Budd-Chiari syndrome in children: Radiological intervention and role of shear wave elastography in monitoring response.

OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI. Medical therapy without anticoagulation and monitoring was done in subjects without RI. The RI and no-RI groups were compared. RESULTS: Ascites (54,90%), hepatomegaly (56,93%) and prominent abdominal-veins (42,70%), were the commonest features. The majority (46,78%) had isolated hepatic vein block. 44 (73%) cases underwent RI, while 16 (27%) were managed conservatively. Both groups were similar at baseline. Post-RI subjects showed significant improvement in clinical findings, liver functions and portal hypertension. LSM [33 (32-34.5) to 19.2 (18-20.67) kPa] and SSM [54.5 (52.3-57.6) to 28.9 (27.6-30.25) kPa] showed a significant decline from baseline value over a follow-up of 12 (6-13) months. Gradual reduction occurred in the LSM and SSM over 1-5 years, with near-normal LSM [10.2 (9.2-11.5) kPa] and SSM [22.3 (20.5-24.3) kPa] values in patients (n-16) with > 5 years follow-up. Patients without RI showed worsening in LSM and SSM. Hepatopulmonary syndrome and hepatocellular carcinoma developed in 4 (8%) and 1 (1.7%) cases respectively. CONCLUSION: RI leads to clinical recovery and reduction with near normalization of LSM and SSM over long-term follow-up in children with BCS. 2D-SWE is a promising tool to monitor outcomes.

Autoimmune Sclerosing Cholangitis in Children: A Prospective Case-Control Study.

PURPOSE: In children overlap of autoimmune hepatitis (AIH) and primary sclerosing cholangitis is labelled as autoimmune sclerosing cholangitis (ASC). The only prospective pediatric study showed a high prevalence of ASC by using endoscopic retrograde cholangiopancreatography. Aims of our study were to find the prevalence of ASC by using magnetic resonance cholangiography (MRC) in AIH and in non-AIH cirrhosis and to compare clinical presentation and outcome of AIH and ASC. METHODS: Prospectively we did MRC in 38 children with AIH (cases) and 19 disease controls (Wilson disease). Multiple biliary strictures with proximal dilatation on MRC were taken as definitive changes of ASC. Detail clinical, laboratory parameters, liver histopathology and treatment outcome were recorded. RESULTS: The median age of cases was 11.5 (3-18) years, 22 (57.9%) were girls and 28 (73.7%) were diagnosed as type 1 AIH. MRC was done in 11 children (28.9%) at the time of diagnosis and in 27 (71.1%) after a median follow-up of 2.5 (0.3-10) years. Abnormal MRC changes were seen in 14/38 (36.8%) of AIH and 8/19 (42.1%) of controls. However, definite changes of ASC were present in four (10.5%) children in AIH and none in controls. None of the clinical, laboratory, histological parameters and treatment response were significantly different between ASC and AIH groups. CONCLUSION: The prevalence of ASC in children with AIH was just 10.5%. We suggest MRC in select group with cholestatic features, inflammatory bowel disease and in those who showed poor response to immunosuppression instead of all children with AIH.

Management of macrocystic lymphatic malformation at uncommon site with aqueous bleomycin sclerotherapy.

INTRODUCTION: Lymphangioma are rare vascular malformation that results from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. Atypical site of cystic hygroma in pediatric age group are usually difficult to diagnose clinically but can be diagnosed easily by ultrasound. The aim of the study was to evaluate the result of the intralesional bleomycin for macrocystic lymphatic malformation (LM) presenting at atypical site. MATERIAL AND METHOD: All patients of LM of other than head& neck, axilla and abdomen presenting in pediatric age group were included in the study. Mainstay of diagnosis was ultrasound and was supplemented by CT scan wherever required. All patients were managed with intralesional bleomycin (ILB) and surgical excision was done only if primary therapy failed. RESULT: Total 15 cases of LM presenting at atypical sites were included in the study. Series include two case of cystic hygroma of breast, 4 cases of cystic hygroma of anterior chest wall, two case of substernal LM, three cases of LM of parotid gland, one case of inguinal region cystic hygroma and 4 cases involving submandicular area. Complete resolution was observed in 13 out of 15 cases, and two cases had less than 50% reduction in size and were managed with surgical excision after second session of ILB. CONCLUSION: Aqueous Intralesional bleomycin is a cost effective alternative to surgery even at rare sites of LM which provide better aesthetic outcome, and avoids complication associated with surgery.

Non-Surgical Management of Bilateral Adrenal Abscess in Neonates: Report of Two Cases.

Adrenal hemorrhage is not uncommon in neonates but the development of an adrenal abscess is extremely rare. Bilateral adrenal abscess is even rarer with less than ten cases documented in the medical literature. It may have a fatal outcome if inadequately treated. Here, we present two cases of neonates with history of obstructed labour and meconium aspiration who presented with bilateral adrenal abscesses. The abscesses were successfully treated using ultrasound-guided percutaneous drainage along with administration of appropriate intravenous antibiotics.

Oncogenic osteomalacia: role of Ga-68 DOTANOC PET/CT scan in identifying the culprit lesion and its management.

OBJECTIVE: The aim of this study was to evaluate the role of 68Ga-DOTANOC positron emission tomography (PET)/CT scan in localization of culprit lesion for biopsy and required intervention [surgical excision/radiofrequency ablation (RFA)] in patients with long-standing oncogenic osteomalacia (OOM)/tumour-induced osteomalacia. METHODS: 17 patients (8 males and 9 females) underwent 68Ga-DOTANOC PET/CT scan. The patients referred with clinical and biochemical evidence of hypophosphatemia and raised fibroblast growth factor-23. Qualitative and semi-quantitative parameters were used to identify culprit lesions. RESULTS: 68Ga-DOTANOC PET/CT scan revealed 52 lesions in 17 patients, and 37/52 of these lesions were tracer avid. 26/37 lesions were non-specific focal tracer-avid skeletal lesions (fractures or degenerative changes). 11/37 tracer-avid skeletal lesions present in 9 patients (3 lesions in 1 patient and 1 each in rest of the 8 patients) were highly suspicious for culprit lesions in view of high maximum standardized uptake value (SUVmax) (range 1.5-15.4; mean 7.0 ± 4.6), lesion size (0.9-5.0 cm; mean 3.3 ± 1.5) and associated soft-tissue component. During subsequent imaging with CT/MRI, 7/9 patients showed concordant lesions which were excised or biopsied and histopathologically verified as phosphaturic mesenchymal tumours. Surgical excision was resorted to in most of the detected lesions, and RFA was performed in one patient. CONCLUSION: There is some overlap in SUVmax between fracture-/bone-associated lesions and culprit lesions with a tendency of most non-culprit lesions to have lower SUVmax and no associated soft-tissue component. In such scenario, intensely tracer-avid, larger non-fracture lesions with soft-tissue component may lead to identification of culprit lesion among multiple lesions. Following detection of culprit lesion, surgical removal is the best treatment. RFA is alternative to surgery in cases where surgery is not possible owing to osteopenia/poor bone health. Advances in knowledge: The main challenge in patients of long-standing OOM is the presence of multiple skeletal lesions (both tumour- or tracer-avid fractures), and it is confusing to identify culprit lesion. This was noted in our study with 68Ga-DOTANOC and has not been mentioned in studies performed with 68Ga-DOTATATE/TOC PET/CT. In such scenario, 68Ga-DOTANOC PET/CT needs to be reviewed and read thoroughly to localize the culprit lesion out of the multiple tracer-avid lesions.

The safe zone for blinded sternal interventions based on CT evaluation of midline congenital sternal foramina.

OBJECTIVE: The aim of this study was to evaluate the safe zone for performing blind sternal procedures based on computed tomography (CT) evaluation of congenital midline sternal foramina using multidetector computed tomography (MDCT). MATERIALS AND METHODS: This retrospective study was carried out on 1,180 patients who underwent MDCT of the thorax from March 2015 to February 2016. The MDCT images were evaluated in axial and reformatted planes. Morphometry and prevalence of midline congenital sternal foramina (SF) and manubrio-foraminal distance (MFD) were evaluated. The safe zone was defined for a blinded intervention, based on palpable anatomical landmarks. Data were presented in terms of percentage, mean ± standard deviation and calculations were carried out using Microsoft Excel. RESULTS: The prevalence of SF in our study sample was 11.6 %. The majority of SF were located in a typical position in the lower sternal body at the level of fifth costo-chondral junction (CCJ) in 108 patients (78.8 %). The structure directly beneath the SF was mediastinal fat in 73 patients (53.3 %), followed by anterior pericardium in 44 patients (32.1 %) and lung parenchyma in 20 patients (14.6 %). The mean MFD in our study population was 11.90 ± 1.31 cm. CONCLUSIONS: Sternal interventions should be avoided at the level of fourth to sixth CCJ, which is considered the danger zone. An intervention at the fourth to sixth CCJ may lead to disastrous consequences in patients who have SF.