Double trouble - dual outflow tract obstruction in congenital heart disease: a case report.

BACKGROUND: Double chambered right ventricle is a rare congenital heart disease that is characterised by the presence of an anomalous muscle bundle that divides the right ventricle into a low pressure superior (distal) chamber and a high pressure inferior (proximal) chamber. It is found in association with a ventricular septal defect in 90% cases with other associations being tetralogy of Fallot, transposition of great vessels, atrial septal defect and Ebstein's anomaly. On the other hand, subaortic membrane is a form of discrete subaortic stenosis that is characterised by a membranous diaphragm in the subvalvular location of the left ventricular outflow tract. Both of these entities are responsible for causing subvalvular outflow tract obstruction. The occurrence of double chambered right ventricle in association with subaortic membrane is an extremely rare entity with only a few case reports available in the literature. CASE REPORT: A 13-year-old male child with history of chest pain and palpitations presented to the outpatient department of a tertiary care center. Transthoracic echocardiography revealed a subaortic membrane producing a pressure gradient across the left ventricular outflow tract with dilatation of the right atrium and right ventricle which could not be fully evaluated on echocardiography. Cardiac computed tomography was then performed which additionally revealed an anomalous muscle bundle coursing across the right ventricle from the septum to the subinfundibular region creating a double chambered right ventricle. The patient was then taken up for reconstruction of right ventricular outflow tract and resection of subaortic membrane. CONCLUSION: Right and left outflow tract obstructions are rare congenital lesions which when seen in combination, become even more infrequent. Echocardiography is a robust tool that detects turbulent flow to identify such lesions. However, poor acoustic window may sometimes result in missing these lesions and computed tomography in such situations can play an important role in detection as well as complete preoperative imaging evaluation.

A pentavalent approach for the evaluation of traumatic brachial plexopathy on MRI: correlation of macropattern and micropattern.

Macropattern analysis of traumatic brachial plexopathy (TBP) by Magnetic Resonance Imaging (MRI) encompasses localization of injured segments and determination of the severity of injury. The micropattern analysis implies the correlation of the MRI features of TBP with Sunderland's grading of the nerve injury, thereby guiding the management protocol. This review article presents a simplified novel pentavalent approach for the radiological anatomy of brachial plexus, MRI acquisition protocol for the evaluation of brachial plexus, cardinal imaging signs of TBP, and their correlation with Sunderland's microanatomical grading.

MRI evaluation of pelvis in Mayer-Rokitansky-Kuster-Hauser syndrome: interobserver agreement for surgically relevant structures.

OBJECTIVES: Diagnostic role of MRI in Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is well documented. Recent studies have shown the relevance of MRI in detailing the pelvic anatomy, particularly small Mullerian remnants. The results are, however, not consistent and reproducibility of MRI features has not been confirmed. The aim of our study was to evaluate the detailed pelvic anatomy in patients with MRKH syndrome and to calculate the interobserver agreement of the MRI features. METHODS: The study was carried out on female patients, aged 15-30 years, with primary amenorrhea. Following clinical, gynaecological, endocrinological, ultrasound and chromosomal work-up and a provisional diagnosis of MRKH syndrome, MRI pelvis was performed on 25 patients using a 1.5 T scanner. MRI images were retrospectively reviewed by two radiologists for a detailed evaluation of Mullerian structures (uterine buds, fibrous bands, midline triangular soft tissue), vagina, and ovaries. κ coefficient was calculated as a measure of interobserver agreement. RESULTS: Interobserver agreement was good to excellent (κ: 0.7788 ± 0.2168 to 1 ± 0) for uterine buds and their characteristics, vagina, ovaries and associated renal/vertebral anomalies; poor to fair for fibrous bands (κ: 0.2857 ± 0.3273 to 0.6032 ± 0.2149) and good for midline triangular soft tissue (κ: 0.7826 ± 0.1474). CONCLUSION: MRI is capable of providing key pre-operative anatomical information in MRKH syndrome non-invasively. There is good to excellent interobserver agreement for MRI features of most of the surgical relevant structures. ADVANCES IN KNOWLEDGE: MRI features of salient preoperative anatomical structures are reproducible between observers.

Imaging diagnosis of Crouzon syndrome in two cases confirmed on genetic studies - with a brief review.

Crouzon syndrome is the most common form of craniofacial dysostosis, characterised by a classical triad of abnormal skull shape, abnormal facies, and exophthalmos. The clinically overt dental abnormalities in these patients, distracts clinicians from the developmental neurological defects and therefore this entity remains relatively under - highlighted in radiology literature. We report and highlight the role of imaging in diagnosis of Crouzon syndrome in two patients, and discuss the relevant differential diagnosis. Moreover, our report is among the few Indian studies in which Crouzon syndrome was confirmed by genetic studies. The classical clinical triad of Crouzon syndrome was observed in both patients. The skull radiographs and cranial CT with 3D reconstruction VRT (Volume rendered technique), revealed characteristic radiological features. Genetic studies reconfirmed the clinical and radiological diagnosis of Crouzon syndrome, in both patients.

Clinicoradiological aspects of pontine tegmental cap dysplasia: Case report of a rare hindbrain malformation.

Malformations involving the brainstem are very rare and present with a varied spectrum of clinical symptoms due to multiple cranial nerve palsies and pyramidal tract involvement. Of these, pontine tegmental cap dysplasia is a very unusual malformation, characterized by ventral pons hypoplasia and an ectopic dorsal band of tissue, projecting into the fourth ventricle, from dorsal pontine tegmentum. A 4-year-old male child, presenting with left facial nerve palsy, revealed hypoplastic ventral pons and an ectopic structure on magnetic resonance imaging (MRI). The ectopic structure was isointense to pons, arose from the left side of dorsal pontine tegmentum, at pontomedullary junction and protruded into the fourth ventricle, impinging upon the left seventh and eighth cranial nerves. Diffusion tensor imaging (DTI) depicted abnormal white matter tracts in ectopic tissue with absent transverse pontine fibres and abnormal middle and superior cerebellar peduncles. The typical MRI appearance, coupled with DTI, helped us reach an accurate diagnosis of pontine tegmental cap dysplasia, in a setting of neurological dysfunction.

Clinicoradiologicial aspects of secretory carcinoma breast: A rare pediatric breast malignancy.

Secretory carcinoma (juvenile carcinoma) is one of the very rare breast malignancy reported to be prevalent in pediatric age group. We report imaging and clinicopathological features of secretory carcinoma breast with distant and axillary metastasis, in an 11-year-old girl, who presented with a painful lump in right breast. Ultrasound revealed a well-defined, partially microlobulated hypoechoic mass with skin and pectoralis muscle involvement and a suspicious morphology right axillary lymph node. Color Doppler revealed increased vascularity in both the breast mass and suspicious axillary node. Magnetic resonance imaging helped in better evaluation of pectoralis muscle involvement. Computed tomography (CT) neck, chest, and abdomen revealed multiple fibronodular opacities in bilateral lung fields. 18 Flouro-Deoxy-Glucose Positron Emission Tomography (FDG PET-CT) showed a hypermetabolic retroareolar breast mass with multiple hypermetabolic bilateral lung nodules suggesting lung metastasis. The histopathology confirmed the diagnosis of secretory carcinoma. The patient was offered chemotherapy for 2 years and put on follow-up since then.