ABSTRACT
Acute pneumatosis cystoides intestinalis (PCI) has been described after bone marrow transplantation (BMT). Several case series have demonstrated successful conservative treatment of PCI in children. We present a child with Fanconi anaemia, who developed severe graft versus host disease of the gastrointestinal tract, skin and liver after BMT and an acute, severe form of PCI. Our case report illustrates the complexity of diagnostic and therapeutic procedures in PCI in immunocompromised children.
Subject(s)
Atrophy/complications , Bone Marrow Transplantation/adverse effects , Colon/pathology , Graft vs Host Disease/complications , Pneumatosis Cystoides Intestinalis/diagnostic imaging , Adolescent , Atrophy/pathology , Colectomy/methods , Diagnosis, Differential , Graft vs Host Disease/drug therapy , Humans , Male , Pneumatosis Cystoides Intestinalis/surgery , Pneumatosis Cystoides Intestinalis/therapy , Postoperative Complications/etiology , Tomography, X-Ray Computed/methods , Treatment OutcomeABSTRACT
Hybrid lesions (HLs) have elements of congenital pulmonary airway malformation and extrapulmonary sequestration (EPS) and belong to the congenital lung lesions. EPS usually arises in the thorax or the abdomen but rarely in the diaphragm. The preoperative diagnostic work-up based on chest radiograph, ultrasound (US) and CT often shows imprecise results. Therefore, the exact localisation of the lesion can only be ascertained intraoperatively. Here we present a patient, with an intradiaphragmatic HL, and demonstrate the difficulties of surgical decision making regarding the localisation of the lesion and discuss the value of minimal invasive surgery.
