treated with T. stocksianum showed loss of cerebellar Purkinje cells. Although it is likely that the accelerated platelet aggregation might have contributed to an ischaemic effect which could, at least in part, have caused the cytotoxicological changes, this does not exclude the possibility of a direct cytotoxicological effect of the plant extract. Further pharmacological and toxicological investigations on Teucrium species seem warranted.

Toxicological effects of Teucrium stocksianum after acute and chronic administration in rats.

Because of the widespread use of T. stocksianum (Boiss) in herbal medicine and reports of the toxicity of Teucrium chamaedrys to man, the effects of acute (2 and 4 g kg-1 single dose) and chronic (4% in lieu of drinking water for 48 days) administration of an aqueous extract of T. stocksianum has been studied in rats. After acute administration no change was found in reduced liver glutathione content, plasma total protein concentration or the enzyme activities of aminotransferase or gamma glutamyl transferase. After chronic administration, no change was noticed in the plasma concentrations of total protein, total bilirubin, creatinine, urea, glucose, triglycerides, calcium or phosphorus or the enzyme activities of aminotransferase, alanine aminotransferase, creatinine kinase, gamma glutamyl transferase or lactate dehydrogenase. There was no change in food or water intake or output of urine or faeces; the body weight of the treated animals was, however, slightly reduced. No change was observed in the weight of vital body tissues. Histological examination revealed occasional hepatic 'apoptosis' and cerebral neuronal loss in the cortex and hippocampus in treated animals; focal loss of Purkinje cells in the cerebellum was particularly noticed. The results did not indicate a major hepatotoxic effect of acute or chronic administration of T. stocksianum, unlike other Teucrium spp. We report a neurotoxic effect, however, which warrants monitoring of neurological function in people taking this plant.

Clinicopathological assessment of gastric biopsy samples of patients with Helicobacter pylori infection--metronidazole resistance and compliance problems in the United Arab Emirates.

The significance of Helicobacter pylori (HP) infection was assessed prospectively in forty-two patients with dyspepsia using histological, bacteriological and biopsy urease techniques. Thirty-eight patients (90.5%) were positive for HP infection and were treated with bismuth subcitrate (De Nol), tinidazole and doxycycline. HP was present in the antrum, corpus, fundus, duodenum and gastric juice in 36, 26, 23, 2 and 2 patients respectively (p < 0.01, X2 test). Histological assessment yielded more positive identifications of HP than the urease test (36 vs 28 positive cases, p < 0.01, McNemar's X2 test), while histology and bacteriology were virtually identical (38 vs 37 of 41 pairs, p > 0.5, X2 test). There was a good correlation between bacterial and polymorphonuclear leucocyte (PMNL) counts per high power field (r = 0.8; p < 0.001; n = 34 pairs). There was resistance to metronidazole in 10 out of 16 isolates, but no resistance was recorded against tetracycline (p < 0.001, X2 test). Among the sixteen patients who attended follow-up endoscopy, there was clinical improvement and no evidence of HP in 5 individuals (31.25%). One patient had amelioration of his symptoms, 5 experienced no change and in 5 their symptoms became worse. Metronidazole resistance may be one of the important factors in the United Arab Emirates and elsewhere.

Imprints, smears, and frozen sections of brain tumors.

In this study, we compared the suitability and accuracy of imprints, smears, and frozen sections of suspected brain and spinal cord tumors of 150 patients. Eighty-six percent of the imprints, 91% of the smears, and 99% of the frozen sections were suitable for interpretation. Among the suitable preparations, 82% of the imprints, 92% of the smears, and 99% of the frozen sections agreed with our diagnosis on paraffin sections. Although frozen sections were clearly more accurate than imprints and smears, the latter two provided diagnoses in patients with acquired immunodeficiency syndrome where frozen sections were not done to avoid contaminating our cryostat and in a patient with an epidermoid cyst of the middle fossa that could not be adequately frozen sectioned. Our study shows that imprints and smears complement frozen sections in the intraoperative diagnosis of tumors of the central nervous system.

Malignant lymphoma presenting as a chronic subdural hematoma.

A 56-year-old man had a right frontotemporoparietal craniotomy for evacuation of an encapsulated subdural hematoma that had reaccumulated after burr-hole decompression 6 weeks before. Histopathologic examination showed a malignant lymphoma in the subdural hematoma. Our patient shows that malignant lymphoma can rarely present as a chronic subdural hematoma.

Aneurysmal bone cyst of the thoracic spine: treatment by excision and segmental stabilization with Luque rods.

Preoperative radiological evaluation with magnetic resonance imaging and computed tomography was valuable in planning the surgical management of a destructive lesion of the posterior elements of the thoracic spine that was causing spinal cord compression in an 18-year-old woman. Preoperative recognition of bilateral involvement of the pedicles in addition to the laminae and spinous process led to use of prophylactic segmental stabilization of the spine with Luque rods after successful excision of an aneurysmal bone cyst. This case provides an example of the usefulness of computed tomographic scanning and magnetic resonance imaging in assessing the distribution and location of vertebral tumor and its potential effect on spinal stability. The efficacy of combining radical excision with stabilization for treatment of aneurysmal bone cysts of the spine is emphasized.