Case Report: Massive Intestinal Pneumatosis and Pneumoretroperitoneum Following Hematopoietic Stem Cell Transplantation in a 2-Year-Old Child

Giorgia Contini,Francesco Morini,Arianna Bertocchini,Roberto Carta,Alessandro Inserra,Pietro Bagolan,Pietro Merli

doi:10.3389/fped.2021.700736

Abstract

A 2-year-old boy with severe combined immunodeficiency (SCID) developed intestinal graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation (HSCT), associated with massive intestinal pneumatosis (IP), pneumoretroperitoneum (PRP), and pneumomediastinum. His fair clinical conditions allowed conservative management, with progressive normalization of imaging findings. The patient did not require surgery and is alive and in good clinical conditions at follow-up. In children with GVHD-related IP but good clinical conditions and no signs of peritonitis, IP is not a mandatory indication for surgery, despite its potentially striking imaging features. Conservative management, with intestinal rest, decompression, and antibiotics, often allows regression of the clinical picture.

Highlights

Graft-versus-host disease (GVHD) is the most common immunological complication of allogeneic hematopoietic stem cell transplantation (HSCT); alloreactive donor T cells recognize recipient’s cells as non-self, causing direct and indirect damages to different organs
We present the case of a child with intestinal Chronic GVHD (cGVHD) following intestinal Acute GVHD (aGVHD) after bone marrow transplantation (BMT) who developed massive intestinal pneumatosis (IP) with pneumoretroperitoneum (PRP) but no signs of pneumoperitoneum (PP) or peritonitis
We report on a child with severe combined immunodeficiency (SCID) who developed IP, PRP, and pneumomediastinum associated with GVHD following HSCT

Summary

INTRODUCTION

Graft-versus-host disease (GVHD) is the most common immunological complication of allogeneic hematopoietic stem cell transplantation (HSCT); alloreactive donor T cells recognize recipient’s cells as non-self, causing direct and indirect damages to different organs. Acute GVHD (aGVHD) usually develops within the first 100 days of HSCT and can involve the skin, liver, and GI. Chronic GVHD (cGVHD) can occur de novo or following aGVHD, arising several months after HSCT, and can involve almost any organ. This complication leads to significant morbidity, reduced quality of life, and decreased overall survival. We present the case of a child with intestinal cGVHD following intestinal aGVHD after bone marrow transplantation (BMT) who developed massive IP with pneumoretroperitoneum (PRP) but no signs of pneumoperitoneum (PP) or peritonitis. The patient did not require abdominal surgery and is alive at 6 months of follow-up; despite several lines of immunosuppressive therapy, he still presents recurrent flare of GI GVHD (Figure 3)

DISCUSSION

Findings

ETHICS STATEMENT