RESUMEN
INTRODUCTION: Surgical procedures which aim at expanding the posterior cranial vault have come to play an increasingly important role in the treatment of syndromic craniosynostosis. The posterior cranial vault may be expanded by formal cranioplasty or by less invasive methods based on gradual posterior cranial vault expansion or distraction. These procedures share the common goals to prevent intracranial hypertension and progression toward turricephaly by effectively increasing the intracranial volume. They also reduce any local brain compression within the posterior cranial fossa. PATIENTS AND METHODS: This review describes the various techniques available for less invasive and gradual posterior cranial vault expansion and their rationale based on our experience on the last 40 cases. Volumetric data for three different surgical techniques have been compared (two cases of posterior cranial vault expansion by free-floating parieto-occipital bone flap, two with springs, and two with internal distractors). The volumetric analysis showed an expansion of 13 and 24 % for the free posterior flap, 18 and 25 % for the translambdoid springs, and 22 and 29 % for the distractors. DISCUSSION: Although these figures are not strictly comparable, our data suggest that these three techniques offer a potential for a significant increase in skull volume. The indications for such procedures must, however, be tailored according to the age and condition of the single patient.
Asunto(s)
Craneosinostosis/cirugía , Procedimientos Neuroquirúrgicos/instrumentación , Procedimientos Neuroquirúrgicos/métodos , Hueso Occipital/cirugía , Humanos , Colgajos QuirúrgicosRESUMEN
CONTEXT: Pediatric somatotropinoma is uncommon but usually more aggressive than in adults, creating therapeutic challenges. No treatment guidelines are available. OBJECTIVES: To describe the features of pediatric somatotropinomas and to assess therapeutic strategies based on an extensive literature review. DESIGN: We describe a pediatric case of aggressive somatotropinoma with an AIP mutation. We identified 137 pediatric somatotropinoma cases published between 1981 and 2010, and found 41 cases with AIP mutations in the main review. RESULTS: We found a slight male preponderance (59%). Median age was 9 years at symptom onset and 14 years at diagnosis. Macroadenomas accounted for 90% of the tumors; 2/3 of the children had hyperprolactinemia at diagnosis. The first-line treatment was pharmacotherapy in one third and surgery in 2/3 of the patients. Pegvisomant was used in 7 patients and produced significant improvement in 4. The male preponderance was higher in the subgroup with AIP mutations. Mutations leading to severe protein abnormalities were more common than reported in adults. CONCLUSION: Higher invasiveness and tumor volume in pediatric somatotropinomas require complex treatment combinations, which produce variable results. Pegvisomant is an effective drug whose usefulness in children remains to be determined. Genetic screening, particularly for AIP mutations, should be performed routinely.