Cleidocranial dysplasia: a case report.

Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal disease. CCD is caused by mutation in the gene on 6p21 encoding transcription factor CBFA1, i.e. runt-related transcription factor 2(RUNX2). The disease is characterized by a persistently open anterior fontanelle and skull sutures, hypoplastic or aplastic clavicles, dental abnormalities, short stature, a wide pubic symphysis, and a variety of other skeletal changes. A major finding of CCD is hypoplasia or aplasia of clavicular bones resulting in the ability of the patient to approximate the shoulders. Delayed closure of the anterior fontanelle and of metopic sutures causes frontal bossing. We report a case of CCD in a 3.5-year-old boy who referred to our clinic because of an unclosed anterior fontanelle and emphasize the importance of clinical findings in CCD.

Low-dose MDCT and virtual bronchoscopy in pediatric patients with foreign body aspiration.

OBJECTIVE: The purpose of this study was to investigate the potential use of low-tube-current MDCT virtual bronchoscopy for the evaluation of children with suspected foreign body aspiration. SUBJECTS AND METHODS: Low-tube-current MDCT was performed in 23 patients (10 girls, 13 boys) with a mean age of 3.3 years (9 months-13 years) with suspicion of foreign body aspiration. Chest radiographs were obtained before CT was performed. MDCT was performed using 25- to 50-mA tube currents. MDCT virtual bronchoscopy images were obtained. Neither sedation nor IV contrast medium was used during CT scanning. All patients underwent endoscopic evaluation within 24 hr after MDCT was performed. MDCT virtual bronchoscopy findings were retrospectively compared with the results of rigid bronchoscopy. RESULTS: The mean tube current was 35 mA (range, 25-50 mA). Imaging quality was excellent in nine studies (39%), good in 12 studies (52%), and poor in two studies (9%). Motion artifacts were present on several slices in five examinations. In 15 patients, all foreign bodies detected by conventional bronchoscopy were also revealed on MDCT virtual bronchoscopy. The foreign body was in the right main bronchus in six patients, in the bronchus intermedius in one patient, and in the left main bronchus in eight patients. No discordance was found between the two techniques. MDCT revealed hyperaeration of the ipsilateral lung in five patients, atelectasis in five patients, infiltration in three patients, and infiltration and bronchiectasis in two patients; it showed infiltration in four patients and atelectasis in one of eight patients without a foreign body detected. There were no abnormal findings in three patients. CONCLUSION: Evaluation of foreign body aspiration of the airway in children can be accomplished by using a low-tube-current MDCT protocol. It may be useful both in showing the exact location of a foreign body before bronchoscopy and in ruling out a foreign body in patients with a low level of suspicion and normal or nonspecific findings on chest radiography.

MDCT cholangiography with volume rendering for the assessment of patients with biliary obstruction.

OBJECTIVE: We sought to evaluate the diagnostic utility of MDCT cholangiography with volume rendering in the evaluation of patients with suspected biliary tree obstruction. SUBJECTS AND METHODS: MDCT was performed in 34 patients who were thought to have biliary obstruction. Portal venous phase scanning was initiated 70 sec after the IV infusion of 150 mL of contrast agent, and no cholangiographic contrast agent was administered. Three-dimensional MDCT cholangiographic images were produced using volume rendering. ERCP was performed in 26 patients, and percutaneous transhepatic cholangiography (PTC) was performed in five patients; 17 patients underwent biopsy or surgery. The findings on MDCT cholangiography were compared with those of ERCP, PTC, biopsy, or surgery. RESULTS: The correct diagnosis was made on MDCT cholangiography for 14 (93%) of the 15 patients with a biliary stone and in 16 (94%) of the 17 patients with malignant biliary obstruction. Microlithiasis in one patient could not be detected on MDCT cholangiography. One patient with polypoid adenocarcinoma and one patient with normal findings were incorrectly diagnosed with a biliary stone on the basis of MDCT cholangiography. In one of the two patients with a benign stricture, the stricture was incorrectly diagnosed as malignant. For the diagnosis of biliary stone, sensitivity and specificity of MDCT cholangiography were 93% and 89%, respectively. For the diagnosis of malignant obstruction, sensitivity and specificity were both 94%. The accuracy of the technique for the diagnosis of the cause of biliary obstruction was 83.3%. CONCLUSION: MDCT cholangiography with volume rendering is a noninvasive and fast imaging technique with high sensitivity and specificity for the diagnosis of the cause of biliary tree obstruction. It is a promising diagnostic tool for the assessment of patients with bile duct obstructions.

[Case report: 3D CT angiography in a patient with patent ductus arteriosus associated with Eisenmenger's syndrome]. / Eisenmenger sendromu gelismis patent duktus arteriyozus olgusunda 3 boyutlu BT anjiyografi.

Presentation of patent ductus arteriosus with Eisenmenger's syndrome can mimic primary pulmonary hypertension. The sensitivity of transthoracic echocardiography for the diagnosis of patent ductus arteriosus with Eisenmenger's syndrome has been estimated as low as 12%. Transesophageal echocardiography is superior to transthoracic echocardiography for the diagnosis of patent ductus arteriosus but has some severe complications. 3D CT angiography can be used to distinguish patent ductus arteriosus with Eisenmenger's syndrome from primary pulmonary hypertension.

[Multi-slice CT angiography in the diagnosis and characterization of cerebral aneurysms]. / Serebral anevrizmalarin tani ve karakterizasyonunda çok-kesitli BT anjiyografi.

PURPOSE: To assess the diagnostic accuracy of multislice computed tomography angiography in the detection and characterization of cerebral aneurysms. MATERIALS AND METHODS: Multislice CT angiography and DSA were performed in 41 patients suspected to have intracranial aneurysms. A volume rendering method was used to produce three-dimensional CT angiograms. The diameter, location, neck and branching pattern of aneurysms were evaluated with CT angiography and DSA and compared with each other. RESULTS: Of 41 patients, DSA depicted 44 aneurysms in 33 patients. Multislice CT angiography depicted 44 aneurysms in 34 patients. One aneurysm demonstrated with DSA was not detected at multislice CT angiography. One aneurysm demonstrated with multislice CT angiography and confirmed at surgery was not demonstrated with DSA. There was no false positive aneurysm in multislice CT angiography. There were 4 aneurysms smaller than 3 mm and multislice CT angiography demonstrated all these aneurysms. In 9 aneurysms, the neck and branching pattern could only be shown with multislice CT angiography. Sensitivity and specificity of multislice CT angiography were 97.7% and 87.5%, respectively. CONCLUSION: Multislice CT angiography has a high sensitivity and specificity in the diagnosis of intracranial aneurysms and is superior to DSA in the delineation of the aneurysm neck and branching pattern. Multislice technology with submillimeter collimation improves the detection of small aneurysms and the delineation of the neck of the aneurysms.

Sclerosing haemangioma arising within extralobar pulmonary sequestration.

Extralobar pulmonary sequestration is a rare anomaly of abnormal pulmonary tissue without any communication to the bronchial tree. Sclerosing haemangioma is a rare lung tumour, generally seen in middle-aged women. The combination of these two rare pathologies has not been previously reported. We describe the CT and CT angiographic findings of sclerosing haemangioma arising within an extralobar pulmonary sequestration in a 2-year-old girl.

Multicentric Castleman's disease in a child with subpectoral involvement.

Castleman's disease is a benign lymphoproliferative disorder characterised by enlarged hyperplastic lymph nodes. It is rare in children and usually presents as localised disease. Subpectoral involvement has not been previously described in multicentric Castleman's disease in children. We present the CT, US and Doppler US findings of hyaline-vascular type multicentric Castleman's disease in a 5 year-old-boy with masses in the left subpectoral region and supraclavicular and axillary lymphadenopathy.

Effect of hypertension and candesartan on the blood flow velocity of the extraocular vessels in hypertensive patients.

OBJECTIVE: To define alterations in the blood flow velocities of the ophthalmic artery (OA), central retinal artery (CRA), posterior ciliary artery (PCA) in essential hypertension and to evaluate the effect of a new antihypertensive drug, candesartan which is an angiotensin II receptor antagonist, on the blood flow velocity in hypertensive patients. METHODS: Blood flow velocity and resistive index (RI) of the OA, CRA, and PCA were measured in 22 hypertensive patients off medication and 15 controls by color Doppler imaging. After treatment with candesartan, blood flow velocity and RI were again measured in the hypertensive patients. RESULTS: In controls (n=15), the OA had a mean peak systolic flow velocity (PSFV) of 48.1+/-2.6 cm/s, mean end diastolic flow velocity (EDFV) of 16+/-1.0 cm/s, and RI of 0.65+/-0.01; the CRA had a PSFV of 20.8+/-0.4 cm/s, EDFV of 9.4+/-0.3 cm/s, and RI of 0.54+/-0.01; the PCA had a PSFV of 23.6+/-0.7 cm/s, EDFV of 11.2+/-0.3 cm/s, and RI of 0.52+/-0.01. There was a significant decrease in the PSFV and EDFV of the vessels in the medication free hypertensive patients when compared with controls (P<0.05). In the hypertensive patients off medication (n=22), the OA had a PSFV of 29.4+/-1.2 cm/s, EDFV of 10.4+/-0.5 cm/s, and RI of 0.71+/-0.01; the CRA had a PSFV of 15.1+/-0.6 cm/s, EDFV of 5.4+/-0.3 cm/s, and RI of 0.65+/-0.02; the PCA had a PSFV of 17.2+/-0.6 cm/s, EDFV of 6.7+/-0.3 cm/s, and RI of 0.61+/-0.01. RI measured in the OA, CRA, PCA were significantly increased in the hypertensive patients when compared with the controls (P<0.05). In hypertensive patients after medication (n=22), OA had a PSFV of 38.3+/-2.5 cm/s, EDFV of 12.3+/-0.7 cm/s, and RI of 0.68+/-0.01; CRA had a PSFV of 19.2+/-0.5 cm/s, EDFV of 7.8+/-0.3 cm/s, and RI of 0.59+/-0.01; PCA had a PSFV of 20.8+/-0.8 cm/s, EDFV of 9.2+/-0.4 cm/s, and RI of 0.56+/-0.01. There was a significant increase in the blood flow velocities of the OA, CRA, PCA (P<0.05) and significant decrease in the RI values in the treated hypertensive patients when compared with the controls (P<0.05). But blood flow velocities and RI values did not reach the control level. CONCLUSION: The increase in the RI values and the decrease in the blood flow velocity of extraocular vessels in the hypertensive patients are thought to be caused by increased peripheral resistance in the vessels of the eye and orbit. Although, it increases blood flow velocity and decreases RI significantly, candesartan treatment in the hypertensive patients cannot increase blood flow velocity and decrease RI to the control level.

Image-guided percutaneous drainage of tuberculous iliopsoas and spondylodiskitic abscesses: midterm results.

PURPOSE: To evaluate midterm results of percutaneous drainage (PD) with image guidance in 21 patients with tuberculous iliopsoas abscesses with or without spondylodiskitis. MATERIALS AND METHODS: Computed tomography (CT)-guided PD was performed in 21 patients with 26 tuberculous iliopsoas abscesses. Nineteen patients had bone involvement of two or more vertebrae. Eleven patients with spondylodiskitis had intradiskal abscesses. Five patients had bilateral psoas abscesses. Easily and safely accessible well-circumscribed abscesses larger than 3 cm were selected for PD. Catheters were inserted into the abscess cavities with Seldinger technique in all cases. In conjunction with PD, all patients had antituberculous drug therapy and underwent clinical and imaging follow-up for at least 1 year. RESULTS: Percutaneous catheter placement was successful in all cases without procedural complications. On the basis of CT findings, complete evacuation of all abscesses was achieved initially. During follow-up, six (29%) of 21 patients had recurrences within 1 and 3 months after catheter removal. A total of 37 catheters were used; eight of the 37 catheters were inserted due to recurrences. Four patients needed two PD procedures, and two patients needed three due to recurrences. Four catheters were changed because of obstruction or dislocation. Drainage duration ranged from 5 to 36 days (mean, 14.9 days). The follow-up period was 12-52 months (mean, 24 months). None of the patients, including those with recurrence, required surgical drainage and débridement due to insufficient PD. CONCLUSION: Image-guided PD in conjunction with antituberculous drug therapy is an effective and safe procedure in the treatment of tuberculous iliopsoas abscesses with or without spondylodiskitis.