Compeán-Lorenzana AJ, De la Torre-Mondragón L, Gil-Vargas M, Martínez-Carreño UT, Madrigal-Malagón A

Language: Spanish
References: 7
Page: 84-86
PDF size: 123.58 Kb.

ABSTRACT

Introduction: Beckwith described a syndrome characterized by macroglossia, abdominal wall defects and growing alterations. The findings associated to BWS are hypoglycemia, tumor, exophtalmus and nevus. This work presents the association of BWS with a lesion not yet described.
Case 1: Newborn with nevus flammeus, exhophtalmus, macroglossia, wide umbilical cord, diastasis of the rectus, abdominal tumor and hypoglycemia. Abdominal ultrasound: cystic form on right hepatic border. Laparotomy: right hepatic cyst. Patology: simple hepatic cyst.
Case 2: Newborn with omphalocele, exophtalmus, macroglossia and hypoglycemia. Abdominal ultrasound: left hypocondrium cyst. TAC: Cyst sorrounding gall bladder. Patology: unilocular hepatic cyst
Discussion: The patients with BWS have risk for hypoglycemia and tumor. After analyzing medical web search of published cases since 1963, reviewing the Beckwith-Wiedemann Support Network and investigating directly with M.D Beckwit; this cases will be the first associated with hepatic cyst.

REFERENCES

1. DeBaun MR, Tucker MA. Risk of cancer during the first four years of life in children from the Beckwith-Wiedemann Syndrome. J Pediatr Surg 1998;132:398-400.

2. Chacon CM, Vega B, Palma CA, Palomo MC. Colestasis neonatal asociada a síndrome de Beckwith-Wiedemann. Bol Hosp Inf Mex 2002;59:718-21.

3. Vaughan WG, Sanders DW, Grosfeld JL, Plumley DA, Rescorla FJ, et.al. Favorable outcome in children with Beckwith-Wiedemann syndrome and intraabdominal malignant tumors. J Pediatr Surg 1995;30:1042-4.

4. Ciftci AO, Salman AB, Tanyel FC, Hicsonmez A. Bilateral multiple adrenal pseudocysts associated with incomplete Beckwith-Wiedemann Syndrome. J Pediatr Surg 1997;32:1388-1390.

5. Choyke PL, Siegel MJ, Craft AW, Green DM, DeBaun MR: Screening for Wilms tumor in children with Beckwith Wiedemann Syndrome or idiopathic hemihypertrophy. Med Pediatr Oncol 1999; 32:196-200.

6. 6.http://www.beckwith-wiedemannsyndrome.org/

7. http://www.beckwith-wiedemann.org