Giant anterior sacral meningocele associated with hydroureteronephrosis and renal injury: illustrative case

Abstract

BACKGROUND Anterior sacral meningocele (ASM) is a defect in the closure of the neural tube. Patients can be asymptomatic or present with genitourinary, neurological, reproductive, or colorectal dysfunction. Magnetic resonance imaging (MRI) is the gold standard test because it can assess communication between the spinal subarachnoid space and the lesion and identify other abnormalities. Surgical correction is the definitive treatment because untreated cases have a mortality rate of more than 30%. OBSERVATIONS A 24-year-old woman with Marfan syndrome presented with polyuria, recurrent urinary tract infections, and renal injury for 3 months along with a globose abdomen, with a palpable mass in the middle and lower third of the abdomen that was massive on percussion. MRI showed an ASM consisting of two cystic lesions measuring 15.4 × 14.3 × 15.8 and 6.7 × 6.1 × 5.9 cm, respectively, compressing the distal third of the right ureter and causing a hydroureteronephrosis. Drainage and ligature of the cystic lesion were performed. The urinary outcome was excellent, with full recovery after surgery. LESSONS ASM should be suspected in all abdominal masses with progressive symptoms in the setting of Marfan syndrome. Computed tomography and MRI are important to investigate genitourinary anomalies or other types of dysraphism to guide the best surgical approach.

Successful Management of Multiple Giant Anterior Sacral Meningoceles Through an Anterior Approach in a Patient With Marfan Syndrome: A Case Report and Literature Review

“…In young patients, because of their small size, ASMs are usually asymptomatic. During the evolution course, ASM growth can exert pressure on surrounding organs, causing compression of the urinary tract, rectum, uterus, or sacral nerve roots, which manifest as lower urinary tract symptoms, chronic constipation, irregular menstruation, and pain or weakness in lower limbs [8][9][10].…”

“…Several surgical approaches have been described in the literature, including anterior transabdominal and posterior sagittal approaches [5,[8][9][10]. Although transrectal and transvaginal aspirations have been used in the past, these options have proven detrimental due to risks of infection and recurrence [15].…”