OBJECTIVE AND IMPORTANCE: Massive congenital intracranial teratomas with extracranial extension are rare. The prognosis in these cases has been poor, with stillbirth or immediate postpartum death as the usual outcome. With recent advances in fetal monitoring, neonatal care, and surgical techniques used for the management of complex tumors of the cranial base, some of these lesions may now be amenable to radical surgical resection and then immediate craniofacial reconstruction. CLINICAL PRESENTATION: A neonate with a large congenital immature teratoma involving the entire left side of the cranium and face was evaluated at our institution 1 day after birth. INTERVENTION: Total resection of the mass and then immediate reconstruction of the deformed orbit, maxilla, and mandible were performed at 9 days of age. Additional operations on the midface and mandible to allow for a functional bite were subsequently required as the child developed during the next 2 years. Four years after resection, the patient exhibited a reasonable cosmetic result and only mild developmental delay. CONCLUSION: We conclude that acceptable functional and cosmetic outcomes can be achieved by early intervention, consisting of radical resection and immediate craniofacial reconstruction, in some neonates with massive congenital craniofacial teratomas.
OBJECTIVE AND IMPORTANCE: Massive congenital intracranial teratomas with extracranial extension are rare. The prognosis in these cases has been poor, with stillbirth or immediate postpartum death as the usual outcome. With recent advances in fetal monitoring, neonatal care, and surgical techniques used for the management of complex tumors of the cranial base, some of these lesions may now be amenable to radical surgical resection and then immediate craniofacial reconstruction. CLINICAL PRESENTATION: A neonate with a large congenital immature teratoma involving the entire left side of the cranium and face was evaluated at our institution 1 day after birth. INTERVENTION: Total resection of the mass and then immediate reconstruction of the deformed orbit, maxilla, and mandible were performed at 9 days of age. Additional operations on the midface and mandible to allow for a functional bite were subsequently required as the child developed during the next 2 years. Four years after resection, the patient exhibited a reasonable cosmetic result and only mild developmental delay. CONCLUSION: We conclude that acceptable functional and cosmetic outcomes can be achieved by early intervention, consisting of radical resection and immediate craniofacial reconstruction, in some neonates with massive congenital craniofacial teratomas.