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GENITOURINARY RADIOLOGY Table of Contents   
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


KHRWS Imageology Centre, Medical College Hospital, Calicut 673008, Kerala, India

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Keywords: Beckwith-Wiedmann syndrome, adrenal gland, cysts

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;11:135-7

How to cite this URL:
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 2017 Jun 27];11:135-7. 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.


   Case Report Top


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.


   Discussion Top


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. [1],[2]

Adrenal manifestations of BWS are adrenal cortical cytomegaly, adrenal cortical cysts, adrenal adenomas, carcinoma, neuroblastoma, adrenal calcifications and adrenal hemorrhages. [3],[4],[5],[6]. Benign hemorrhagic adrenocortical macrocysts (BHAM) are a rare manifestation of BWS [7]. Bilateral BHAM has not been reported in literature previously, to the best of our knowledge [8].

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 [9]. 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 [10]

 
   References Top

1.Cohen MM Jr, Gorlin RJ, Feingold M, Bensel RW. The Beckwith Weidmann Syndrome - seven new cases. Am J Dis Child 1971; 122: 515-519   Back to cited text no. 1  [PUBMED]  
2.Sotelo-Avila C, Siger DB. Syndrome of hyperplastic fetal visceromegaly and neonatal hypoglycaemia (Beckwith Syndrome). A report of 7 cases. Paediatrics 1970; 46: 240-251.  Back to cited text no. 2    
3.Lee F. Radiology of Beckwith Weidmann Syndrome. Radiol Clin North Am 1972; 10: 261-276  Back to cited text no. 3    
4.Pignatti CB, Maggiore G, Bianchi E, Magrini U, Ripamonte L. Adrenal hemorrhage in a new born with Beckwith Weidmann Syndrome. Am J Dis Child 1978; 132: 1296-1297.   Back to cited text no. 4    
5.Ichiba Y, Aoyama K,. Adrenal calcification in Beckwith Weidmann Syndrome. Am J Dis Child 1977; 131: 1296-1297.   Back to cited text no. 5    
6.Agrons GA, Lonergan GJ, Dickey GE, Perez-Monte JE. Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics 1999; 19: 989-1008   Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.McCauley RGK, Beckwith JB, Elias ER, Faerber EN, Prewitt Jr.LH, Berdon WE. Benign haemorrhagic adrenocortical macrocysts in Beckwith- Wiedemann Syndrome. AJR Am J Roentgenol 1991; 157: 549   Back to cited text no. 7    
8.Silvermann FN, Kuhn JP. The genitourinary tract. In: Caffey's paediatric Xray diagnosis - 9th ed. St. Louis: Mosby, 1993: 2144  Back to cited text no. 8    
9.Schwarzler P, Bernard JP, Senat MV, Ville Y: Prenatal diagnosis of fetal adrenal masses: differentiation between haemorrhage and solid tumour by color doppler sonography. Ultrasound Obstet Gynecol 1999; 13: 351-355  Back to cited text no. 9    
10.Sotelo-Avila C, Gonzalez -Crussi F, Fowler JW. Complete and incomplete forms of Beckwith Weidmann Syndrome: their oncologic potential. J Pediatr 1980: 96: 47-50   Back to cited text no. 10    

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Correspondence Address:
P V Ramachandran
KHRWS Imageology Centre, Medical College Hospital, Calicut 673008, Kerala
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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