Limited Dorsal Myeloschisis and Congenital Dermal Sinus: Comparison of Clinical and MR Imaging Features.

BACKGROUND AND PURPOSE: While limited dorsal myeloschisis is a distinctive form of spinal dysraphism, it may be confused with congenital dermal sinus. The aim of this study was to describe clinical and MR imaging findings of limited dorsal myeloschisis that can distinguish it from congenital dermal sinus. MATERIALS AND METHODS: We retrospectively reviewed the clinical and MR imaging findings of 12 patients with limited dorsal myeloschisis and 10 patients with congenital dermal sinus. Skin abnormalities, neurologic deficits, and infectious complication were evaluated on the basis of clinical information. We evaluated the following MR imaging features: visibility of the tract along the intrathecal course, attachment site of the tract, level of the conus medullaris, shape of the spinal cord, and presence of intradural lesions such as dermoid/epidermoid tumors. RESULTS: A crater covered with pale epithelium was the most common skin lesion in limited dorsal myeloschisis (10/12, 83%). Infectious complications were common in congenital dermal sinus (6/10, 60%), whereas none were found in limited dorsal myeloschisis (P = .003). The following MR imaging findings were significantly different between the 2 groups (P < .05): 1) higher visibility of the intrathecal tract in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), 2) the tract attached to the cord in limited dorsal myeloschisis (12/12, 100%) versus various tract attachments in congenital dermal sinus, 3) dorsal tenting of the cord in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), and 4) the presence of dermoid/epidermoid tumors in congenital dermal sinus (6/10, 60%) versus none in limited dorsal myeloschisis. CONCLUSIONS: Limited dorsal myeloschisis has distinct MR imaging features: a visible intrathecal tract with dorsal tenting of the cord at the tract-cord union. Limited dorsal myeloschisis was not associated with infection and dermoid/epidermoid tumors.

Quantitative Assessment of Neovascularization after Indirect Bypass Surgery: Color-Coded Digital Subtraction Angiography in Pediatric Moyamoya Disease.

BACKGROUND AND PURPOSE: For the postoperative follow-up in pediatric patients with Moyamoya disease, it is essential to evaluate the degree of neovascularization status. Our aim was to quantitatively assess the neovascularization status after bypass surgery in pediatric Moyamoya disease by using color-coded digital subtraction angiography. MATERIALS AND METHODS: Time-attenuation intensity curves were generated at ROIs corresponding to surgical flap sites from color-coded DSA images of the common carotid artery, internal carotid artery, and external carotid artery angiograms obtained pre- and postoperatively in 32 children with Moyamoya disease. Time-to-peak and area under the curve values were obtained. Postoperative changes in adjusted time-to-peak (ΔTTP) and ratios of adjusted area under the curve changes (ΔAUC ratio) of common carotid artery, ICA, and external carotid artery angiograms were compared across clinical and angiographic outcome groups. To analyze diagnostic performance, we categorized clinical outcomes into favorable and unfavorable groups. RESULTS: The ΔTTP at the common carotid artery increased among clinical and angiographic outcomes, in that order, with significant differences (P = .003 and .005, respectively). The ΔAUC ratio at the common carotid artery and external carotid artery also increased, in that order, among clinical and angiographic outcomes with a significant difference (all, P = .000). The ΔAUC ratio of ICA showed no significant difference among clinical and angiographic outcomes (P = .418 and .424, respectively). The ΔTTP for the common carotid artery of >1.27 seconds and the ΔAUC ratio of >33.5% for the common carotid artery and 504% for the external carotid artery are revealed as optimal cutoff values between favorable and unfavorable groups. CONCLUSIONS: Postoperative changes in quantitative values obtained with color-coded DSA software showed a significant correlation with outcome scores and can be used as objective parameters for predicting the outcome in pediatric Moyamoya disease, with an additional cutoff value calculated through the receiver operating characteristic curve.

Postoperative pain and influencing factors among living liver donors.

PURPOSE: In Korea, living donor transplantation is increasing steadily as a life-saving alternative. It is essential to provide living donors the mental and physical care they need throughout their lives including postoperative period. Therefore, this study explored postoperative pain among living liver donors. METHODS: We used a convenience sampling at a university-affiliated hospital from March 1 to August 30, 2009 including 102 subjects. Face-to-face interviews with questionnaires and medical records were used to assess postoperative pain levels, state and trait anxiety as well as satisfaction. Data were analyzed using SPSS 14.0 (SPSS Inc., Chicago, Ill, USA). RESULTS: Average age of donors was 28.9±7.7 years (ranged 16 to 53) with 70.6% male. Most donors (80.4%, n=82) were immediate family members. Ninety-one (89.2%) participants made the decision by themselves. To control postoperative pain, all participants had patient-controlled anesthesia with several types of analgesics as prescribed by physician's preference. The mean values of state anxiety, trait anxiety, and satisfaction in this study were 2.1±1.89, 36.7±7.25 and, 8.9±1.79, respectively. Multivariate analysis showed that trait anxiety and number of analgesics use were significantly associated with postoperative pain. Overall, approximately 29.7% of total variability in postoperative pain could be explained by the nine variables in this model (R2=0.297, F9,102=4.28, (P<.001). There was no multicollinearity checked by tolerance, variation inflation factor, or condition index. CONCLUSION: This study of postoperative pain among living liver donors may contribute to developing the safest, most effective strategy to relieve postoperative pain after living liver donation.

Epidemiologic and phylogenetic characteristics of porcine reproductive and respiratory syndrome viruses in conventional swine farms of Jeju Island as a candidate region for PRRSV eradication.

Jeju island is the biggest island in Korea. The imports of pigs or their relatives from mainland Korea to this island has been banned since 1998. With this unique geographical and epidemiological context, epidemiology of porcine reproductive and respiratory syndrome virus (PRRSV) was investigated on the island. While all investigated farms showed 100% of seropositive rate for PRRSV, pigs on 37.2% (16/43) of the farms had viremia with type II PRRSV. The seropositive and viremia-positive rates for PRRSV in 30- to 60-day-old pigs were significantly higher in the western area ('swine farm complex' area) than the eastern area ('Scattered swine farm' area) of Jeju island. When 21 ORF5 sequences obtained from viremic sera were phylogenetically analysed, lineage 5 and Kor (newly termed in this study) of type II PRRSV were only found in Jeju island without changes from a previous report (2002-2003). Because other lineages of type II PRRSV (lineage 1 and 3) and type I PRRSV have recently emerged in mainland Korea, the banning of pigs' movement might be effective to protect the island from the introduction of these new PRRSV genotypes. Under this epidemiological condition (no introduction of new strains except for the modified-live vaccine (MLV) strain), the positive selection sites were analysed based on ORF5 of the virus. The amino acid 58 of GP5 (located on the hypervariable region) was predicted as a strong positive selection site. Although 51.2% (22/43) of the investigated farms had applied MLV, field strains of type II PRRSV were still circulating with strong positive selection. However, the restricted population of type II PRRSV (lineage 5 and Kor) without introduction of type I PRRSV or the other lineages of type II PRRSV indicate that the island has an effective quarantine system, which would allow PRRSV eradication.

Safety of ultra-rapid intravenous infusion of hepatitis B immunoglobulin in liver transplant recipients.

PURPOSE: To evaluate the safety of institutional protocol for ultra-rapid hepatitis B immunoglobulin (HBIG) infusion (10,000 IU in 30 minutes) for hepatitis B virus prophylaxis in adult liver transplant recipients. METHODS: In this case-controlled study, prospectively recruited liver transplant recipients received ultra-rapid infusions of HBIG (10,000 units in 30 minutes) for 6 months. The historical control group consisted of patients who had received 1-hour HBIG infusions (conventional rapid infusion) for the precedent 6 months. RESULTS: We found that 1472 patients had received 5744 ultra-rapid HBIG infusions, whereas 1343 patients had received 5200 conventional rapid HBIG infusions. Adverse side-effects were observed after 7 (0.13%) and 9 (0.16%) infusions, respectively (P = .763). The number of infusions per month increased significantly, from 878 ± 34 before the introduction of ultra-rapid infusion to 957 ± 29 afterwards (P < .001), an increase of 10.5%. The maximal capacity of HBIG infusions per day in the outpatient clinic increased from 53 for conventional rapid infusion to 65 for ultra-rapid infusion, without expansion of the outpatient facility or equipment. CONCLUSIONS: Nearly all adult liver recipients able to tolerate 1-hour infusions of HBIG can also tolerate ultra-rapid infusions well. Thus, it seems to be reasonable to perform ultra-rapid infusion protocol widely for patient convenience.

Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children.

AIM: To investigate the imaging and clinical findings of central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RTs) in children. MATERIALS AND METHODS: The computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical records of 16 children with CNS AT/RTs were retrospectively reviewed. Tumour location, size, composition, enhancement pattern, peritumoural oedema, signal intensity (SI) on MRI and CT attenuation were evaluated. RESULTS: A total of 17 lesions from 16 patients (median age 2.3 years, age range 0.7-15 years) were included in the evaluation. Tumour location was infratentorial for 11 lesions and supratentorial for six lesions. The mean diameter of the largest dimension for a tumour was 4 cm. The tumour was mainly solid in 65% of cases, and solid and cystic or cystic and solid in 35% of cases. The solid component of the tumours had a homogeneous iso SI (n=15) on T2-weighted MRI images and iso SI (n=14) on T1-weighted images. Moderate to strong enhancement of the solid component was noted in most cases. In spite of a large tumour size, peritumoural oedema was minimal or mild except in four cases. Rapid growth of the tumour was demonstrated in three cases. Seven patients died from tumour progression, with a mean survival time of 8.4 months (range 2-12 months). CONCLUSION: Although the AT/RTs had non-specific imaging findings, the tumours tended to be large in size, have iso SI on T1 and T2-weighted MR images with prominent enhancement, and relatively mild peritumoural oedema. Rapid growth of the tumour was seen during the follow-up period.

MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation.

BACKGROUND AND PURPOSE: Although brain MR imaging findings in adult Wilson disease have been described in considerable detail, a paucity of information currently exists regarding brain MR imaging findings in pediatric Wilson disease. The purpose of this study was to analyze the brain MR imaging findings in Wilson disease of childhood at the initial stage and during follow-up after treatment and to correlate these observations with clinical response. METHODS: We evaluated 50 patients with pediatric Wilson disease. Fifty initial and 20 follow-up MR images from 15 patients following penicillamine treatment were analyzed retrospectively, and the data were correlated with clinical findings. RESULTS: Patients were categorized into 3 groups on the basis of initial MR imaging findings. Group I (n = 23) showed normal MR imaging findings. Group II (n = 15) was characterized by T1-weighted images with increased signal intensity in the globus pallidus (n = 15, 100%) followed by the putamen, midbrain, and caudate nucleus. Group III (n = 12) demonstrated T2-weighted images with increased signal intensity in the putamen (n = 10, 83%), followed by the caudate nucleus, globus pallidus, thalamus, midbrain, and pons. There was a significant difference in mean age, the presence of neurologic symptoms, and Child-Pugh classification among the 3 groups (P < .001). Following copper chelating therapy, the changes on follow-up MR imaging were strongly correlated with clinical response to treatment (P < .001). CONCLUSION: Brain MR imaging in children with Wilson disease can be categorized into distinct groups and demonstrated a significant correlation with clinical findings. Interval changes on follow-up MR imaging were also closely correlated with clinical findings and helpful in assessing the clinical response.

MR findings of desmoplastic cerebral astrocytoma of infancy.

Desmoplastic cerebral astrocytoma of infancy (DCAI) presents as a large supratentorial mass consisting of a central cystic component and an enhancing solid component associated with peripheral dural attachment. We report the unusual MR findings of a DCAI that differed from previously reported cases in terms of the presence of calcification, which is not considered a feature of this tumor, and the absence of an enhancing peripheral dural component.

Comparison of contrast-enhanced ct angiography and gadolinium-enhanced MR angiography in the detection of subsegmental-sized pulmonary embolism. An experimental study in a pig model.

PURPOSE: To compare contrast-enhanced CT angiography (CTA) and gadolinium-enhanced MR angiography (MRA) for the detection of subsegmental-sized pulmonary emboli in a pig model. MATERIAL AND METHODS: In 5 anesthetized pigs, 3-mm diameter embolic materials made of Konjac, a semisolid food, were introduced through the internal jugular vein into pulmonary arteries. After embolization, CTA and MRA images were obtained. Respiration was suspended during CTA and MRA image acquisition. Two readers reviewed the CTA and MRA images to detect emboli. The pigs were sacrificed, and sliced specimens of inflated lung served as the gold standard. RESULTS: Thirty-six emboli were detected within peripheral arteries. The sensitivity (and 95% confidence intervals) of CTA for the two readers were 57% (39-74%) and 66% (48-81%), and 88% (69-98%) and 92% (74-94%) for MRA. The specificity of CTA was 95% (91-97%) and 98% (96-99%), and that of MRA was 85% (74-93%) and 90% (80-96%). Interobserver agreement was higher for MRA (kappa 0.898) than CTA (kappa 0.574). CONCLUSION: For the detection of subsegmental pulmonary emboli, MRA was superior to CTA, with a higher sensitivity and interobserver agreement by demonstrating perfusion deficits.

Teratoid Wilms tumor in childhood: CT and ultrasonographic appearances.

Teratoid Wilms tumor is defined as an unusual variant of nephroblastoma, in which there is a significant diversity of cell types and tissues in a neoplasm, where areas of classic nephroblastoma tissue are also present. We report a case of teratoid Wilms tumor demonstrated as a unilateral cystic and solid renal mass containing fatty tissue on ultrasonography and computed tomography.

Acute encephalitis associated with measles: MRI features.

We document the MRI features in six patients aged 5-14 years with acute encephalitis following measles. The diagnosis was made on a characteristic morbiliform rash and detection of specific IgM and IgG antibodies. The symptoms of encephalitis occurred 1-11 days after the appearance of the rash. All patients underwent MRI within 1-4 days of the onset of neurological symptoms. Diffusion weighted images (DWI) were obtained in three patients. In all patients, T2-weighted images showed widely distributed, multifocal high signal in both cerebral hemispheres with swelling of the cortex, with bilateral, symmetrical involvement of the putamen and caudate nucleus. The lesions had showed low apparent diffusion coefficients. Three patients showed subacute gyriform haemorrhage, and asymmetrical gyriform contrast enhancement on follow-up MRI.

The CNS midline cells control the spitz class and Egfr signaling genes to establish the proper cell fate of the Drosophila ventral neuroectoderm.

The spitz class genes, pointed (pnt), rhomboid frho), single-minded (sim), spitz (spi)and Star (S), as well as the Drosophila epidermal growth factor receptor (Egfr) signaling genes, argos (aos), Egfr, orthodenticle (otd) and vein (vn), are required for the proper establishment of ventral neuroectodermal cell fate. The roles of the CNS midline cells, spitz class and Egfr signaling genes in cell fate determination of the ventral neuroectoderm were determined by analyzing the spatial and temporal expression patterns of each individual gene in spitz class and Egfr signaling mutants. This analysis showed that the expression of all the spitz class and Egfrsignaling genes is affected by the sim gene, which indicates that sim acts upstream of all the spitz class and Egfr signaling genes. It was shown that overexpression of sim in midline cells fails to induce the ectodermal fate in the spi and Egfr mutants. On the other hand, overexpression of spi and Draf causes ectopic expression of the neuroectodermal markers in the sim mutant. Ectopic expression of sim in the en-positive cells induces the expression of downstream genes such as otd, pnt, rho, and vn, which clearly demonstrates that the sim gene activates the EGFR signaling pathway and that CNS midline cells, specified by sim, provide sufficient positional information for the establishment of ventral neuroectodermal fate. These results reveal that the CNS midline cells are one of the key regulators for the proper patterning of the ventral neuroectoderm by controlling EGFR activity through the regulation of the expression of spitz class genes and Egfr signaling genes.

Herpes simplex encephalitis: MRI findings in two cases confirmed by polymerase chain reaction assay.

Herpes simplex virus (HSV) type I causes a fulminant necrotising meningoencephalitis distinguished from other encephalitides by its focal and often haemorrhagic nature. Specific antiviral therapy with acyclovir can significantly improve the prognosis. We present MRI findings of two cases of herpes simplex encephalitis (HSE) confirmed by PCR analysis, focusing on the serial changes after acyclovir therapy: gyral swelling, high signal intensity on T2-weighted images in the subfrontal region, temporal lobe and insula in the initial stage, then regional extension with enhancement and haemorrhage despite appropriate acyclovir therapy, and finally encephalomalacia and brain atrophy.

Cerebellopontine angle ganglioglioma: MR findings.

We present a case of cerebellopontine (CP) angle ganglioglioma in a young child with developmental delay and no trigeminal nerve symptoms. MR imaging demonstrated a mass of homogeneous low signal intensity in the left CP angle on T1-weighted images with no enhancement with gadolinium, and of relatively homogeneous high signal intensity on T2-weighted images.

Gangliocytoma of the spinal cord: a case report.

We present a case of intramedullary spinal gangliocytoma in a 7-year-old girl who presented with scoliosis and progressive weakness of both legs. The tumour involved the whole spinal cord and medulla oblongata and was composed of inner cystic and outer solid components. On MRI, the solid portion of the lesion showed strong enhancement at the thoracolumbar level and mild enhancement at the cervical and medullary levels. Histological examination of the surgical specimen showed neoplastic ganglion cells arranged irregularly in benign normocellular glial background, which made a diagnosis of gangliocytoma.

Complex bronchopulmonary foregut malformation: extralobar pulmonary sequestration associated with a duplication cyst of mixed bronchogenic and oesophageal type.

We report a 13-year-old girl with an unusual, complex bronchopulmonary foregut malformation. The malformation included extralobar pulmonary sequestration and a duplication cyst of mixed bronchogenic and oesophageal type. Preoperative CT and MRI demonstrated the cystic and solid portions of the mass and indicated an aberrant vascular supply, suggesting the possibility of bronchopulmonary foregut malformation and several other differential diagnoses. A direct communication between the cyst and the bronchus of the sequestrated lung was found on pathological examination. This unusual combination of an extralobar pulmonary sequestration and a foregut cyst points to a common embryological pathogenesis.

Calcified subcutaneous fat necrosis induced by prolonged exposure to cold weather: a case report.

We present a 22-day-old infant with extensive subcutaneous calcifications due to subcutaneous fat necrosis caused by prolonged exposure to cold.

MR diagnosis of cerebellar infarction due to vertebral artery dissection in children.

Posterior circulation infarction is uncommon in children. We describe the clinical presentation and radiological findings in two children with cerebellar infarction resulting from dissection of the vertebral artery. We emphasize that vertebral artery injury should be considered in a child with acute symptoms and signs of ischaemia in the posterior circulation. MRI and MRA may be helpful in the diagnosis of cerebellar infarction and vertebral artery abnormality.

The CNS midline cells coordinate proper cell cycle progression and identity determination of the Drosophila ventral neuroectoderm.

The CNS midline cells, specified by the single-minded (sim) gene, are required for the proper patterning of the ventral CNS and epidermis, which are derived from the Drosophila ventral neuroectoderm. Defects in the sim mutant are characterized by the loss of the gene expression, which is required for the proper formation of the ventral neurons and epidermis, and by a decrease in the spacing of longitudinal and commissural axon tracks. Molecular and cellular mechanisms for these defects were analyzed to elucidate the precise role of the CNS midline cells in proper patterning of the ventral neuroectoderm during embryonic neurogenesis. These analyses showed that the ventral neuroectoderm in the sim mutant fails to carry out its proper formation and characteristic cell division cycle. This resulted in the loss of the dividing neuroectodermal cells that are located ventral to the CNS midline. The CNS midline cells are also required for the cell cycle-independent expression of the neural and epidermal markers. This indicates that the CNS midline cells are essential for the establishment and maintenance of the ventral epidermal and neuronal cell lineage by cell-cell interaction. On the other hand, the CNS midline cells do not cause extensive cell death in the ventral neuroectoderm. This study indicates that the CNS midline cells play important roles in the coordination of the proper cell cycle progression and the correct identity determination of the adjacent ventral neuroectoderm along the dorsoventral axis.

US identification of the anal sphincter complex and levator ani muscle in neonates: infracoccygeal approach.

PURPOSE: To identify the anal sphincter complex and levator ani muscle at transperineal ultrasonography (US) with the infracoccygeal approach. MATERIALS AND METHODS: Infracoccygeal US was performed with a 7-MHz linear-array transducer in 40 healthy neonates. The babies were placed in the supine position, and both legs were drawn up to the chest. The buttocks were accordingly lifted up. The approach site was just inferior to the coccyx and posterior to the anus. Scanning was performed to obtain transverse images of the anorectal area. The thickness of the anal sphincter complex and the puborectalis muscle were measured. RESULTS: Infracoccygeal US revealed the internal anal sphincter (IAS), the external anal sphincter (EAS), and the puborectalis muscle in all babies. The IAS and EAS were depicted as inner and outer hypoechoic ringlike structures, respectively. A round, hyperechoic space (intersphincteral space) was present between the hypoechoic IAS and EAS. The puborectalis muscle was identified as a U-shaped hypoechoic structure. The bulbocavernosus and ischiocavernous muscles were also identified. Mean thicknesses were as follows: IAS, 1.3 mm +/- 0.3 (SD) (range, 0.8-1.9 mm); EAS, 1.6 mm +/- 0.3 (range, 1.2-2.3 mm); and puborectalis muscle, 1.1 mm +/- 0.3 (range, 0.6-1.9 mm). CONCLUSION: Infracoccygeal transperineal US is an excellent diagnostic modality for demonstrating the anal sphincter complex and levator ani muscle in neonates.