Year : 2001 | Volume
: 11 | Issue : 3 | Page : 135--137
Case report : Benign hemorrhagic adrenocortical macrocysts - a rare manifestation of beckwith - wiedmann syndrome in a newborn
PV Ramachandran, E Devarajan, Della Harigovind, M Shamsudeen, CK Sasidharan
KHRWS Imageology Centre, Medical College Hospital, Calicut 673008, Kerala, India
P V Ramachandran
KHRWS Imageology Centre, Medical College Hospital, Calicut 673008, Kerala
|How to cite this article:|
Ramachandran P V, Devarajan E, Harigovind D, Shamsudeen M, Sasidharan C K. Case report : Benign hemorrhagic adrenocortical macrocysts - a rare manifestation of beckwith - wiedmann syndrome in a newborn.Indian J Radiol Imaging 2001;11:135-137
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Ramachandran P V, Devarajan E, Harigovind D, Shamsudeen M, Sasidharan C K. Case report : Benign hemorrhagic adrenocortical macrocysts - a rare manifestation of beckwith - wiedmann syndrome in a newborn. Indian J Radiol Imaging [serial online] 2001 [cited 2018 Jan 23 ];11:135-137
Available from: http://www.ijri.org/text.asp?2001/11/3/135/28392
Beckwith-Wiedmann syndrome (BWS) in neonates has a spectrum of manifestations in kidneys / adrenals, as well as in other organs. Amongst the renal / adrenal lesions, most of them lead to palpable masses; a majority being malignant lesions. One rare manifestation of BWS is benign hemorrhagic adrenocortical macrocysts (BHAM), reported in literature as usually being unilateral. We would like to report a case of bilateral BHAM, with characteristic US and CT imaging features that will help to differentiate it from malignant lesions.
A newborn was referred for abdominal US due to bilateral flank masses. The child had macroglossia and developed airway obstruction that needed immediate endotracheal intubation. Clinical examination revealed hemi-hypertrophy, protuberant abdomen and bilateral palpable flank masses. Blood examination revealed hypoglycemia. Clinically, the child was diagnosed to have Beckwith-Wiedmann Syndrome
US showed large multiloculated cystic masses bilaterally, pushing the kidneys downwards. The cysts were of varying sizes, some of them showing fluid-fluid levels [Figure 1],[Figure 2]. Other solid abdominal organs were apparently normal. CT showed large bilateral suprarenal multiloculated cystic masses displacing the kidneys downwards. A few cysts showed hyperdense layering in dependent parts, suggestive of hemorrhage into the cysts [Figure 3],[Figure 4]. Other abdominal organs were within normal limits.
In 1963 Beckwith reported three cases of a new syndrome consisting of macroglossia, omphalocele, visceromegaly, cytomegaly of adrenal cortex and hyperplasia of gonadal interstitial cells. In 1964 Wiedmann independently reported the syndrome. Beckwith-Wiedmann Syndrome (BWS) is characterized by fetal gigantism, visceromegaly, macroglossia, microcephaly, nevus flammeus, diaphragmatic hernia, hemihypertrophy, pancreatic and adrenal hyperplasia and omphalocele. Increased incidence of intra-abdominal tumors and hypoglycemia are also described. ,
Adrenal manifestations of BWS are adrenal cortical cytomegaly, adrenal cortical cysts, adrenal adenomas, carcinoma, neuroblastoma, adrenal calcifications and adrenal hemorrhages. ,,,. Benign hemorrhagic adrenocortical macrocysts (BHAM) are a rare manifestation of BWS . Bilateral BHAM has not been reported in literature previously, to the best of our knowledge .
Microscopic cortical cysts are a consistent finding in adrenal glands of patients with BWS. These cysts occur in the outer portion of the adrenal cortex and are lined by adrenocortical epithelium. Usually they are either empty or contain proteinaceous, cell-free fluid. Similar cysts may normally occur early in development and their occurrence later in gestation has been attributed to stress or maternal steroid use. In adrenal glands with BWS, usually these cysts are minute, but some accumulate blood or fluid and start enlarging. Hemorrhage may occur as a sequelae of regression of fetal cortex and dissection of hematoma peripherally to the permanent cortex where it apparently disrupts the walls of the cysts and enters the cyst cavities or as a result of focal necrosis of the cyst-lining wall. Hence the hemorrhage in BWS occurs in the permanent (outer) cortex of the adrenal, whereas in neonatal adrenal hemorrhage bleeding occurs in the fetal cortex.
Moreover, the cortical cysts that are a fundamental component of BHAM are not observed in neonatal adrenal hemorrhage. Once hemorrhage occurs, repeated episodes can cause progressive enlargement of cysts with coalescence. Organization with fibrosis and hemosiderin deposition follows. Calcification is a late sequelae of this process.
US is an excellent modality for screening of the adrenals, kidneys and liver in BWS. When necessary, further imaging can be done with CT. Solid adrenal tumors can be differentiated from adrenal hemorrhage - the commonest adrenal mass in a child. This has even been reported possible antenatally by color Doppler US . Because there is increased incidence of malignant renal / adrenal neoplasms in BWS patients, any complex echogenic adrenal mass in these patients should be viewed with caution.
Knowledge of the characteristic imaging features in US and CT helps in differentiating non-neoplastic BHAM from neoplastic lesions and helps in avoiding surgery in these patients. Follow- up however is essential to look for development of not only tumors in the region like nephroblastoma, neuroblastoma, adrenocortical carcinoma and hepatoblastoma, but also distant ones like glioblastoma of the brainstem, rhabdomyosarcoma of the orbit, fibrous tumor of the heart, umbilical myxoma, retroperitoneal ganglioneuroma and carcinoid of the appendix 
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