Wandering Spleen in a Pediatric Lung Transplant Patient With Filamin A Deficiency: An Incidental Finding

Filamin A (FLNA) deficiency is a known cause of progressive lung disease and need for pediatric lung transplant; however, what may be less well known to lung transplant providers are the extrapulmonary complications of FLNA deficiencies, such as wandering spleen. We present a patient who underwent a lung transplant for FLNA deficiency and later developed posttransplant abdominal pain.

An 11-year-old female who had previously undergone a bilateral lung transplant due to FLNA deficiency, causing progressive lung disease, presented with abdominal pain and diarrhea. The patient's stool was tested for causes of gastroenteritis using a gastrointestinal pathogen panel (GIPP). Additionally, an initial abdominal ultrasound was obtained to rule out surgical causes of acute abdomen. The initial abdominal ultrasound showed the spleen in the correct anatomical location. However, subsequent abdominal ultrasounds revealed an incidental finding of wandering spleen in multiple locations in the abdomen. As she has remained stable despite the migration of her spleen, the decision was made not to pursue surgical intervention and to continue monitoring with medical and surgical follow-up.

There are multiple complications caused by FLNA deficiency besides progressive respiratory failure, which include gastrointestinal (GI) complications such as wandering spleen. Wandering spleen is a rare clinical entity and, to our knowledge, this is the first case report of it being identified in a pediatric lung transplant patient. This case highlights the importance of transplant providers remaining vigilant when evaluating seemingly benign complaints such as abdominal pain in this population.