[Surgical treatment of a pheocromocytoma bilateral in a 5 year old patient with the von Hippel-Lindau disease].
BACKGROUND
The disease of Von Hippel Lindau (VHL) is hereditary and causes a predisposition to the development of tumours. Organs such as the cerebellum, the pancreas, the kidney, the suprarenal glands and the retina are more usually affected by this disease.


METHODS
We present the case of a 5-year-old patient who suffers from asiymptomatic high blood pressure. In the family antecedents, it is relevant the case of the father, with pheocromocytoma bilateral, which led us to carry out a genetic study of his two sons. Our patient, the younger; presented a mutation of the VHL gene in the short arm of the chromosome 3. In one of the periodic controls, it could be detected high blood pressure of 160/100 mm. Hg, clinically asymptomatic. The other child did not present a genetic mutation and has no disease. The presence of high catecholamines, the detection of a 3 cm left suprarenal mass through the ecography, the TAC that did not show a right suprarenal pathology and the MBIG scintigraphy confirmed the diagnostic of pheocromocytoma. The RNM showed another 0.8-cm mass which confirmed a pheocromocytoma bilateral. We started the treatment against high blood pressure with fenoxibenzamine and diltiazem, and we controlled this problem. We also prepared the pre-and-post operation anesthetic strategy, which is so important for the surgical success. The operation started by a laparoscopic, we made left adrenalectomy and we had to reconvert to laparotomy to make partial right adrenalectomy. Six months after the operation, the patient is free from symptomatology and follows a treatment with glucocorticoides with smaller and smaller doses.


CONCLUSIONS
The case is exceptional because it embodies the following characteristics: early diagnostic age, family affectation and discovery of asymptomatic high blood pressure. It needed an appropriate preanesthetic and anesthetic preparation, which gave way to an operation without complications. The postoperation was also stable and presented no complications.
Servicio de Cirugía Pediátrica, Hospital "Germans Trias i Pujol", Badalona.
J A
JA
Blanco
D
D
Blanco
A
A
Alastrue
A
A
Castellví
R M
RM
Isnard
G
G
Pintos
A
A
Mangas
N
N
Roig
J M
JM
Casasa
D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000601:surgery*; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications / Q000601:surgery*; D010673:Pheochromocytoma / Q000150:complications / Q000601:surgery*; D006623:von Hippel-Lindau Disease / Q000150:complications*