Huge acoustic neurinomas presenting in the late stage of pregnancy - Treatment options and review of literature

Background. Even though vestibular schwannomas rarely present during pregnancy, symptoms may appear or worsen particularly in this period. The clinical picture may include tinnitus, hearing abnormalities, and in large tumors, brain-stem and cerebellar compression with involvement of additional cranial nerves. Large vestibular schwannomas (also known as Acoustic Neurinomas) present a great challenge in peripartum management of both the mother and the fetus. Material and method. We present a case of a 24-year old woman, with headache, papilledema, ataxia, and multiple cranial nerve weakness, diagnosed in the 35th week of pregnancy. MRI demonstrated a huge vestibular schwannoma compressing the brainstem and causing obstructive hydrocephalus. Result. In the presence of high intra-cranial pressure a ventriculo-peritoneal shunt was first inserted, enabling delay of tumor surgery until after delivery. A successful elective cesarean section followed at 37 weeks, and radical tumor surgery was performed a week later. Maternal and fetal outcome were excellent. Discussion. The options, sequence and timing of the neurosurgical and obstetrical interventions are discussed. Other reports of large vestibular schwannomas that presented during pregnancy are reviewed. Advances in neurosurgery, neuroradiology, neuroanesthesiology and obstetrics are highlighted, and their impact on outcome is discussed in comparison to the poor results reported in the past. Emphasis is made on the importance of early diagnosis, that necessitates high-index of suspicion by the obstetrician, in any pregnant woman presenting abnormal neurological signs. Conclusion. We conclude that with a cooperative team approach, maternal and fetal prognosis can today be excellent, even in cases of large vestibular schwannomas diagnosed in the late stage of pregnancy.