[Pancreatectomy level in the persistent hyperinsulinic hypoglycemia].
BACKGROUND
Persistent neonatal and infantile hyperinsulinemic hypoglycemia (PNHH) is a rare entity which remains to be elucidated but associated with severe lesions in pediatric patients. The aim of this study is to present our current surgical strategy face to this disease, based on our pathology findings and clinical experience.


METHODS
It is a retrospective study of 29 patients treated at our centre, medically and surgically. In 15 surgical patients, morphologic, morphometric and immunohistochemical studies for insulin, somatostatin and glucagon were performed and consequently it has been possible to establish a focal and different forms of a diffuse type.


RESULTS
Out of 29 patients studied, 25 were diagnosed before their first year old and 4 patients between the first and second infancy. Of the 25 first patients, one died at 7 hours postpartum. Twelve patients only received medical treatment, one of them died at 45 days of life and the remaining 11 patients had good outcome. Another 12 patients received in addition surgical treatment. In 2 of them we observed adenoma which was removed and patients cured. In the remaining 10 patients a subtotal pancreatectomy was performed. (One case was informed as normal and cured and the other 9 had the diffuse type). Of these 9 patients with diffuse type, 4 died, 3 cured and 2 underwent second surgery. Out of the 4 patients diagnosed in infancy, 3 underwent surgery (2 adenomas and 1 diffuse type) and the other one received only medical treatment.


CONCLUSIONS
Currently, we give medical treatment in all types and forms of PNHH. If the patient is resistant, we dismiss adenoma. If this is diagnosis we remove it. If the type is diffuse, near-total pancreatectomy is performed with a peroperatory biopsy. In cases of hyperplasia or mixed form we recommend a total pancreatectomy and in case of nesidioblastosis we recommend a partial pancreatectomy.
Martínez Ibáñez
V
V
Departamento de Cirugía Pediátrica, Hospital "Vall d'Ebron, Barcelona.
Gussinyer
M
M
Torán
N
N
Lloret
J
J
Abad
P
P
Carrascosa
A
A
D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications / Q000601:surgery*; D007003:Hypoglycemia / Q000209:etiology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010180:Pancreatectomy; D012189:Retrospective Studies