Radiology

We describe a new family with Adams-Oliver syndrome (AOS). The propositus is a 14-month-old boy presenting with aplasia cutis congenita, distal limb transverse defects, growth retardation, and a wide atrial septal defect. Central nervous system abnormalities included central hypotonia, and magnetic resonance imaging (MRI) findings consistent with periventricular leukomalacia (PVL). Fetal MRI at 26 weeks' gestation had shown bilateral dilatation of lateral ventricles and periventricular cysts at the site of postnatal lesions. The patient's father and paternal grandfather also had manifestations indicative of AOS. Antenatal and postnatal MRI findings suggest that our patient's PVL represents an unusual congenital feature of AOS, possibly due to vascular disruption and decreased perfusion during critical periods of fetal brain development.

Purpose: To assess the shortest time for catheter removal with regard to the transhepatic or transperitoneal approach in patients undergoing percutaneous cholecystostomy (PC).Methods: In this prospective study, 40 consecutive high-risk patients with acute cholecystitis (calculous, n = 22; acalculous, n = 18) underwent PC by means of a transhepatic (n = 20) or transperitoneal (n = 20) access route. In 28 patients (70%) computed tomography was used for puncture guidance, while in the remaining 12 (30%) the procedures were performed under ultrasound control. A fistulography was performed on the 14th postprocedural day in all patients and was repeated weekly if the tract was found to be immature. The catheter was removed only if a mature tract without evidence of leakage was delineated.Results: In 36 of 40 patients the procedure was technically successful (90%). Three of the unsuccessful punctures were attempted transperitoneally and one transhepatically. Thirty-five of 36 patients showed rapid improvement within the first 48 hr following the procedure (96%). Three of them died of their severe underlying disease (7.5%) and in another three the catheter was accidentally removed prior to the first fistulography (7.5%). A total of 30 patients could be fully evaluated after the procedure: 15 with a transhepatic, and 15 with a transperitoneal PC. Whereas 14 of 15 patients (93%) with transhepatic gallbladder access developed a mature tract after 14 days and the remaining patient after 3 weeks, only 2 of 15 patients (13%) with a transperitoneal route presented a mature tract after 2 weeks (p < 0.0001; chi2 test with Yates' correction). Eleven patients (73%) with transperitoneal access required 3 weeks and two patients (13%) 4 weeks for complete tract formation.Conclusion: A period of 2 weeks suffices for the majority of patients to develop a mature tract when the transhepatic access route is used; when using the trans- peritoneal route at least 3 weeks are required. We suggest that the transhepatic route is preferable since it allows earlier removal of the catheter and reduces the incidence of complications and discomfort for the patients.

Aim of this study is to illustrate the multidetector row computed tomographic findings related to oesophageal injuries and their significance for therapeutic decisions. From April 2002 to April 2005 we studied 16 patients with suspected oesophageal injury. Ten patients underwent standard chest radiograph, while five patients with suspected foreign body ingestion were submitted to cervical plain film and gastrografin swallow study. All 16 patients underwent multidetector row CT examination. In six patients with cervical, thoracic and abdominal trauma, CT showed the presence of thoracic traumatic lesions and findings suggestive of perforation of the oesophagus. In five patients with foreign body ingestion cervical radiography was positive in only one case, while CT showed the presence of a foreign body in all cases. In three patients with post-intubation complications, CT showed the presence of perioesophageal fluid collection containing small gas bubbles in two cases and an oesophageal-aortic fistula in one case. In the remaining two patients with suspected spontaneous oesophageal perforation, CT demonstrated an oesophageal wall oedema and thickening in one case, and oesophageal fluid distension with perioesophageal small bubbles gas and fluid in the second case. Oesophageal injuries, when complicated with perforation, constitute a life-threatening condition. Knowledge of the CT signs of oesophageal injuries has important implications for the role of imaging at the time of initial diagnosis.

Sports injuries may be unique in childhood and adolescence due to the inherent weakness of the growing skeleton at specific sites, mainly the cartilaginous parts. Many injuries are predictable based on the known mechanism of injury encountered in certain sports. There are two distinct patterns of injury in sports; acute, and chronic or overuse. Imaging plays an important role in the diagnosis and management of these entities. Radiologists should be familiar with the advantages and limitations of the various imaging modalities when evaluating the injured young athlete. The present review focuses on the radiological findings and appropriate imaging approach in injuries that are typically or most commonly encountered in the skeletally immature athletes.

The purpose of this study was to determine the gonadal dose, effective dose and relevant radiogenic risks associated with pediatric patients undergoing voiding cystourethrography (VCUG). Exposure parameters were monitored in 118 consecutive children undergoing VCUG. The entrance surface dose (ESD) was determined by thermoluminescent dosimeters (TLDs). For male patients, the gonadal dose was determined by TLDs attached on the anterior scrotum. For female patients, the gonadal dose was estimated by converting ESD to the ovarian dose. ESD-to-ovarian dose conversion factors were determined by thermoluminescence dosimetry and physical anthropomorphic phantoms representing newborn and 1-, 5- and 10-year-old individuals. The effective dose was estimated by using ESD and data obtained from the literature. The mean fluoroscopy time and number of radiographs during VCUG were 0.73 min and 2.3 for female and 0.91 min and 3.0 for male pediatric patients, respectively. The gonadal dose range was 0.34-5.17 mGy in boys and 0.36-2.57 mGy in girls. The corresponding ranges of effective dosage were 0.12-1.67 mSv and 0.15-1.45 mSv. Mean radiation risks for genetic anomalies and carcinogenesis following VCUG during childhood were estimated to be up to 15 per million and 125 per million, respectively. Radiation risks associated with pediatric patients undergoing VCUG should not be disregarded if such a procedure is to be justified adequately.

Dear Sir, Pyruvate dehydrogenase complex (PDHc) is an intramitochondrial multienzyme complex essential for the aerobic oxidation of glucose. PDHc deficiency may result in diverse manifestations of CNS dysfunction, including severe neonatal encephalopathy, ...

The purpose of our study was to examine variations in normal splenic size in relation to age, gender and body habitus in vivo, and to determine normative data for splenic volume on CT. The width (W), length (L), thickness (Th), cross-sectional areas and volume (Vol) of the spleen were obtained from abdominal CT examinations of 140 patients who underwent CT for indications unrelated to splenic disease. Splenic volume did not vary significantly (-0.04 < r < 0.05, p > 0.10) with the patient's age, gender, height, weight, body mass index or the diameter of the first lumbar vertebra, the latter considered as representative of body habitus on CT. The mean value of the measured splenic volume (S Vol) was 214.6 cm3 with a range from 107.2 to 314.5 cm3. S Vol correlated well with all the linear and the maximal cross-sectional area measurements and could be calculated using the formula: S Vol = 30 + 0.58 (W x L x Th.). Employing the same formula splenic volume was reliably assessed in 47 patients with clinically evident splenomegaly. Quantitative assessment of splenic volume might be of value in assessing mild variations in splenic size, because splenomegaly is the most common manifestation of splenic involvement in many disorders.

The optimal anticoagulation following Fontan operation and its modifications remain controversial and it is even less well defined as regards patients with inherited thrombophilia. We present a case of a child with bidirectional Glenn anastomosis for double inlet left ventricle that suffered a stroke despite aspirin prophylaxis; the patient was combined homozygous for prothrombin G20210A mutation and for methylenetetrahydrofolate reductase C677T mutation as well. The family history was positive for fetal loss and premature cardiovascular disease. Large-scale studies are needed to evaluate whether carriers of thrombophilia mutations need more intense thromboprophylaxis.

Introduction: Bacterial meningitis is a complex, rapidly progressive disease in which neurological injury is caused in part by the causative organism and in part by the host's own inflammatory responses. Case presentation: We present the case of a two-year-old Greek girl with pneumococcal meningitis and an atypical curvilinear-like skin eruption, chronologically associated with cerebral vasculitis. A diffusion-weighted MRI scan showed lesions with restricted diffusion, reflecting local areas of immunologically mediated necrotizing vasculitis. Conclusions: Atypical presentations of bacterial meningitis may occur, and they can be accompanied by serious unexpected complications.

The aim of the current study was to ͑a͒ provide normalized dose data for the estimation of the radiation dose from upper gastrointestinal tract contrast ͑UGIC͒ studies carried out to infants and ͑b͒ estimate the average patient dose and risks associated with radiation from UGIC examinations performed in our institution. Organ and effective doses, normalized to entrance skin dose ͑ESD͒ and dose area product ͑DAP͒ were estimated for UGIC procedures utilizing the Monte Carlo N-particle ͑MCNP͒ transport code and two mathematical phantoms, one corresponding to the size of a newborn and one to the size of a 1-year-old child. The validity of the MCNP results was verified by comparison with dose data obtained in physical anthropomorphic phantoms simulating a newborn and a 1-year-old infant using thermoluminescence dosimetry ͑TLD͒. Data were also collected from 25 consecutive UGIC examinations performed to infants. Study participants were ͑a͒ 12 infants aged from 0.5 to 5.9 months ͑group 1͒ and ͑b͒ 13 infants aged from 6 to 15 months ͑group 2͒. For each examination, ESD and dose to comforters were measured using TLD. Patient effective doses were estimated using normalized dose data obtained in the simulation study. The risk for fatal cancer induction was estimated using appropriate coefficients. The results consist of tabulated dose data normalized to ESD or DAP for the estimation of patient dose. Conversion coefficients were estimated for various tube potentials and beam filtration values. The mean total fluoroscopy time was 1.26 and 1.62 min for groups 1 and 2, respectively. The average effective dose was 1.6 mSv for group 1 and 1.9 mSv for group 2. The risk of cancer attributable to the radiation exposure associated with a typical UGIC study was found to be up to 3 per 10 000 infants undergoing an UGIC examination. The mean radiation dose absorbed by the hands of comforters was 47 Gy. In conclusion, estimation of radiation doses associated with UGIC studies performed to infants can be made using the normalized dose data provided in the current study. Radiation dose values associated with UGIC examinations carried out to infants are not low and should be minimized as much as possible.

Enterococcal urinary tract infection (UTI) is usually hospital-acquired and affects individuals with predisposing conditions. The aim of this study was to evaluate the community-acquired enterococcal UTIs in otherwise well children. We reviewed all the 257 first UTI episodes in children hospitalized in a General Hospital during a 5-year period. Enterococcus faecalis was isolated in 13 episodes, accounting for 5.1% of the total UTIs. All strains were susceptible to ampicillin, vancomycin and nitrofurantoin. Imaging studies revealed major urinary tract abnormalities in 9 and parenchymal defects in 8 children. During a follow-up period from 2 to 6 years, 4 children suffered break-through infections despite antibiotic prophylaxis, 3 developed renal scarring and 4 underwent corrective surgical procedures. Children with enterococcal UTIs presented with significantly higher rates of anatomical abnormalities and worse prognosis in terms of renal scarring, recurrences and corrective surgery compared with the total cohort of children with Gramnegative UTIs. However children with enterococcal UTIs did not present with a worse prognosis when compared with a group of children with Gram-negative UTIs matched for age and degree of reflux. Enterococcal infection is not an independent risk factor for poor outcome, nevertheless positive urine culture including enterococci is highly indicative for underlying urinary tract abnormalities, recurrences, renal scarring, and need for surgical intervention.

We read with great interest the Pictorial Interlude by P. R. Gutierrez, in the September 1998 issue of Pediatric Radiology [1], describing an unusual physeal injury through the sacro-coccygeal joint imaged with plain radiography and follow-up CT.