Knight Savannah Ellis, Donado Mayel Yepez, Gazzaneo Maria Carolina
Division of Pediatric Pulmonary, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas, USA.
Division of Pediatric Pulmonary and Critical Care, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas, USA.
Pediatr Transplant. 2025 Sep;29(6):e70161. doi: 10.1111/petr.70161.
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.
细丝蛋白A(FLNA)缺乏是进行性肺病和儿童肺移植需求的已知原因;然而,肺移植提供者可能不太了解的是FLNA缺乏的肺外并发症,如游走脾。我们报告一名因FLNA缺乏接受肺移植,随后出现移植后腹痛的患者。
一名11岁女性因FLNA缺乏导致进行性肺病,此前接受了双侧肺移植,出现腹痛和腹泻。使用胃肠道病原体检测板(GIPP)对患者的粪便进行了胃肠炎病因检测。此外,进行了初步腹部超声检查以排除急腹症的手术病因。最初的腹部超声显示脾脏位于正确的解剖位置。然而,随后的腹部超声检查发现脾脏在腹部多个位置意外游走。由于尽管脾脏移位但她仍保持稳定,因此决定不进行手术干预,而是继续通过医学和手术随访进行监测。
除了进行性呼吸衰竭外,FLNA缺乏还会引起多种并发症,包括游走脾等胃肠道(GI)并发症。游走脾是一种罕见的临床病症,据我们所知,这是第一例在儿童肺移植患者中发现游走脾的病例报告。该病例强调了移植提供者在评估该人群中看似良性的主诉(如腹痛)时保持警惕的重要性。