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3. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Author

Baiges, A., Turon, F., Simón-Talero, M., Tasayco, S., Bueno, J., Zekrini, K., Plessier, A., Franchi-Abella, S., Guerin, F., Mukund, A., Eapen, C.E., Goel, A., Shyamkumar, N.K., Coenen, S., Gottardi, A. De, Majumdar, A., Onali, S., Shukla, A., Carrilho, F.J., Nacif, L., Primignani, M., Tosetti, G., Mura, V. La, Nevens, F., Witters, P., Tripathi, D., Tellez, L., Martínez, J., Álvarez-Navascués, C., López, M.L. Fraile, Procopet, B., Piscaglia, F., Koning, B. de, Llop, E., Romero-Cristobal, M., Tjwa, E.T., Monescillo-Francia, A., Senzolo, M., Perez-LaFuente, M., Segarra, A., Sarin, S.K., Hernández-Gea, V., Patch, D., Laleman, W., Hartog, H., Valla, D., Genescà, J., García-Pagán, J.C., Baiges, A., Turon, F., Simón-Talero, M., Tasayco, S., Bueno, J., Zekrini, K., Plessier, A., Franchi-Abella, S., Guerin, F., Mukund, A., Eapen, C.E., Goel, A., Shyamkumar, N.K., Coenen, S., Gottardi, A. De, Majumdar, A., Onali, S., Shukla, A., Carrilho, F.J., Nacif, L., Primignani, M., Tosetti, G., Mura, V. La, Nevens, F., Witters, P., Tripathi, D., Tellez, L., Martínez, J., Álvarez-Navascués, C., López, M.L. Fraile, Procopet, B., Piscaglia, F., Koning, B. de, Llop, E., Romero-Cristobal, M., Tjwa, E.T., Monescillo-Francia, A., Senzolo, M., Perez-LaFuente, M., Segarra, A., Sarin, S.K., Hernández-Gea, V., Patch, D., Laleman, W., Hartog, H., Valla, D., Genescà, J., and García-Pagán, J.C.

Abstract

Contains fulltext : 220051.pdf (Publisher’s version ) (Closed access), Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

Published
2020

8. Redefining caliceal anatomy in axial plane: selective overlapping of the appropriate contiguous sections on delayed post-contrast CT

Author

Das, S.K., Dhanraj, P.M., Shyamkumar, N.K., Kota, K.G., Kekre, N., and Gopalakrishnan, G.

Subjects
Kidneys -- Properties, Health
Abstract

Developments in endourology, percutaneous nephrostolithotomy and nephrostomy with newer advances in radiological imaging of the pelvicaliceal system have rekindled interest in the axial anatomy of the collecting system. The objective of this study was to evaluate the placement of the posterior calyx in relation to the coronal plane of the body viz., the corporo-caliceal angle. A simple, but less cumbersome measurement was utilized than the previously defined methods to assess the relationship of the posterior calices in relation to the frontal plane of the body. Data from 32 right kidneys and 31 left kidneys from 41 patients who underwent delayed CT pyelogram in supine or prone position was examined over a year. The angles subtended by the posterior calices in supine and prone position for the right and left kidneys were 61 and 63 degrees and 52 and 64.3 degrees, respectively. The variations in the orientation of the posterior calices on either side (p< 0.05) and in supine or prone position (p < 0.05) were statistically not significant. The calices were seen to vary considerably in position, shape and number with only a few falling into the classical Brodel and Hodson's type. There was no significant change in the intrarenal anatomy with change in position. Hence, the data obtained from a supine study can be extrapolated to a procedure done in prone position., Introduction Choice of an ideal end-on posterior interpolar calyx located along the avascular plane of the Brodel is essential to safely perform endourological procedures. Until now, the information available on [...]

Published
2009

9. Redefining caliceal anatomy in axial plane: selective overlapping of the appropriate contiguous sections on delayed post-contrast CT

Author

Das, S.K., Dhanraj, P.M., Shyamkumar, N.K., Kota, K.G., Kekre, N., and Gopalakrishnan, G.

Subjects
Kidneys -- Properties, Health
Abstract

Developments in endourology, percutaneous nephrostolithotomy and nephrostomy with newer advances in radiological imaging of the pelvicaliceal system have rekindled interest in the axial anatomy of the collecting system. The objective of this study was to evaluate the placement of the posterior calyx in relation to the coronal plane of the body viz., the corporocaliceal angle. A simple, but less cumbersome measurement was utilized than the previously defined methods to assess the relationship of the posterior calices in relation to the frontal plane of the body. Data from 32 right kidneys and 31 left kidneys from 41 patients who underwent delayed CT pyelogram in supine or prone position was examined over a year. The angles subtended by the posterior calices in supine and prone position for the right and left kidneys were 61 and 63 degrees and 52 and 64.3 degrees, respectively. The variations in the orientation of the posterior calices on either side (p< 0.05) and in supine or prone position (p < 0.05) were statistically not significant. The calices were seen to vary considerably in position, shape and number with only a few falling into the classical Brodel and Hodson's type. There was no significant change in the intrarenal anatomy with change in position. Hence, the data obtained from a supine study can be extrapolated to a procedure done in prone position., Table of Contents Abstract Introduction Material and Methods Results Discussion Conclusion References Introduction Choice of an ideal end-on posterior interpolar calyx located along the avascular plane of the Brodel is [...]

Published
2008

10. Intraosseus schwannoma

Author

Isaac, J., Shyamkumar, N.K., and Karnik, S.V.

Subjects
Cardiovascular tumors -- Diagnosis, Cardiovascular tumors -- Case studies, Schwann cells -- Diseases, Schwann cells -- Diagnosis, Schwann cells -- Case studies, Bone tumors -- Diagnosis, Bone tumors -- Case studies
Abstract

A 35-year-old lady presented with gradually increasing swelling over right arm for the last four years without a history of trauma. The initial painless swelling showed a sudden increase in [...]

Published
2004

14. Tuberculosis—The Great Mimicker.

Author

Prapruttam, Duangkamon, Hedgire, Sandeep S., Mani, Sunithi Elizabeth, Chandramohan, Anuradha, Shyamkumar, N.K., and Harisinghani, Mukesh

Abstract

Tuberculosis is an immense health problem in the developing world, and it remains a health care challenge in the developed world. It can affect virtually any organ system in the body. Diagnosis of tuberculosis is often difficult. Many patients with tuberculosis present with nonspecific symptoms, negative purified protein derivative skin test result, and negative findings on culture specimens. Cross-sectional imaging with ultrasound, multidetector computed tomography, and magnetic resonance imaging plays an important role in the diagnosis of tuberculosis. Tuberculosis demonstrates a variety of radiologic features depending on the organ involved and can mimic a number of other disease entities. Cross-sectional imaging alone is insufficient in reaching a conclusive diagnosis. Tuberculosis is a great mimicker as its radiologic manifestations can simulate numerous other diseases across the body systems. However, recognition and understanding of the common and uncommon radiologic manifestations of tuberculosis should alert considering tuberculosis in the high-risk population and correct clinical setting to enable appropriate treatment. [Copyright &y& Elsevier]

Published
2014
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15. Isolated fourth ventricular cysticercus cyst: MR imaging in 4 cases with short literature review.

Author

Singh, S., Gibikote, S.V., and Shyamkumar, N.K.

Subjects
BRAIN tumors, CYSTICERCUS, HYDROCEPHALUS, MEDICINE, NEUROSCIENCES
Abstract

We describe the magnetic resonance imaging (MRI) signal characteristics of isolated (solitary lesion) intra fourth ventricular cysticercus cyst in 4 patients who clinically presented with obstructive hydrocephalus. All patients had routine MRI sequences (T1, T2, & proton density-weighted imaging), Fluid Attenuation Inversion Recovery (FLAIR), and postgadolinium imaging followed by cerebrospinal fluid (CSF) flow study. It revealed a CSF signal intensity (on all pulse sequences), intra fourth ventricular cyst with a nidus (scolex), and wall enhancement. On T1-weighted and FLAIR images, the cyst wall and nidus (scolex) were seen in 3 cases, which were not seen in other routine sequences. The CSF flow study showed the intraluminal nature of the cyst. The MRI features suspected a cysticercus cyst, and per-operative findings and histopathological examination confirmed the diagnosis. The review of literature of the intra fourth ventricular cyst is briefly discussed. [ABSTRACT FROM AUTHOR]

Published
2003

16. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Author

Baiges, Anna, Turon, Fanny, Simon-Talero, Macarena, Tasayco, Stephanie, Bueno, Javier, Zekrini, Kamal, Plessier, Aurelie, Franchi-Abella, Stephanie, Guerin, Florent, Mukund, Amar, Eapen, CE, Goel, Ashish, Shyamkumar, Nidugala Keshava, Coenen, Sandra, De Gottardi, Andrea, Majumdar, Avik, Onali, Simona, Shukla, Akash, Jose Carrilho, Flair, Nacif, Lucas, Primignani, Massimo, Tosetti, Giulia, La Mura, Vicenzo, Nevens, Frederik, Witters, Peter, Tripathi, Dhiraj, Tellez, Luis, Martinez, Javier, Alvarez-Navascues, Carmen, Fraile Lopez, Miguel Lopez, Procopet, Bogdan, Piscaglia, Fabio, de Koning, Barbara, Llop, Elba, Romero-Cristobal, Mario, Tjwa, Eric, Monescillo-Francia, Alberto, Senzolo, Marco, Perez-LaFuente, Mercedes, Segarra, Antonio, Sarin, Shiv Kumar, Hernandez-Gea, Virginia, Patch, David, Laleman, Wim, Hartog, Hermien, Valla, Dominique, Genesca, Joan, Carlos Garcia-Pagan, Juan, Garcia-Criado, Angeles, Darnell, Anna, Belmonte, Ernest, Ferrusquia-Acosta, Jose, Magaz, Marta, Vidal-Gonzalez, Judit, Horia, Stefanescu, Nicoara-Farcau, Oana, Joseph, Philip, Zachariah, Uday, Moses, Vinu, Mammen, Suraj, Ahmed, Munawwar, Koshy, George, Eapen, Anu, Sajith, KG, Vyas, FL, Raju, RS, Rymbai, Manbha L, Arulkumar, S, Ramachandran, Jeyamani, Elias, Elwyn, Baiges A., Turon F., Simon-Talero M., Tasayco S., Bueno J., Zekrini K., Plessier A., Franchi-Abella S., Guerin F., Mukund A., Eapen C.E., Goel A., Shyamkumar N.K., Coenen S., De Gottardi A., Majumdar A., Onali S., Shukla A., Carrilho F.J., Nacif L., Primignani M., Tosetti G., La Mura V., Nevens F., Witters P., Tripathi D., Tellez L., Martinez J., Alvarez-Navascues C., Fraile Lopez M.L., Procopet B., Piscaglia F., de Koning B., Llop E., Romero-Cristobal M., Tjwa E., Monescillo-Francia A., Senzolo M., Perez-LaFuente M., Segarra A., Sarin S.K., Hernandez-Gea V., Patch D., Laleman W., Hartog H., Valla D., Genesca J., Garcia-Pagan J.C., Gastroenterology & Hepatology, Pediatrics, and Surgery

Subjects
0301 basic medicine, Male, Vascular Malformations, medicine.medical_treatment, International Cooperation, Liver transplantation, 0302 clinical medicine, Malalties hereditàries, Hepatopulmonary syndrome, 610 Medicine & health, Child, Hepatic encephalopathy, Portal Vein, Malalties del fetge, Liver Neoplasms, vascular diseases of the liver, Middle Aged, Abernethy, Child, Preschool, 030211 gastroenterology & hepatology, Female, medicine.symptom, Genetic diseases, Adult, medicine.medical_specialty, Adenoma, Adolescent, Hypertension, Pulmonary, Asymptomatic, 03 medical and health sciences, Young Adult, extrahepatic portosytemic shunt, medicine, Humans, Liver diseases, Aged, Retrospective Studies, Abernethy Malformation, Hepatology, business.industry, Infant, Retrospective cohort study, medicine.disease, Pulmonary hypertension, Surgery, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], 030104 developmental biology, Hepatic Encephalopathy, Congenital malformation, Portosystemic shunt, business, Hepatopulmonary Syndrome
Abstract

Contains fulltext : 220051.pdf (Publisher’s version ) (Closed access) Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

Published
2018

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