Endoscopic Ventriculocysternostomy, Magendie Foraminoplasty, and Plexusectomy With Craniovertebral Shunt Placement in a Pediatric Patient With Hydrocephalus and VACTERL Association: A Novel Treatment Option.

Endoscopic third ventriculocysternostomy (ETV) is a minimally invasive neurosurgical technique with good results in the treatment of obstructive hydrocephalus. The VACTERL (vertebrae, anorectal, cardiovascular, tracheal, esophageal, renal, limb defects) association, or VATER syndrome, is defined as congenital malformations, mostly derived from the mesoderm, affecting specific areas. It is diagnosed by the presence of at least three of the seven characteristic malformations that describe it. The association of this pathology and obstructive hydrocephalus in pediatric age is not common, making management and conventional neurosurgical procedures difficult due to the number of underlying pathologies. In this study, we report the management of hydrocephalus and VACTERL association with multiple congenital malformations in a 30-day-old premature neonate (birth at 29 weeks). Operations performed prior to admission to our service included: coloesophagoplasty and placement of esophagostoma in the left anterior cervical region, perineal anorectoplasty, gastrostomy and placement of sigmoidostomy in the left anterior abdominal wall, relaparotomy, gastric suture, sanitation, and abdominal drainage. Upon admission, the patient showed a Grade 3 intraventricular hemorrhage and internal occlusive hydrocephalus due to circulatory blockage of the cerebrospinal fluid (CSF) at the level of the outlet of the fourth ventricle. This was accompanied by intracranial hypertension and refractory cervical syringomyelia. We performed endoscopic ventriculocysternostomy plus plexusectomy plus Magendie foraminoplasty with craniovertebral shunt placement, achieving excellent results after two interventions. This is the first case described in the literature placing a craniovertebral shunt using a lateral-ventricle-to-the-subarachnoid-spinal-space-stenting technique in a patient with VACTERL association, which represents an innovation in the field of minimally invasive pediatric neurosurgery.

Evaluation of the effectiveness of drug-eluting transarterial chemoebolization in hepatocellular carcinoma.

Background and Aim: Transarterial Chemoembolization (TACE) therapy is currently considered as first option therapy in the intermediate stage HCC. The purpose of our study is to assess the efficacy and prognostic factors related to the DEB- TACE therapy. Materials and Methods: The data from 133 patients with unresecetable HCC who were treated with DEB-TACE and followed between January 2011-March 2018 were retrospectively evaluated. To assess the efficacy of therapy, control imagings were performed at 30th and 90th days after the procedure. Response rates, survival outcomes, and prognostic factors were investigated. Results: According to the Barcelona staging system, 16 patients (13%) were in the early stage, 58 patients (48%) were in the intermediate stage and 48 patients (39%) were in the advanced stage. There were complete response (CR) in 20 patients (17%), partial response (PR) in 36 patients (32%), stable disease (SD) in 24 patients (21%) and progressed disease (PD) in 35 (30%) patients. Median follow-up time was 14 months (range 1-77 months). Median PFS and OS were 4 months and 11 months, respectively. In multivariate analysis, posttreatment AFP ≥400 ng/ml was found to be an independent prognostic factor on both PFS and OS. Child-Pugh classification and tumor size >7 cm were independent prognostic factors on OS. Conclusion: DEB-TACE is effective and a tolerable treatment method for unresectable HCC patients.

Kidney and Urinary Tract Involvement in Epidermolysis Bullosa: Is Routine Follow-Up Necessary?

BACKGROUND: Several renal and urinary tract complications have been reported in patients with epidermolysis bullosa. OBJECTIVE: This study investigated kidney and urinary tract involvement in patients with epidermolysis bullosa. PATIENTS AND METHODS: Patients with epidermolysis bullosa in treatment at the Dermatology Unit were included in the study. Glomerular and tubular functions were investigated. RESULTS: The study included 16 patients (4 females, 12 males) of mean 11.1 years (SD = 8.1 years). Estimated GFR was normal in all patients except one with end-stage renal disease. Excluding this patient, the urinary albumin/creatinine ratio and the fractional excretion of sodium were normal. The mean beta-2 microglobulin/creatinine ratio was 278.8 µg/g, and it was abnormally high in 2 patients. The mean tubular phosphorus reabsorption was 92.6%; it was abnormally low in 1 patient. Severe kidney or urinary tract involvement was present in 2 patients with recessive dystrophic EB-generalized severe (RDEB-GS): one patient had obstructive bullous lesions in the urethra; the other had end-stage renal disease secondary to focal segmental glomerulosclerosis and was on peritoneal dialysis for 3 years. CONCLUSIONS: Assessment for renal and urinary tract involvement should become a routine part of the evaluation of patients with any type of EB, but especially of patients with RDEB-GS. Patients with mild tubular dysfunction need long-term follow-up to detect early deterioration of renal function.

Neurodevelopmental Findings and Epilepsy in Malformations of Cortical Development.

AIM: The purpose of this study is to classify the malformations of cortical development in children according to the embryological formation, localization, and neurodevelopmental findings. Seizure/epilepsy and electrophysiological findings have also been compared. MATERIAL AND METHODS: Seventy-five children (age: 1 month-16.5 years; 56% male) followed with the diagnosis of malformation of cortical development, in Marmara University Pendik Research and Educational Hospital Department of Pediatric Neurology, were included in the study. Their epilepsy characteristics, electroencephalogram (EEG) findings, and prognosis were reported. Neurodevelopmental characteristics were evaluated by the Bayley Scales of Infant and Toddler Development (Bayley-III) for the ages of 0-42 months (n = 30); the Denver Developmental Screening Test-II (DDST-II) for ages 42 months-6 years (n = 11); and the Wechsler Intelligence Scales for Children (WISC-R), used for children 6 years and older (n = 34). RESULTS: The patients were classified as 44% premigrational (14.6% microcephaly, 24% tuberous sclerosis, 2.7% focal cortical dysplasia, 1.3% hemimegalencephaly, and 1.3% diffuse cortical dysgenesis); 17.3% migrational (14.6% lissencephaly, 2.7% heterotopia); and 38.6% postmigrational (14.6% schizencephaly, 24% polymicrogyria) developmentally. According to involved area, the classification was 34.7% hemispheric/multilobar, 33.3% diffuse, and 32% focal. Seventy-five percent of the patients had a history of epilepsy, and 92% were resistant to treatment. The seizures started before the age of 12 months in diffuse malformations, and epileptic encephalopathy was more common in microcephaly with a rate of 80% and lissencephaly with a rate of 54.5% in the first EEGs. Ninety-five percent of patients had at least one level of neurodevelopmental delay detected by DDST/Bayley-III; this was more common in patients with accompanying epilepsy (P < .05). As seen more commonly in patients with diffuse pathologies and intractable frequent seizures, mental retardation was detected by WISC-R in 64.5% of patients (P < .05). CONCLUSION: In cases with cortical developmental malformation, epilepsy/EEG features and neurodevelopmental prognosis can be predicted depending on the developmental process and type and extent of involvement. Patients should be followed up closely with EEG.

Predictive factors for treatment success of transforaminal epidural steroid injection in lumbar disc herniation-induced sciatica

Background/aim: The aim of this study was to identify predictive factors for treatment success in transforaminal epidural steroid injection in patients with lumbar disc herniation-induced sciatica. Materials and methods: A total of 219 patients who were diagnosed with unilateral sciatica and underwent transforaminal epidural steroid injections at the level of L4-5, L5-S1, or S1 neural foramina between March 2016 and May 2018 were retrospectively analyzed. The presence of transitional vertebrae and the grade of nerve root compression were evaluated by a radiologist. Data including age, sex, body mass index, duration of symptoms, injection levels, and pain scores were recorded. Pain scores were evaluated using the numerical rating scale. Treatment success was defined as a ≥50% decrease in pain scores at 3 months. Results: The study included 118 female and 101 male patients with a mean age of 43.65 ± 12.18 years. The mean duration of symptoms was 25.64 ± 2.17 weeks. Although the duration of symptoms was longer in patients for whom treatment failed, it did not reach statistical significance. Decreased pain scores at 1 h had a significant effect on treatment success (p = 0.012, odds ratio (OR): 1.015, 95% confidence interval (CI), 1.003­1.026). Conclusions: Our study results suggest that a decreased pain score at 1 h is a predictor for a favorable three-month response to transforaminal epidural steroid injection in patients with lumbar disc herniation-induced sciatica.

Venous vessel wall thickness in lower extremity is increased in male patients with Behcet's disease.

Vascular involvement, especially in young males, is seen in up to 40% of the patients with Behcet's disease (BD) and is a major cause of mortality and morbidity. In this study, we investigated vessel wall thickness (VWT) and dilatation in lower extremity veins with Doppler ultrasound (US) in male BD patients. Sixty-one male patients with BD, 37 healthy male controls (HC) and 27 male patients, with ankylosing spondylitis (AS), were included in the study. Venous Doppler US was performed by an experienced radiologist blinded to cases. Bilateral common femoral vein (CFV) wall thickness and great/small saphenous vein (SV) dilatations were assessed. All venous measurements were significantly higher in BD compared to AS and HC (p < 0.001 for all). Both right and left extremity CFV thicknesses had a high area under the ROC curve (> 0.8). Cut-off values for right and left CFV thicknesses for BD was 0.49 and 0.48 mm, respectively. High sensitivity and specificities are observed for both measurements (right CFV: sensitivity 81%, specificity 78.4%; left CFV: sensitivity 82.8%, specificity 81.1%). We found increased CFV thickness in BD patients independent of vascular involvement. As a similar change was not observed in controls, increased CFV thickness may be a specific sign of venous inflammation in BD. Our acceptable sensitivity and specificity values of CFV measurements suggest that assessment of femoral vein thickness with US may be a candidate diagnostic tool, especially in young males suspected of BD.

The effectiveness of ultrasonography and ultrasonographic elastography in the diagnosis of carpal tunnel syndrome and evaluation of treatment response after steroid injection.

PURPOSE: To evaluate if there is a place for ultrasonography (US) and ultrasonographic elastography (UE) in the diagnosis and follow up of carpal tunnel syndrome treatment. MATERIALS AND METHODS: The study was performed on 25 patients (study group) and 17 healthy volunteers (control group). Measured US and UE criteria were median nerve area (MNA), proximal median nerve area (pMNA), difference between MNA and pMNA (dMNA) and strain values of carpal tunnel content (CTC) and median nerve (MN). Patients in the study group were also evaluated using the Boston questionnaire, Visual Analogue Scale (VAS) and nerve conduction studies. Thirty-three wrists in 23 patients received steroid injections on the same day. Patients were re-evaluated 6 weeks after steroid injection. To establish a cut off value for MNA, ROC Curve analysis was used. RESULTS: Mean MNA and dMNA values in the control group were significantly lower than in the study group (7.33 ± 1.31 vs. 15.44 ± 5.10; p=<0.05 and 1.58 ± 0.75 vs. 8.91 ± 4.93; p = 0.00 respectively). Median nerve and CTC strain indices were significantly higher in the control group (p = 0.00 and p = 0.036). Decrease in MNA and dMNA values after treatment was meaningful (p = 0.00). While there was no significant change in MN elasticity, mean CTC strain index of the study group (4.680 ± 1.664) decreased significantly after treatment (3.621 ± 1.054 p = 0.002). This decrease in the CTC index was more pronounced in patients who benefited from treatment (p = 0.001). CONCLUSION: US and UE can be useful in the diagnosis of CTS and its response to treatment, if used together with clinical and electroneurophysiological tests.

Initial inferior vena cava and aorta diameter parameters measured by ultrasonography or computed tomography does not correlate with vital signs, hemorrhage or shock markers in trauma patients.

BACKGROUND: Ultrasonography (US) is noninvasive, readily available, and cheap. The diameter of inferior vena cava (dIVC) and its respiratory variation were proposed as a good surrogate of the hemodynamic state. However, recent studies have shown conflicting results, and the value of IVC-derived parameters in the estimation of fluid status and hemorrhage remains unclear. METHODS: This was an observational study of trauma patients who presented to emergency department. dIVC and aorta diameter (dAorta) were measured at the initial US and CT in all patients. The correlation of these measurements and all parameters derived from those measurements along with the initial vital signs and laboratory values of hemorrhage (hemoglobin, hematocrit) and shock (lactate, base excess) were assessed. US and CT values were also compared for accuracy using Bland-Altman analysis. RESULTS: The final study population was 140, with a mean age of 38 years and 79.3% were male. dIVC and dAorta did not have any clinically significant correlation with any of the vital signs or laboratory values of hemorrhage or shock when measured by US or CT. A good and significant correlation was observed between dIVC and dAorta measured by US and CT. CONCLUSION: The value of an initial and single measurement of IVC and aorta parameters in the evaluation of trauma patients should be questioned. However, the change in the measured parameters may be of value and should be investigated in further studies.

Computed tomography findings of primary epiploic appendagitis as an easily misdiagnosed entity: Case series and review of literature.

BACKGROUND: Primer epiploic appendagitis (PEA) is an uncommon condition. METHODS: We retrospectively reviewed the clinical records and computed tomography (CT) findings of 45 patients with PEA. RESULTS: On the basis of physical examination and pain localization, presumptive clinical diagnosis was acute appendicitis (n=13), acute cholecystitis (n=2), acute diverticulitis (n=19), renal colic (n=7) and ovarian pathology (n=4). CONCLUSION: Although it has no characteristic clinical and laboratory features, CT is the best modality for accurate diagnosis of PEA.

Crouzon syndrome: Virtual planning of surgical treatment by application of internal distractors.

Crouzon syndrome is one of the frequent pathologies within craniosynostosis syndrome. Current progress in computers and biotechnologies allows improving surgical approach and forecasting final result of reconstruction as well. We present a case of successful surgical treatment of Crouzon syndrome, done by application of virtual planning allowing determining "monobloc" features, type of reconstruction and distraction protocol as well. A 20-year-old female had presented with craniofacial deformity. Clinical and radiological investigation revealed Crouzon syndrome. The "monobloc" creation, cranioplasty and internal distractors positioning, direction and schedule of advancement were done according to preoperative virtual planning data achieved by Materialise Mimics Research software. Nine months postoperative functional and esthetic result and radiological findings showed to be reasonable. That application of virtual simulation significantly allows to determine best direction of distraction and improves postoperative outcomes of surgical treatment of Crouzon syndrome.

Multiparametric breast MRI with 3T: Effectivity of combination of contrast enhanced MRI, DWI and 1H single voxel spectroscopy in differentiation of Breast tumors.

OBJECTIVES: To evaluate the diagnostic accuracy of dynamic contrast enhanced breast MRI (DCE-MRI) combined with diffusion weighted imaging (DWI) and 1H single-voxel magnetic resonance spectroscopy (1HMRS) in differentiating malignant from benign breast lesions. METHODS: One hundred twenty-nine patients with 138 lesions were included in the study. Multiparametric MRI of the breast was performed with a 3T unit. A DWI is followed by DCE-MRI and 1HMRS. All lesions were biopsied within one week after MRI. Histopathologic findings were accepted as the standard of reference. Probability of malignancy was assessed according to BI-RADS for DCE-MRI. ADC values were measured for DWI and choline peaks were assessed using a semi-quantitative method in 1HMRS. Two blinded radiologists evaluated findings in consensus. Diagnostic performance of DCE-MRI, DWI and 1HMRS alone or in combination for multiparametric imaging were statistically evaluated. RESULTS: Histopathology revealed malignancy in 54.4% of lesions (75/138). DCE-MRI showed the highest AUC (0.978), sensitivity (97.33%) and specificity (88.89%) compared to DWI and 1HMRS. Sensitivity was 100% when a positive result from any one of three techniques was accepted as malignancy, albeit with a trade-off for 65.1% specificity. Highest specificity (98.4%) was attained when all three techniques were required to be positive, though with a lower sensitivity (82.7%) as trade-off. Logistic regression analysis confirmed significant association with DCE-MRI (p<0.001) and 1H MRS (p=0.009) but not with DWI (p=0.127). There was one case of fat necrosis which was false positive in all three techniques. CONCLUSIONS: Multiparametric imaging with combination of DCE-MRI, DWI and 1HMRS does not improve, and may even reduce the diagnostic accuracy of breast MRI. Although, the specificity may be improved with a trade-off for lower sensitivity, we have not set a convenient algorithm for the combined use of these techniques.