Application of three-tesla magnetic resonance imaging for diagnosis and surgery of sellar lesions

OBJECT: The aim of this study was to determine the value of high-field magnetic resonance (MR) imaging for diagnosis and surgery of sellar lesions. METHODS: High-field MR images were obtained using a 3-tesla unit with emphasis on sellar and parasellar structures in 21 patients preoperatively to delineate endo-, supra-, and parasellar anatomical structures. Special attention was given to the medial border of the cavernous sinus and possible invasion of a sellar tumor therein, and to assessing the application of high-resolution images during intraoperative neuronavigation. The 3-tesla MR images were compared with the standard MR images already obtained and with intraoperative findings. Anatomical structures were studied in all 42 cavernous sinuses; in 32 of them comparisons with intraoperative findings were possible. The medial cavernous sinus border was rated intact in 53% on standard MR images, in 72% on 3-tesla MR images, and in 81% intraoperatively. With a positive correlation to surgical findings on 84% of 3-tesla MR images compared with 59% of standard MR images, a sensitivity of 83% compared with 67%, and a specificity of 84% compared with 58% (p = 0.016, McNemar test), 3-tesla MR imaging was superior for predicting tumor invasion through the medial cavernous sinus border. Although no difference was noted in delineation of the medial, superior, and inferior compartments, there was a better delineation of the lateral cavernous sinus compartment with 3-tesla MR imaging. This compartment was clearly visible on 40 sides (95%) on 3-tesla MR images compared with 34 sides (81%) on standard MR images. Identification of the cavernous sinus segments of the third, fourth, fifth (V1 and V2), and sixth cranial nerves was improved using high-resolution 3-tesla imaging compared with standard MR imaging. A mean of four cranial nerves was found as hypointense spots (range two-five spots) on 3-tesla MR imaging compared with a mean of three (range zero-four spots) on standard MR imaging. After addition of contrast agents, the anterior pituitary gland was found to be highly intense on 78% of T1-weighted three-dimensional magnetization-prepared rapid acquisition gradient-echo (MPRAGE) 3-tesla MR images compared with 73% of standard T1-weighted MR images. The optochiasmatic system displayed increased intensity on pre-contrast T1-weighted MPRAGE 3-tesla compared with standard T1-weighted MR images; it was hyperintense on 76% of 3-tesla compared with 15% of standard MR images, which was helpful for its delineation from suprasellar pituitary and tumor structures. Intraoperative navigation guided by fusion of 3-tesla MR images and computerized tomography (CT) scans was performed in seven patients. Whereas CT scanning was used during the transsphenoidal approach to depict the nasal bone structures, 3-tesla MR imaging was particularly useful for the visualization of parasellar tumor extension during microsurgical and/or endoscopic resection. CONCLUSIONS: Due to its higher resolution, 3-tesla MR imaging was found to be superior to standard MR imaging for the delineation of parasellar anatomy and tumor infiltration of the cavernous sinus, and this modality provided improved imaging for intraoperative navigation.     

Osteogenesis imperfecta in childhood: impairment and disability. A prospective study with 4-year follow-up

OBJECTIVES: To study (1). changes in anthropometrics, joint range of motion (ROM), muscle strength, functional ability, caregiver assistance, and level of ambulation in children with osteogenesis imperfecta (OI) and (2). the prediction of clinical characteristics at the level of ambulation at follow-up and the prediction of clinical characteristics on progression or regression at the level of ambulation over time. DESIGN: Prospective study with follow-up of 4 years. SETTING: A children's hospital that serves a nationwide center for treatment and research in children with OI in the Netherlands. PARTICIPANTS: At follow-up, 49 children (24 boys, 25 girls; mean age +/- standard deviation, 11.3+/-3.8y; range, 5.2-19.4y) participated. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Anthropometry, joint ROM, muscle strength, fracture frequency, intramedullary rodding, level of ambulation, functional ability, and caregiver assistance. RESULTS: In type I OI, total joint ROM decreased significantly over time, especially in the lower extremities, with a significant decrease in generalized joint hypermobility according to Bulbena (median start, 7.5; interquartile range [IQR], 4-9; median end, 6; IQR, 2-7; P<.001). In types III and IV, a severe decrease in total joint ROM was present without significant changes over time. No significant changes in total muscle strength (upper or lower extremities) in the different types of OI were measured at follow-up. In OI type I, a significant increase in self-care (P=.003) and social function (P=.008) was measured; in type III, a significant increase in self-care (P=.003), mobility (P=.004), and social function (P=.005) was measured, with a significant decrease in parental assistance in self-care (P=.02) and mobility (P=.005). In type IV, a significant increase was observed in the self-care (P=.01) and social function domains (P=.02). Type of OI (regression coefficient=-1.0; 95% confidence interval [CI], -1.64 to -0.47) and total muscle strength were the only significant predictors for level of ambulation (regression coefficient=.01; 95% CI,.17-.32). Body weight was significantly lower in the group that progressed in level of ambulation (P=.03), whereas children with a decline in level of ambulation had significantly higher body weight (P=.05). CONCLUSIONS: Ours is the first study with a long-term follow-up that provides information concerning the natural course of developmental outcome parameters of OI in childhood. Joint ROM and muscle strength did not change significantly over time, possibly because of the biomechanical skeletal properties of the different OI types. Functional ability improved significantly over time, but, especially in types III and IV, did not reach normative values, possibly because of a plateau phase in functional ability. Knowledge of the natural course of the disease is essential to interpret the results from intervention studies.     

Microsurgery for glioblastoma preserves short-term quality of life both in functionally impaired and independent patients

OBJECTIVE: Considering the short remaining life expectancy with glioblastoma multiforme, numerous patients and their referring physicians are hesitant to use aggressive surgical treatment for fear of greater risk of neurological deterioration. The aim of our study was to determine whether surgical treatment of glioblastoma, utilizing modern microsurgical dissection techniques and tools, interferes with short-term quality of life. METHODS: The functional status before and after 67 microsurgical operations in 65 patients with glioblastomas was studied retrospectively. To evaluate the patients' quality of life, their functional neurological status was correlated with the Karnofsky performance scale (KPS). Post-operative evaluation was done within 7 days of surgery. RESULTS: No significant difference was found between the preoperative and the early postoperative functional status. Neither patient age nor the preoperative functional status determined a significant risk for postoperative functional deterioration. Patients with tumors involving so-called eloquent brain areas statistically worsened (p = 0.018); however, they showed only mild clinical impairment that was represented by a mean KPS status decline of less than -10. After total tumor resection, patients showed functional improvement. CONCLUSION: Microsurgery is safe and effective in order to improve or preserve short-term quality of life in glioblastoma patients. Total tumor resection is not associated with a significantly greater risk for neurological deterioration, either in patients with preoperative functional impairment, or in functionally independent patients.     

The Interaction Between Sillence Type and BMD in Osteogenesis Imperfecta

Clinical studies with bisphosphonates in children with osteogenesis imperfecta (OI) show an increase in BMD and a decrease in fracture rate. Bone strength in children with OI is not only influenced by changes in BMD but also by changes in collagen I structure of the organic bone matrix. Therefore, we studied the interaction between these two factors in a cross-sectional, single center study including 54 children. We assumed that vertebral deformities in OI represent an unbalance between load and bone strength. Body weight was considered to be a well quantifiable load on vertebral bodies. BMD served as a marker, representing the amount of bone tissue available for vertebral load bearing, and the Sillence classification, either type I or III/IV, as a marker representing the quality of the organic bone matrix. Independent associations were observed between the prevalence of vertebral deformities and (1) Sillence type (OR: 5.7, 95%Cl:1.2–26.8), (2) BMD (OR: 0.003, 95%Cl: 0–0.25) and (3) body weight (OR: 1.15, 95%Cl: 1.05–1.25). Regarding the anthropometrical differences among the different types of OI, the BMD/body weight ratio was introduced to evaluate the BMD in relation to body size. Prevalent vertebral deformities were associated with low BMD/body weight ratios (OR: 0.04, 95%Cl: 0.008–0.2) in OI type I, but no association was found in type III/IV. It was concluded that BMD and Sillence type have independent relationships with vertebral deformities. The BMD/body weight ratio correlates with vertebral deformities in children with OI type I. Its meaning in types III/IV needs further research with larger samples because of the relatively high prevalence of vertebral deformities in this group.     

Osteogenesis imperfecta in childhood: MR imaging of basilar impression

OBJECTIVE: To determine on radiographs the presence of Basilar Impression (BI) in children with Osteogenesis Imperfecta (OI). To confirm this sign and altered geometrical relationships of the craniocervical junction in course of time with magnetic resonance imaging (MRI). METHODS AND PATIENTS: In a cohort study of 130 patients with OI (OI type I: 85; OI type III: 21; OI type IV: 24) lateral radiographs of the skull and cervical spine were made in a standardised way. MRI scans were performed when BI was suspected based upon protrusion of the odontoid above Chamberlain's line. Intracranial abnormalities as well as the basal angle were described. Neurological examination was performed in patients with conclusive BI at MRI-scan. RESULTS AND DISCUSSION: In eight patients BI could be confirmed by MRI-scan. None of the children had or developed in time neurological symptoms or signs. Follow up of BI by MRI scans was done in seven patients (mean: 5 years; range: 2-6 years). No alteration of intracranial findings were seen at subsequent investigation, although in one child Chamberlain's line increased from 8 (first MRI) to 15 mm (last MRI). BI can be diagnosed by radiographs but in the extreme osteoporotic bone and altered anatomy of the craniocervical junction of children with OI MRI is preferable. As intracranial pathology can be demonstrated by MRI, also a relation can be laid to possible neurological symptoms and signs at clinical examination. CONCLUSION: In our cohort study no alteration of the intracranial contents was seen at subsequent MRI scans. Although anatomic deformations exist in BI, no neurological symptoms or signs were present in our study and no operative reconstruction had to be performed. Periodical MRI-scan has not been of influence on the clinical decision making process. At the moment we perform a MRI-scan if BI is suspected at lateral skull radiographs. The MRI images serve as reference findings to anticipate on possible future symptoms and signs of neurological deficit.     

Scoliosis in children with osteogenesis imperfecta: influence of severity of disease and age of reaching motor milestones

We studied the relationship between the age of reaching motor milestones, especially anti-gravity activities, and the age of development of pathological spinal curvatures in children with osteogenesis imperfecta (OI). We hypothesized that earlier achievement of anti-gravity motor milestones predicts a later development of pathological spinal curvatures. Ninety-six children participated in this retrospective study. The severity of the disease was classified according to Sillence into types I-IV. Spinal radiography was performed annually and spinal deformities were measured according to the Cobb angle. Scoliosis was defined as a Cobb angle exceeding 9°. Pathological thoracic kyphosis was defined as a Cobb angle exceeding 40°. The parents were asked to report the age at which the child achieved motor milestones, and data were checked against health care records. Thirty-seven of 96 children (39%) developed a scoliosis of more than 9°. Nine of 96 children (9%) developed a pathological kyphosis. The age of developing scoliosis was significantly lower than the age of development of the pathological kyphosis (P=0.01). Bone mineral density was measured by dual energy X-ray absorptiometry (DEXA) in 53 children, 28 of whom developed scoliosis, and 25 of whom did not. The mean DEXA Z-score of the 28 children with scoliosis was significantly lower than that of the 25 children without (-5.2, SD 1.3 vs -3.2, SD 1.9; P-value <0.001). Children with OI type IV, but particularly OI type III, reached motor milestones much later than children with OI type I. The motor milestone "supported sitting" showed a significant inverse association with time of the first presence of scoliosis with a Cobb angle greater than 9° (linear regression coefficient: -1.3, 95% confidence interval: -2.6 to -0.03). The age of achieving the motor milestones "lifting the head to 45° in prone position", "rolling", and "supported-" and "unsupported standing" were not significantly associated with age of the first presence of scoliosis with a Cobb angle greater than 9°. However, the directions of associations suggest that here, too, there is a tendency for later development of scoliosis in those who reach milestones at earlier ages. Multivariable analyses showed that the motor milestone "sitting with support" was significantly associated with age of first achieving scoliosis, independent of gender and type of OI (linear regression coefficient: -0.9, 95% confidence interval: -1.3 to -0.5). We conclude that in children with OI, the age of anti-gravity motor milestones was associated with the age of development of pathological spinal curvatures. Earlier achievement of the motor milestone "supported sitting" predicted significantly a later development of pathological spinal curvatures, independent of gender and type of OI.     

Pulmonary cystic hydatid disease in Ireland

Background Hydatid disease is rare in Ireland and its incidence and prevalence are unknown. Most cases are diagnosed by a combination of clinical findings, morphological features on imaging and by serological testing. Aims We describe an Irish case of pulmonary hydatid disease detected at bronchoscopy by bronchoalveolar lavage, and discuss the diagnosis and treatment of the disorder.     

No evidence for direct modulatory effects of delta 9-tetrahydrocannabinol on human polymorphonuclear leukocytes

The effects of cannabinoids (CB) that have been reported in various leukocyte populations were mainly immunosuppressive or immunomodulatory. Almost nothing is known, however, about direct interactions of cannabinoids with human polymorphonuclear cells (PMN), although m-RNA for the cannabinoid receptor-2 (CB(2)) was found in human PMN. In order to investigate a potential influence of cannabinoids on human PMN, the migration and phagocytosis of PMN were studied in the presence of Delta(9)-Tetrahydrocannabinol (Delta(9)-THC) at final concentrations between 10(-10) and 10(-5) M. No effect was detectable on these essential PMN functions; and besides, no CB(2)-receptor expression could be detected using the Western blotting technique. Thus, circulating human PMN from healthy individuals remain unaffected by Delta(9)-THC due to the absence of functional CB(2)-receptor expression.     

Absorption and endogenous faecal excretion of calcium by low birthweight infants on feeds with varying contents of calcium and phosphate

Low birthweight infants aged 4-41 days were given from birth one of three experimental milk formulae varying widely in content of calcium and phosphate. Ca and P in feed, urine, and faeces were measured between carmine markers corresponding to a metabolic period of 48 hours. Calcium enriched in 46Ca to provide a marker for the dietary Ca was added to one feed and 46Ca measured in urine and faeces. True absorption of Ca and endogenous excretion into the bowel could then be inferred. True absorption of Ca was proportional to Ca intake and independent of P intake. Endogenous faecal excretion seemed to be independent of both Ca P intakes, and varied widely between different infants in the range 4-150 mg/day. Urine Ca was low and retention was essentially the difference between true absorption and endogenous faecal excretion. Retention of Ca tended to be much greater on a high Ca intake, but the variability in retention between infants on a given intake was large, paralleling the variability in endogenous faecal excretion. The variability in plasma Ca concentrations in newborn infants may in part be a consequence of wide individual variability in endogenous faecal excretion. The 46Ca marker technique provides a means of investigating the factors determining this variability.     

Prolonged QT interval and cardiac arrhythmias in two neonates: sudden infant death syndrome in one case

Two neonates with arrhythmias and the long QT syndrome are described. The arrhythmias were detected in utero and both infants were apparently well after birth. The first infant, although well, had a bradycardia for the first 9 days of life. A normal heart rate was documented at 10 days but a prolonged QT interval was not appreciated on the ECG. He was discharged from hospital but died suddenly and unexpectedly 3 days later. A post-mortem examination failed to find a cause for his death which therefore fell into the category of the sudden infant death syndrome (SIDS). A retrospective analysis of the perinatal electrocardiogram showed a probable junctional rhythm with 2:1 conduction to the ventricle; the QT interval was prolonged at 0.52 seconds (QTC = 0.63). The second infant had a QT interval of 0.52 seconds (QTC = 0.54) and frequent ventricular premature beats on a 24-hour electrocardiogram. She was treated with propranolol and remains well 2 years later. Sudden infant death has often been described in the siblings of children with the long QT syndrome and one other report described a case of SIDS which was said to have had a prolonged QT interval on the perinatal ECG. This report, however, provides unquestionable evidence, in one case, of an association between the long QT syndrome and SIDS.