An 8-year-old female with Poland syndrome of the left chest initially presents to be considered for reconstructive surgery. On the preoperative MRI, along with the expected hypoplastic left hemithorax including the ribs, pectoralis major, and minor muscles, there is the absence of a left ovary in the pelvis and left kidney. Furthermore, there is an incidental 9 mm multicystic structure at the anterior spleen. No further workup was done at that time. After undergoing reconstructive surgery as well as scoliosis, occasional chest pain is experienced 10 years later. During the clinical workup, incidentally noted on the CT chest with contrast is increased anterior splenic hypodensity. The possibility for ectopic ovary or splenogonadal fusion was raised as well as involuted or atrophic multicystic dysplastic kidney, neoplasm, or vascular malformation. Initial workup included the US which revealed a 3.3 cm nonspecific lesion with posterior acoustic enhancement and without substantial internal vascularity. MRI of the spleen was performed which revealed a multicystic lesion, with imaging characteristics highly suggestive of an ectopic left ovary containing multiple physiologic follicles, from splenogonadal fusion. The uterus is also noted to be a right-sided unicornuate uterus. Furthermore, tiny non-communicative cystic changes in a linear manner left lateral to the psoas muscle, were identified, which may represent involuted/atretic remnant of the left multicystic dysplastic kidney.
SPR 2023 Annual Meeting & Postgraduate Course
Burgos Daylen Sire,