Bilateral hemorrhagic adrenal cysts in an incomplete form of Beckwith-Wiedemann syndrome: MRI and prenatal US findings

Abstract

Benign hemorrhagic adrenal cysts are a cause of subdiaphragmatic mass in the fetus and neonate with Beckwith-Wiedemann syndrome. Characteristic imaging features on ultrasonography, color Doppler, and magnetic resonance imaging help differentiate adrenal hemorrhage from neonatal neuroblastoma and help avoid unnecessary surgery in these patients. Bilateral adrenal hemorrhage is self-limiting, and spontaneous resolution is the usual outcome. This report presents this rare condition with prenatal ultrasonographic and magnetic resonance imaging findings and reviews the differential diagnosis of neonatal adrenal masses.

Adrenal masses associated with Beckwith Wiedemann syndrome in the newborn

“…Differentiation of congenital adrenal haemorrhage from cystic neuroblastoma is difficult even with MRI. [2] Colour-coded Doppler ultrasonography may be useful. [7] According to Sauvat et al in a series of 53 patients on outcome of suprarenal localized masses when diagnosed during the perinatal period, localized neuroblastoma was found to be the most common suprarenal lesion in the perinatal period.…”

“…[8] Our review of the literature revealed 10 cases of incomplete BWS associated with either unilateral or bilateral adrenal cysts. [1,2,4,9] Most of the cystic adrenal lesions were on the right side (five), followed by left side (three) and bilateral lesions (two). Most of these masses showed adrenal cystomegaly with multiloculated hemorrhagic cysts in the adrenal gland without any evidence of tumour.…”

“…[4] Hemihypertrophy is considered as an incomplete form of BWS. [2,5] BWS, whether complete or partial, is associated with development of various tumours such as Wilms' tumour, neuroblastoma, hepatoblastoma and rhabdomyosarcoma. Various adrenal lesions are associated with both complete and partial BWS which included cortical hyperplasia, foetal cortical cytomegaly, pheochromocytoma, adrenal calcification, cystic adrenal haemorrhage, and neuroblastoma.…”

“…Beckwith Wiedemann syndrome (BWS) consists of macroglossia, abdominal wall defects, visceromegaly, gigantism, hemihypertrophy, hypoglycaemia, ear crease, nevus flavus, adrenal cystomegaly, midface hypoplasia and associated various tumours. [1][2][3] Incomplete BWS refers to a condition where two or three of these features are present. [4] The prevalence of malignancy in this syndrome is around 7.5% to 11%.…”

Neuroblastoma Presented with Polyhydramniosis

“…The differential diagnoses of fetal adrenal mass include adrenal hemorrhage, neuroblastoma, adrenal and cortical renal cysts, duplication of the renoureteral system, Beckwith-Wiedemann syndrome, and adrenal carcinoma and some of them (hemorrhage or neuroblastoma) can also be diagnosed prenatally by Doppler ultrasonagraphy [8][9][10].…”