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Case Report

Sclerosing Angiomatoid Nodular Transformation Presend Nodulartransformation Presenting with Abdominal Hemorrhage: First Report in Infancy

1
Pediatric Surgery Department, Children’s Hospital “G. di Cristina”, ARNAS "Civico-Di Cristina-Benfratelli", Palermo
2
Pathology Unit, Spedali Civili di Brescia
3
Pathology Unit, ARNAS “Civico-Di Cristina-Benfratelli”, Palermo
4
Pediatric Radiology Unit, Children’s Hospital “G. di Cristina”, ARNAS "Civico-Di Cristina-Benfratelli", Palermo
5
Gastroenterology Section, Department of Medicine, University of Perugia Medical School, Perugia
6
Pediatrics and Adolescent Unit, Department of Internal Medicine University of Pavia; Pediatric Unit, Fondazione IRCCS Policlinico San Matteo, Pavia
*
Author to whom correspondence should be addressed.
Pediatr. Rep. 2019, 11(2), 7848; https://doi.org/10.4081/pr.2019.7848
Submission received: 23 August 2018 / Revised: 23 August 2018 / Accepted: 13 December 2018 / Published: 23 May 2019

Abstract

A limited number of sclerosing Angiomatoid Nodular Transformation (SANT) have been reported in pediatric age. We describe the first case of SANT occurring in a nine-week-old female infant that was admitted to our unit for severe abdominal distension and rectal bleeding. Enlarged spleen was detected on physical examination. Laboratory investigations revealed severe anemia and coagulation abnormalities. Abdominal ultrasound and computed tomography revealed ascites and splenomegaly with a large mass at the lower medial splenic pole. A diagnosis of intraabdominal hemorrhage was presumed and an exploratory laparotomy was performed. A complete transformation of the giant splenomegaly to bossellated masses and multiple bleeding capsular ruptures without subcapsular hematoma were found and an urgent splenectomy was performed. At histology, a SANT was diagnosed (CD34, CD31, CD8 positivity). The postoperative follow up was uneventful. SANT may also occur in infancy with a potentially lifethreatening presentation. Splenectomy may represent the only treatment in severe cases.
Keywords: sclerosing angiomatoid nodular transformation; infant; spleen sclerosing angiomatoid nodular transformation; infant; spleen

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MDPI and ACS Style

Pelizzo, G.; Villanacci, V.; Lorenzi, L.; Doria, O.; Caruso, A.M.; Girgenti, V.; Unti, E.; Putignano, L.; Bassotti, G.; Calcaterra, V. Sclerosing Angiomatoid Nodular Transformation Presend Nodulartransformation Presenting with Abdominal Hemorrhage: First Report in Infancy. Pediatr. Rep. 2019, 11, 7848. https://doi.org/10.4081/pr.2019.7848

AMA Style

Pelizzo G, Villanacci V, Lorenzi L, Doria O, Caruso AM, Girgenti V, Unti E, Putignano L, Bassotti G, Calcaterra V. Sclerosing Angiomatoid Nodular Transformation Presend Nodulartransformation Presenting with Abdominal Hemorrhage: First Report in Infancy. Pediatric Reports. 2019; 11(2):7848. https://doi.org/10.4081/pr.2019.7848

Chicago/Turabian Style

Pelizzo, Gloria, Vincenzo Villanacci, Luisa Lorenzi, Orietta Doria, Anna Maria Caruso, Vincenza Girgenti, Elettra Unti, Laura Putignano, Gabrio Bassotti, and Valeria Calcaterra. 2019. "Sclerosing Angiomatoid Nodular Transformation Presend Nodulartransformation Presenting with Abdominal Hemorrhage: First Report in Infancy" Pediatric Reports 11, no. 2: 7848. https://doi.org/10.4081/pr.2019.7848

APA Style

Pelizzo, G., Villanacci, V., Lorenzi, L., Doria, O., Caruso, A. M., Girgenti, V., Unti, E., Putignano, L., Bassotti, G., & Calcaterra, V. (2019). Sclerosing Angiomatoid Nodular Transformation Presend Nodulartransformation Presenting with Abdominal Hemorrhage: First Report in Infancy. Pediatric Reports, 11(2), 7848. https://doi.org/10.4081/pr.2019.7848

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