Isolated ruptured dissecting aneurysm of PICA with extremely narrow neck managed by a novel endovascular technique-a case report and review of literature.

Dissecting aneurysms of posterior inferior cerebellar artery (PICA) are usually extensions of vertebral artery dissection. Isolated dissecting aneurysms of PICA are extremely rare. A 5-month-old female child presented with history of fall from a height of around 2 ft., followed by incessant crying and multiple episodes of vomiting. The anterior fontanelle was lax. Plain CT scan brain showed IVH in the fourth ventricle with no hydrocephalus. Twenty-four hours after admission, the child had one episode of vomiting and became unresponsive. The child was intubated, and a repeat CT scan brain showed thick acute SDH in the posterior fossa with hydrocephalus. Emergency suboccipital craniectomy and evacuation of thick acute subdural hematoma were done. Organized clot noted in the midline between the tonsils was not removed. Digital subtraction angiography (DSA) done 48 h after surgery showed ruptured dissecting aneurysm of left PICA. Endovascular temporary parent artery occlusion by coil deployment was performed, after which a check angiogram revealed complete exclusion of aneurysm from circulation. Endovascular proximal parent artery occlusion is the preferred treatment modality for dissecting aneurysms of PICA. Selective coil or Onyx embolization of the aneurysm with parent vessel preservation technique is a valid and increasingly used alternative. In this case, a novel technique of endovascular temporary parent artery occlusion by coil deployment was performed successfully. This technique may be recommended in select cases.

Surgical nuances in the separation of craniopagus twins - Our experience and a follow up of 15 years.

Craniopagus twins are conjoined twins fused at the cranium. This is the rarest anomaly seen in conjoined twins and craniopagus twins account for 2- 6% of conjoined twins. Conjoined twins are also extremely rare, with the anomaly seen in about 10-20 subjects per billion births. A female preponderance has been noted. Craniopagus twins can be classified into complete or partial, depending on whether or not they have shared dural venous sinuses. They can be further classified into angular or total depending on the alignment of the inter-twin longitudinal axis. Surgical separation of these cases can be an elective procedure or an emergency, mandated by the death of one of the twins. Surgical separation of craniopagus twins is a complex exercise needing detailed evaluation and planning. For the successful management of these twins, a multi-disciplinary approach involving neurosurgeons, plastic surgeons, anesthetists, radiologists, pediatric critical care specialists and ancillary staff is mandatory. We present a case of partially successful elective separation of partial angular craniopagus twins performed in 2002.The surviving twin was managed conservatively for a cerebrospinal fluid leak. The patient subsequently developed a pseudomeningocele, necessitating re-exploration, excision of the gliotic tissue, and repair of the dura and the overlying scalp flap. The patient has a skull defect for which cranioplasty has been deferred. The child is neurologically intact. The post-operative evaluation and the detailed periodic neurological assessment till date (with a follow up of 15 years) have been presented in this study.

Unenhanced renal magnetic resonance angiography in patients with chronic kidney disease & suspected renovascular hypertension: Can it affect patient management?

BACKGROUND & OBJECTIVES: Renal artery stenosis (RAS) is an important cause of severe hypertension in patients with chronic kidney disease (CKD). It is important to detect RAS early as it can reverse hypertension and stop rapid deterioration of renal function. The potential drawbacks of various imaging modalities used to detect RAS including contrast-related adverse effects associated with diagnostic angiography have led to increasing interest in unenhanced magnetic resonance (MR) renal angiography. The aim of this study was to detect and grade RAS in patients with CKD and suspected renovascular hypertension using unenhanced MR angiography (UMRA) and to identify patients with significant RAS (>70%) who would subsequently require further investigation and revascularization. METHODS: Thirty five CKD patients with suspected RAS were subjected to UMRA using non-contrast MR angiography of ArTery and VEins 3D True fast imaging with steady state precession technique over a three year period. Patients with RAS >70 per cent on UMRA were subjected to digital subtraction angiography (DSA) with intervention if indicated. RESULTS: In all, 76 renal arteries were evaluated using UMRA in 35 patients, of which 18 arteries showed stenosis and 11 were haemodynamically significant (eight patients). Seven patients (10 renal arteries) underwent DSA. INTERPRETATION & CONCLUSIONS: An association between UMRA and DSA findings was obtained in six patients (nine renal arteries), and these patients were stented. Post-procedure follow up showed good improvement in blood pressure and renal function. UMRA was found to be a useful non-invasive imaging modality to detect RAS in CKD patients. It can identify patients who require further invasive angiography and revascularization.

Subtelomeric rearrangements in Indian children with idiopathic intellectual disability/developmental delay: Frequency estimation & clinical correlation using fluorescence in situ hybridization (FISH).

BACKGROUND & OBJECTIVES: Subtelomeres are prone to deleterious rearrangements owing to their proximity to unique sequences on the one end and telomeric repetitive sequences, which increase their tendency to recombine, on the other end. These subtelomeric rearrangements resulting in segmental aneusomy are reported to contribute to the aetiology of idiopathic intellectual disability/developmental delay (ID/DD). We undertook this study to estimate the frequency of subtelomeric rearrangements in children with ID/DD. METHODS: One hundred and twenty seven children with idiopathic ID/DD were tested for subtelomeric rearrangements using karyotyping and FISH. Blood samples were cultured, harvested, fixed and GTG-banded using the standard protocols. RESULTS: Rearrangements involving the subtelomeres were observed in 7.8 per cent of the tested samples. Detection of rearrangements visible at the resolution of the karyotype constituted 2.3 per cent, while those rearrangements detected only with FISH constituted 5.5 per cent. Five deletions and five unbalanced translocations were detected. Analysis of parental samples wherever possible was informative regarding the inheritance of the rearrangement. INTERPRETATION & CONCLUSIONS: The frequency of subtelomeric rearrangements observed in this study was within the reported range of 0-35 per cent. All abnormal genotypes were clinically correlated. Further analysis with array technologies presents a future prospect. Our results suggest the need to test individuals with ID/DD for subtelomeric rearrangements using sensitive methods such as FISH.

Allergic bronchopulmonary aspergillosis presenting as complete lung collapse with respiratory failure.

Allergic bronchopulmonary aspergillosis (ABPA) is an allergic fungal disease that commonly complicates the natural course of patients with asthma and cystic fibrosis. Patients with ABPA commonly present with recurrent pulmonary infiltrates or bronchiectasis. They also experience difficulty treating asthma. Characteristic radiological findings include central bronchiectasis and high-attenuation mucus. Complete unilateral lung collapse is an uncommon presentation of ABPA, with few cases reported in published literature. We present a case of a man in his mid-40s, with acute cerebrovascular disease, who subsequently developed neurological deterioration, compounded by development of respiratory failure attributed to a complete left lung collapse, requiring invasive mechanical ventilation. Initially suspected to have aspiration pneumonia, he was eventually diagnosed with ABPA and was treated accordingly. This case illustrates an uncommon aetiology for complete lung collapse in this clinical setting and serves to remind us to consider ABPA as a differential diagnosis in such patients as well.

Diagnostic accuracy of 64-slice multidetector computed tomography in evaluation of post-coronary artery bypass grafts in correlation with invasive coronary angiography.

64-slice multidetector computed tomography (MDCT) allows more reliable and non-invasive evaluation of the coronary artery bypass grafts for occlusion or stenosis both in symptomatic and asymptomatic patients and also progression of disease in native coronary vessels.

Is Diagnosis of Low-Grade Urothelial Carcinoma Possible in Urine Cytology?

BACKGROUND: Urine cytology is a useful modality, primarily for the diagnosis and follow-up surveillance of high-grade urothelial carcinoma (HGUC). Its utility in diagnosing low-grade urothelial carcinoma (LGUC) remains controversial because of low reported sensitivity compared to cystoscopy. AIM: To study the cytomorphology of LGUC in voided urine samples and analyze its utility in diagnosis. MATERIALS AND METHODS: This is a retrospective study of one year, including 48 voided urine samples in cases which were confirmed as LGUC on subsequent histology. Urine cytology smears of these cases, originally stained with Papanicolaou stain were reviewed, critically analyzed and the specific cytomorphologic and cystoscopic findings were documented. RESULTS: On review 18 samples were re-categorized as LGUC which included 10 samples initially diagnosed as Negative for HGUC, 2 as Atypical Urothelial Cells - Not Otherwise Specified (AUC-NOS) and 6 as Suspicious for Carcinoma. In addition, another 3 samples with initial diagnosis of LGUC remained as LGUC on review. Thus, a total of 21 LGUC samples were identified after the review. 26 (54%) samples with a diagnosis of negative for HGUC remained negative even after review, as the tumor cells were not identified either due to sampling error or unrecognizable morphology. One (2%) samples of AUC-NOS remained the same on review due to very scant atypical cells. In 21 LGUC samples, cytology showed a dual population of benign differentiated urothelial cells and small urothelial cells with subtle nuclear atypia such as irregular and thickened nuclear membrane with increased nuclear cytoplasmic ratio. In 12 false negative LGUC samples, the diagnostic cells were camouflaged by their subtle nuclear atypia coupled with an overwhelming background of differentiated benign urothelial cells as both appeared almost similar in morphology. Papillary fragments were identified only in 2 samples. CONCLUSIONS: Diagnosis of LGUC on cytology is challenging and depends on the presence of diagnostic cells, pick up of diagnostic cells on screening and accurate interpretation. Special attention to papillary fragments and aforementioned nuclear atypia should be paid as tumor cells may resemble normal urothelial cells and can be easily missed.

Comparative analysis of physical doses and biomarker changes in subjects underwent Computed Tomography, Positron Emission Tomography-Computed Tomography, and interventional procedures.

Even though the medical uses of ionizing radiation are well-acknowledged globally as vital tools for the improvement of human health, they also symbolize the major man-made sources of radiation exposure to the population. Estimation of absorbed dose and biological changes after radiation-based imaging might help to better understand the effects of low dose radiation. Because of this, we measured the Entrance Surface Dose (ESD) at different anatomical locations using Lithium tetraborate doped with manganese (Li2B4O7: Mn), recorded Dose Length Product (DLP) and Dose Area Product (DAP), analyzed Chromosomal Aberration (CA), Micronucleus (MN), gamma-H2AX (γ-H2AX), and p53ser15 proteins in the blood lymphocytes of patients (n = 267) underwent Computed Tomography (CT), Positron Emission Tomography-CT (PET/CT), and interventional procedures and healthy volunteers (n = 19). The DLP and effective doses obtained from PET/CT procedures were significantly higher (p < 0.05) when compared to CT. Fluoroscopic time and DAP were significantly higher (p < 0.05) in therapeutic compared to diagnostic interventional procedures. All the anatomical locations registered a significant amount of ESD, the ESD obtained from CT and interventional procedures were significantly (p < 0.05) higher when compared to PET/CT. Fluoroscopic time did not correlate with the ESD (eye, head, thyroid, and shoulder; R2 = 0.03). CA frequency after PET/CT was significantly higher (p < 0.001) when compared to CT and interventional procedures. MN frequency was significantly higher in 24-hs (p < 0.001) post-interventional procedure compared to 2-hs. The mean ± SD of mean fluorescence intensity of γ-H2AX and p53ser15 obtained from all subjects underwent PET/CT and interventional procedures did not show a significant difference (p > 0.05) between pre- and post-procedure. However, the relative fluorescence intensity of γ-H2AX and p53ser15 was >1 in 58.5 % and 65.8 % of subjects respectively. Large inter-individual variation and lack of correlation between physical dose and biomarkers suggest the need for robust dosimetry with a large sample size to understand the health effects of low dose radiation.

Evaluation of atypical squamous cells on conventional cytology smears: An experience from a screening program practiced in limited resource settings.

BACKGROUND: The Bethesda system (TBS) 2001 has subdivided the category of atypical squamous cells (ASC) into: ASC-US (undetermined significance) and ASC-H (cannot exclude high-grade squamous intraepithelial lesion (HSIL)). The present study is an analysis of ASC-US and ASC-H cases diagnosed in a screening program practiced in limited resource settings. METHODS: During the period January 2005 to December 2008, a total of 9190 smears were received, of which 568 were unsatisfactory. Cases initially diagnosed as ASC-US (n=74) and ASC-H (n=29) on conventional cytology smears were reviewed. Biopsy and human papilloma virus (HPV) results were available in limited cases. RESULTS: On review, diagnosis of ASC-US was retained in 49 (66.2%) of the 74 initially diagnosed ASC-US cases. Remaining 12 cases were re-labeled as negative for intraepithelial lesion or malignancy (NILM), nine as low-grade squamous intraepithelial lesion (LSIL), three as ASC-H and one case as squamous carcinoma (SCC). Similarly, on review, diagnosis of ASC-H cases was retained in 17 of the 29 initially diagnosed ASC-H cases. Seven cases were re-labeled as NILM, three as HSIL and one case each as ASC-US and SCC. Overall, 8622 cases (96.6%) were diagnosed as NILM, 72 (0.83%) as LSIL, 121 (1.40%) as HSIL, 23 (0.26%) as SCC, 50 (0.57%) as ASC-US cases, 20 (0.23%) as ASC-H, five (0.05%) as atypical glandular cells (AGC) and two cases as adenocarcinomas. Out of 50 ASC-US cases, biopsy in 23 cases showed presence of CIN 1 in 16 cases (69.5%) and CIN 2 in one case (4.34%), while the remaining six cases were negative for CIN/malignancy. The remaining 20 cases with unavailable biopsy results were HPV-positive. Out of 20 ASC-H cases, biopsy in 15 revealed CIN 2 and above in 11 cases (73.3%). Three cases (20%) revealed CIN 1. CONCLUSIONS: Critical review is helpful in further reducing the number of ASC cases. The percentage of cases with CIN 2 and above is higher with ASC-H cases. The reason for relative increase in HSILs in the present study included referral bias in the screening program.

Tandem occlusion involving accessory middle cerebral artery in acute ischaemic stroke: management strategies.

Occlusion of both the limbs of accessory middle cerebral artery (AMCA) poses a unique challenge in management of acute ischaemic stroke by mechanical thrombectomy. The patient is a 30-year-old man, presenting with acute onset of left-sided hemiparesis for 4 hours. Non-contrast CT brain showed no bleed and three-dimensional CT angiogram of head and neck vessels showed dissection involving right carotid bulb and tandem occlusion of right MCA with presence of two MCA stumps. Aspiration thrombectomy was done using a reperfusion catheter, advanced separately into both the MCA stumps and complete recanalisation of both the limbs of MCAs was achieved, which was found to be AMCA. Balloon angioplasty for the internal carotid artery dissection was performed. Although occlusion of AMCA in acute ischaemic stroke is a chance occurrence, it is important to identify this variant as recanalisation of both the limbs by mechanical thrombectomy is required for favourable outcome after the procedure.

Exposure of patients to low doses of X-radiation during neuro-interventional imaging and procedures: Dose estimation and analysis of γ-H2AX foci and gene expression in blood lymphocytes.

Radiation has widespread applications in medicine. However, despite the benefits of medical radiation exposures, adverse long-term health effects are cause for concern. Protein and gene biomarkers are early indicators of cellular response after low-dose exposure. We examined DNA damage by quantifying γ-H2AX foci and expression of twelve candidate genes in the blood lymphocytes of patients exposed to low doses of X-radiation during neuro-interventional procedures. Entrance surface dose (ESD; 10.92-1062.55 mGy) was measured by thermoluminescence dosimetry (TLD). Absorbed dose was estimated using γ-H2AX focus frequency and gene expression, with in vitro dose-response curves generated for the same biomarkers. γ-H2AX foci in post-exposure samples were significantly higher than in pre-exposure samples. Among the genes analysed, FDXR, ATM, BCL2, MDM2, TNFSF9, and PCNA showed increased expression; CDKN1A, DDB2, SESN1, BAX, and TNFRSF10B showed unchanged or decreased expression. Absorbed dose, estimated based on γ-H2AX focus frequency and gene expression changes, did not show any correlation with measured ESD. Patients undergoing interventional procedures receive considerable radiation doses, resulting in DNA damage and altered gene expression. Medical procedures should be carried out using the lowest radiation doses possible without compromising treatment.

DNA damage and gene expression changes in patients exposed to low-dose X-radiation during neuro-interventional radiology procedures.

Interventional radiology-based imaging is the preferred choice for diagnosis and therapy of many complex diseases, despite possible adverse effects of the radiation exposures. We have measured induced DNA damage and changes in gene expression in relation to entrance surface dose (ESD) in peripheral blood samples of patients (n = 51) who underwent neuro-interventional radiological procedures. The ESD values, measured by thermoluminescence dosimetry, were 4.9-273 mGy (forehead), 14-398 mGy (eyes), 8-433.3 mGy (shoulders), and 4.7-242.5 mGy (thyroid). The in-built recorded Dose Area Product (DAP) values were 74.61-558.55 and 13.17-2825.12 Gy*cm2 for diagnostic and therapeutic procedures, respectively. The mean fluorescence intensity (MFI) on the phosphorylation of γ-H2AX and p53ser-15 was higher in samples obtained post-exposure vs. pre-exposure. However, the increase was statistically significant only for p53ser-15 (P < 0.01). Consistent with γ-H2AX, CDKN1A, FDXR, BAX, DDB2, SESN1, BCL2, MDM2, TNFSF10B, and PCNA showed (non-significant) decreased expression while GADD45A, ATM, and TNFSF9 showed (non-significant) increased expression. Our results suggest that most of the patients had increased DNA damage and altered gene expression after receiving relatively low doses of ionising radiation. This implies that these procedures should be carried out at the lowest possible doses of radiation that do not compromise image quality.

Entrance surface dose and induced DNA damage in blood lymphocytes of patients exposed to low-dose and low-dose-rate X-irradiation during diagnostic and therapeutic interventional radiology procedures.

The ionizing radiation received by patients and health workers due to radiological imaging may increase the risks of radiation effects, such as cancer and cataracts. We have investigated the dose received by specific areas around the head and related this to DNA damage in the blood lymphocytes of subjects exposed to interventional imaging. The entrance surface doses (ESD) to the forehead, neck, and shoulder were measured with a thermoluminescence dosimeter (CaSO4 disc or polycrystalline powder of lithium tetraborate doped with Mn) and compared with that of dose area product (DAP). DNA damage was measured by γ-H2AX, p53ser15, chromosomal aberration (CA), and micronucleus (MN) assays in lymphocytes of patients (n=75), before and 2 and 24h after exposure. The measured ESD values were 230.5±4.9, 189.5±3.55 and 90.7±3.4mGy for the forehead, neck, and shoulder, respectively. The DAP varied from 1.8 to 2047 Gy*cm2, showing a correlation with fluoroscopy time (r=0.417). Received doses did not increase early markers of DNA damage (γ-H2AX and p53ser15 assays), but residual damage (CA and MN frequencies) showed a significant (p<0.001) increase at 2 and 24h post-exposure compared to pre-imaging, despite poor correlation with DAP (r=0.1). Our results show that interventional imaging procedures deliver significant radiation doses and induce measurable DNA damage in lymphocytes of subjects, highlighting the need for rigorous patient safety protocols.

Multimodality approach in the diagnosis and management of bilateral giant juvenile breast fibroadenoma.

Juvenile giant fibroadenoma is a very rare breast disease affecting young girls of premenarche and adolescent ages. It is a benign fibroepithelial tumour characterised by stromal and epithelial proliferation that causes rapidly growing breast mass. Bilateral symmetrical involvement is extremely rare. In this article, we describe this entity in a girl aged 13 years who presented with bilateral gigantically enlarged breasts. Ultrasonography and MRI showed large, multilobulated masses involving both breasts entirely. Endovascular embolisation of bilateral internal mammary arteries and lateral thoracic arteries supplying the masses was performed prior to surgery to reduce their vascularity. The patient subsequently underwent excision of bilateral breast masses and reduction mammoplasty. Histopathologically, bilateral breast masses were confirmed to be juvenile fibroadenomas.

Magnetic resonance signal intensity measurements in the diagnosis of fetal central nervous system anomalies.

OBJECTIVES: To evaluate the possible role of Magnetic Resonance (MR) signal intensity measurements in diagnosing Central Nervous System (CNS) anomalies antenatally. METHODS: MR images of 110 fetal brains between 18 and 38 weeks were studied. Nine were excluded due to destroyed brain. 50 had CNS anomalies. 51 had normal CNS and were used as controls. Regions of interest (ROI) cursors were placed in Vitreous, cerebellar vermis, thalamus, frontal white matter, corona radiata, periventricular region and grey matter. The lateral ventricle diameters were also obtained. Signal intensity ratio (SIR) was calculated by the signal intensity of each of the above regions to that of the vitreous. SIR in controls were compared with fetuses having: (1) Hydrocephalus. (2) Arnold Chiari type-2 Malformation (ACM-2) (3) Non-progressive ventriculomegaly (4) Miscellaneous CNS anomalies. The correlation of the normalcy or abnormalcy of the brain was based on Clinical/Physical examination in 51, Ultrasound in 20, MRI in 2 and autopsy in 28. RESULTS: In hydrocephalus and ACM-2, the SIR of vermis and periventricular region were higher than controls whereas in non-progressive ventriculomegaly and miscellaneous CNS anomalies there was no significant difference. CONCLUSION: Signal intensity measurements are useful to differentiate physiological and non-progressive ventriculomegaly from hydrocephalus and ACM-2.

Endovascular management of vein of galen aneurysm malformation: A series of two case reports.

Vein of Galen Aneurysm malformation is one of the most difficult intracranial vascular lesions to manage. The difficulty in management of its shunts is aggravated by the hemodynamic effect and shunting reflected in the growing brain of infants and children. At present, the therapeutic options are widened by the opening of a new horizon in Intervention Neuroradiology. We present a series of two case reports of these malformations treated using the endovascular method, at our institution.