Brain magnetic resonance imaging in tyrosinemia

A 3.5-year-old girl with tyrosinemia is reported. A computed tomography scan of the abdomen revealed multiple hepatic nodules. Brain magnetic resonance imaging revealed bilateral high-signal changes confined to the globus pallidus on T2-weighted images. Globus pallidus lesions likely represented neuropathologic changes such as astocytosis, delayed myelination, and status spongiosus (myelin splitting and vacuolation).     

Hearing impairment associated with spinal anesthesia

OBJECTIVES: Hearing loss after spinal anesthesia has been reported to be related to the transmission of a reduced subarachnoid pressure to the inner ear via the cochlear aqueduct due to loss of spinal fluid. However, there are also some controversies related to this phenomenon, which require systematic investigation. MATERIAL AND METHODS: The effect of spinal needle diameter on hearing loss was investigated using audiometric tests in a prospective comparative study of 30 patients who were scheduled for surgery with spinal anesthesia. The bony structure of the cochlear and vestibular aqueducts was determined from temporal bone CT scans. RESULTS: Four out of 15 patients (26.67%) treated with a 22 G spinal needle demonstrated hearing loss the day after surgery, which recovered within 2-5 weeks. However, none of the patients treated with a 27 G spinal needle had statistically significant hearing loss in either ear at any frequency. Three out of four patients with hearing loss had an acute-onset balance problem. There was no difference between the two groups in terms of the widths of the vestibular and cochlear aqueducts. CONCLUSIONS: It has been shown audiometrically that the diameter of the spinal needle used to induce spinal anesthesia seems to have an effect on subsequent hearing loss. The patency of the bony canal determines the transmission of pressure changes to the inner ear. However, the individual risk of this complication is not predictable as there is no radiological abnormality of the canal.     

An infant with pentalogy of Cantrell and limb defects diagnosed prenatally

We present a case of pentalogy of Cantrell which was diagnosed prenatally on routine ultrasound examination. There were several associated limb defects. We discuss the differential diagnosis and conclude that our case probably had a variant form of this syndrome.     

Neurocognitive functions after aortic arch repair with right brachial artery perfusion

Background

Satisfactory neurologic outcome following aortic arch repair through right brachial artery perfusion is well established. However, how neurocognitive functions are affected following selective cerebral perfusion, still needs to be elucidated.

Methods

In a period between April 2002 and March 2003, 22 patients (19 male, 3 female, with a mean age of 46.8 ± 12; range: 26 to 70 years old), underwent aortic arch repair using right brachial artery low flow (8 to 10 mL · kg⁻¹ · min⁻¹) selective antegrade cerebral perfusion under moderate hypothermia (26°C). There were 6 Stanford type-A dissections and 16 ascending aortic aneurysms. All patients were evaluated preoperatively and postoperatively (at seventh day and second month) for neurocognitive functions.

Results

There was no operative mortality. The average cardiopulmonary bypass time was 115.0 ± 24.2 minutes and the average antegrade cerebral perfusion time was 29.8 ± 7.1 minutes (19 to 38 minutes). No major neurologic deficit was observed in the postoperative period. In terms of neurocognitive test results, between the preoperative and postoperative assessments for both hemispheric cognitive functions no deterioration was detected.

Conclusions

The low-flow selective antegrade cerebral perfusion technique through the right brachial artery may safely be used for the great majority of patients undergoing aortic arch repair without causing deteriorations in neurocognitive functions.     

Emery-Dreiffus muscular dystrophy: MR imaging and spectroscopy in the brain and skeletal muscle

Emery-Dreifuss muscular dystrophy is a rare disorder characterized by childhood onset of contractures, humeroperoneal muscle atrophy, and cardiac conduction abnormalities. This report presents the cases of two brothers with this dystrophy in whom bilateral hypomyelination of the deep periatrial white matter was noted. In the hypomyelinated regions, a prominent peak centered at 1.5 parts per million was present on short-TE MR spectra likely representing prominence of proteolipids in the macromolecular region. Major peaks (N-acetyl-aspartate, creatine, choline, and myoinositol) were normal. With respect to muscle changes, atrophy of the medial head of the gastrocnemius muscle was noted at MR imaging, and phosphorus spectroscopy of this muscle revealed decreased phosphocreatine and inorganic phosphate peaks.     

Subacute sclerosing panencephalitis with pontine involvement

In subacute sclerosing panencephalitis, brainstem involvement is rare. This paper reports an 11-year-old boy with the disease with prominent pontine involvement. Diffusion magnetic resonance imaging had superior information with respect to involvement of the nerve fibers. In the ventral part of the pons, the regions containing the longitudinal pontine nerve bundles were spared bilaterally and symmetrically. The pontine tegmentum was also spared. High signal in the affected regions was revealed by b = 1000 s/mm2 images. The apparent diffusion coefficient values in these regions were low, ranging between 0.43 x 10(-3) to 0.54 x 10(-3) mm2/s. Those of the spared tegmentum and longitudinal bundles were normal, ranging between 0.89 x 10(-3) to 1.04 x 10(-3) mm2/s, compared with the values from the normal-appearing regions of the cerebellar parenchyma.     

Regional versus general anesthesia for donor nephrectomy: effects on graft function

Various general and regional anesthesia methods are used successfully in living-donor kidney transplantation. This study compared kidney graft function after general versus combined spinal-epidural anesthesia for donor nephrectomy. The study groups included recipients who received grafts from donors who had undergone nephrectomy under general anesthesia (GA group; n=10), and recipients who received grafts from donors who had combined spinal-epidural anesthesia (CSE group, n=10). Standard continuous epidural anesthesia was administered during all transplantations. Graft function was assessed using scintigraphy and Doppler ultrasonography on days 3 and 7. Urine levels of microalbumin, creatinine, and creatinine clearance rate were measured/calculated in 24-hour urine samples collected on postoperative days 3 and 7. There were no differences on either day 3 or day 7 with respect to glomerular filtration rate, microalbuminuria, or creatinine clearance rate (P >.05 for all). There were also no differences between the groups with respect to other scintigraphic findings on day 3 or day 7 (P >.05 for all). Ultrasonography on day 7 showed significantly higher mean peak systolic flow in the main renal artery in the CSE group than in the GA group (P=.035). The results suggest that GA and CSE for donor nephrectomy have similar effects on kidney graft function in recipients.     

Hydatid Cyst in the Pulmonary Artery: An Uncommon Localization

Abstract Pulmonary artery involvement of hydatid disease caused by the Echinococcus granulosus parasite is an uncommon condition resulting from the opening of a visceral hydatid cyst into the venous circulation or the rupture of a cardiac hydatid cyst. We report a case of a 31-year-old woman with a hydatid cyst located in the right pulmonary artery. Clinical presentation was fatigue, cough, and dyspnea. Diagnosis was made by chest x-ray, computed tomography, and magnetic resonance imaging. The cyst was extracted under total circulatory arrest. Diagnosis and surgical therapy of the intraluminal pulmonary arterial hydatid cyst prevented possible occurrence of severe complications, such as cyst rupture, anaphylactic shock, and sudden death.     

Effect of intra-abdominal pressure level on gastric intramucosal pH during pneumoperitoneum

The present study was designed to examine the effect of intra-abdominal pressure level on gastric intramucosal pH using gastric tonometry during pneumoperitoneum. One hundred patients were prospectively randomized into 5 equal groups (N = 20 each). Intra-abdominal pressure levels were 8, 10, 12, 14, and 16 mm Hg in groups I, II, III, IV, and V, respectively. Intramucosal pH measurement was done 2 times: 30 minutes following insufflation and 1 hour after the ending of the surgery. In the first and second measurements, intramucosal pH values were found as 7.39 +/- 0.02 and 7.36 +/- 0.03 in group I; 7.41 +/- 0.03 and 7.38 +/- 0.03 in group II; 7.37 +/- 0.03 and 7.37 +/- 0.03 in group III; 7.36 +/- 0.03 and 7.37 +/- 0.03 in group IV; and 7.39 +/- 0.03, 7.36 +/- 0.03 in group V, respectively. Statistical significance was not found in the comparison of these values within the groups and between the groups (P > 0.005, for each). In conclusion, intra-abdominal pressure between 8 and 16 mm Hg did not cause significant difference in gastric intramucosal pH.