A rare combination: a single common pulmonary vein with polysplenia syndrome.

A single common pulmonary vein is a rare anomaly, mostly occurring with asplenia. We wanted to report our case because it co-exists with polysplenia and ciliary dyskinesia.A baby girl about 2 months old with respiratory arrest was admitted to the emergency room of our hospital. She responded to pulmonary resuscitation. We ascertained from her medical history that she had been hospitalised twice because of recurrent lung infections. After the initial check and treatment, we applied imaging modalities to detect underlying problems of recurrent lung infection. Echocardiographic evaluation was suboptimal because of the widespread pulmonary infiltration. Still, we noticed a single common pulmonary vein without obstruction at the junction of the left atrium. Subsequently, a chest CT scan with contrast was performed. It revealed a single common pulmonary vein entering the left atrium with a single orifice, atrial septal defect, two lobes in both lungs, hepatic veins draining directly into the right atrium, and polysplenia. When the flow of a single common pulmonary vein goes directly to the right atrium through the defect, it may cause right heart dilatation as it did in our case. Therefore, patients may need to undergo surgery at an earlier age.

Effectiveness of two-dimensional shear-wave sonoelastography in the diagnosis and follow-up of infantile hypertrophic pyloric stenosis.

INTRODUCTION: We sought to determine the effectiveness and utility of two-dimensional shear-wave sonoelastography (2D-SW-SE) in the diagnosis and postoperative follow-up of infantile hypertrophic pyloric stenosis (IHPS). MATERIALS AND METHODS: Twenty-three infants were included in the study, 13 in the IHPS group and 10 in the control group (CG). Preoperative B-mode ultrasonography measurements (longitudinal length and single-wall thickness of the pylorus) and 2D-SW-SE measurements (pylorus tissue stiffness and shear-wave propagation speed) were compared between the groups. The infants with IHPS then underwent Ramstedt pyloromyotomy and were invited for follow-ups on the tenth day and the first, third, and sixth months postoperatively. Measurements taken at the follow-ups were compared with the preoperative values. RESULTS: No differences were found between the groups regarding age, gender, body weight, or week of birth. The pyloric lengths in the IHPS group were longer than in the CG (p < 0.001), and the single-wall thicknesses were thicker (p < 0.001). The pylorus in the IHPS group was four times stiffer than in the CG (27.4 kPa versus 7.66 kPa), and the shear-wave propagation speed in the tissue was higher (1.34 m/s versus 2.69 m/s; p < 0.001). Both values decreased over time in the IHPS group and were normal by the third postoperative month. CONCLUSIONS: 2D-SW-SE can be used as an assistive imaging tool alongside B-mode ultrasound for diagnosing IHPS. It can also be used to identify inadequate surgery by detecting whether the pyloric tissue has softened at follow-up.

Atypical ß-Catenin Activated Child Hepatocellular Tumor.

Hepatocellular adenomas are a benign, focal, hepatic neoplasm that have been divided into four subtypes according to the genetic and pathological features. The ß-catenin activated subtype accounts for 10-15% of all hepatocellular adenomas and specific magnetic resonance imaging features have been defined for different hepatocellular adenomas subtypes. The current study aimed to report the magnetic resonance imaging features of a well differentiated hepatocellular carcinoma that developed on the basis of ß-catenin activated hepatocellular adenomas in a child. In this case, atypical diffuse steatosis was determined in the lesion. In the literature, diffuse steatosis, which is defined as a feature of the hepatocyte nuclear factor-1α-inactivated hepatocellular adenomas subtype, has not been previously reported in any ß-catenin activated hepatocellular adenomas case. Interlacing magnetic resonance imaging findings between subtypes show that there are still many mysteries about this topic and larger studies are warranted.

Sonoelastographic assessment of the age-related changes of the Achilles tendon.

AIMS: Tendons are crucial for optimal muscle force transfer and subject to changes with aging which may impair functional ability of elderly individuals. Achilles is the largest and the strongest tendon in the body; therefore it is an excellent site for the radiologic investigation of aging of tendons. Sonoelastography (SE) is a new ultrasound-based imaging technique that provides information on elastic properties and stiffness of tissues. The aim of our study was to investigate the age-related alterations in Achilles tendons using SE. MATERIAL AND METHODS: Forty five geriatric (age ≥ 65 years) and 42 young (age 18-40 years) healthy consecutive subjects were enrolled. Subjects with known history of metabolic or endocrine diseases, sports or traumatic injuries, peripheral vascular disorders were excluded. Both Achilles tendons were scanned with a real-time SE probe at a frequency of 6-15 MHz. Strains of Achilles tendons' proximal, middle and distal parts were assessed semi-quantitatively with comparing a reference tissue. RESULTS: Both SE methods -color coded evaluation and strain measurement- showed a remarkably stiffer tendon in the elderly subjects compared to young subjects in all thirds of Achilles tendons. In young subjects 84.9 % tendon thirds were blue, and 15.1% were green whereas, in elders 93.7% were blue and 6.3% were green (p=0.024). There was a significant correlation between age and stiffness of tendons assessed with strain indices. CONCLUSION: Our result showed increased tendon stiffness in elderly subjects which might be responsible for the high prevalence of Achilles tendinopathies observed in elderly subjects.

The prevalence of pineal cyst in patients with cerebral palsy.

PURPOSE: Pineal cysts are common incidental findings during magnetic resonance imaging (MRI) examinations. The etiology of pineal cyst development is still unclear. We aimed to determine whether there is an association between periventricular leukomalacia and pineal cyst prevalence. METHODS: Clinical and MRI data of 201 patients with periventricular leukomalacia (110 female, 91 male; mean age, 6 years; range, 2-18 years) and 687 control patients (355 female, 332 male; mean age, 6 years¸ range, 2-18 years) who did not have any evidence of periventricular leukomalacia were independently evaluated by two radiologists for presence or absence of pineal cyst. RESULTS: Pineal cysts were detected in 32.3% of the study group (65/201) and 8.4% of the control group (58/687) (P < 0.001). Patients with periventricular leukomalacia were more likely to have a pineal cyst. In terms of pineal cyst detection on MRI, interobserver reliability was high between the two radiologists. CONCLUSION: The prevalence of pineal cysts is higher in patients with periventricular leukomalacia. We suggest that an ischemic process may have a role in the etiopathogenesis of pineal cyst development.

Radiologic manifestation of a BCS1L-mutated patient.

There are various phenotypes of mutations in BCS1L which encode a mitochondrial inner membrane protein that facilitates the insertion of Rieske iron-sulfur protein into respiratory chain complex III. Herein we report the radiologic findings of a patient with bc1 synthesis-like (BCS1L) gene mutation who was presented with convulsions.