Prenatal diagnosis of a craniopharyngioma using ultrasonography and magnetic resonance imaging

Abstract

We report an infant with a craniopharyngioma which was detected in utero. Maternal uterine ultrasonography, done at 27 weeks because of polyhydramnios, revealed a 4 cm midline mass near the base of the fetal skull. At 31 weeks, magnetic resonance imaging of the maternal abdomen confirmed the presence of a mass in the region of the third ventricle and revealed hydrocephalus. Two days post-partum a computed tomography (CT)-guided needle biopsy of the mass was performed and recovered tissue which was histologically consistent with a craniopharyngioma. The infant's postnatal period was complicated by seizures, which were treated with phenobarbital, and by progressive hydrocephalus, necessitating placement of a ventriculo-peritoneal shunt. He also received therapy for central hypothyroidism and diabetes insipidus. The infant's parents refused permission for attempted resection of the tumour and he died at 8 weeks of age. This represents the second reported case of an antenatally detected craniopharyngioma. Four other cases of different intracranial tumours have been detected in utero using ultrasound, with no reported survivors past 3 days of age. There is a uniformly poor prognosis of such infants, but earlier diagnosis and intervention may change this result.

Radical Surgery in a Neonate with Craniopharyngioma

“…About 20 cases of craniopharyngioma detected in the prenatal or neonatal period are reported to date [2, 3, 12, 13, 14, 15, 16]. To our knowledge, the presented boy is the 3rd case in which craniopharyngioma was detected by fetal ultrasonography [2, 3].…”

“…To our knowledge, the presented boy is the 3rd case in which craniopharyngioma was detected by fetal ultrasonography [2, 3]. The management of neonatal craniopharyngioma is controversial.…”

“…Freeman et al [17]suggested that radical surgery in neonates is not indicated, whereas Hurst et al [15]recommended early surgery to prevent irreversible pressure effects. However, independent of fatal nihilism or aggressive therapy, the outcome of neonatal craniopharyngioma is very poor [2, 12, 17]. In those cases where surgery had been performed, death usually occurred in the early postoperative period [12].…”

“…The role of radiation is still debated, but in the very young child, the sequelae of this treatment are deleterious [5]. Overall prognosis of connatal brain tumors is considered to be very poor [2]. A common problem in the intra- and postoperative course of patients with craniopharyngioma is the disturbance of the salt-to-water ratio, which is seen especially in neonates and very young children [6, 7, 8, 9, 10, 11].…”

“…In utero detection of craniopharyngioma is extremely rare [2, 3]. If complete resection is possible, definitive cure may be achieved [4].…”