Presentations

Abstract

Introduction: Vestibular schwannomas (VS) are Schwann cell-derived lesions that originate from the vestibular segment of the eighth cranial nerve at the cerebellopontine angle (CPA). Roughly 40 cases of VS diagnosed or causing symptoms during pregnancy have been globally documented in the literature. Corresponding to this paucity is the lack of in-depth discussion on the experience of managing these tumors, which can culminate in devastating consequences for both the mother and fetus (e.g. increased intracranial pressure (ICP), hydrocephalus, gestational hypertension, and premature delivery), we present two cases of VS during pregnancy. Here we present two cases of VS during pregnancy to emphasize the importance of a coordinated, multi-disciplinary approach involving neurosurgery, obstetrics, and anesthesia.

Case Information: Patient 1 is a 27-year-old G2P1 female with noncontributory history who presented with nausea, vomiting, headache, gait disturbances, lower extremity weakness, facial droop, and visual changes at 31 weeks gestation. Symptoms had been persistent and worsening since 11 weeks. MRI demonstrated a 4.5 x 3.7 x 3.6 cm, extra-axial, left posterior fossa mass with extensive mass effect on adjacent structures including the brainstem, left middle cerebellar peduncle, fourth ventricle, and left cerebellum. An EVD was placed, and the patient was scheduled for c-section. She underwent emergency c-section at 33 weeks due to spontaneous rupture of membranes. Left retrosigmoid craniotomy was then performed for tumor resection. Pathology confirmed VS. Post-operatively, the patient experiences 7th nerve palsy, headaches, and depression which have all shown gradual improvement. At 1-year follow-up, MRI demonstrated stable residual enhancement that continues to be monitored. Patient 2 is a 28-year-old G1P0 female with noncontributory history who presented with intermittent headaches, tinnitus, and blurred vision at 19 weeks. MRI showed a 4.5 cm right CPA mass with local mass effect and hydrocephalus. She was followed outpatient with a plan to delay surgery until after delivery but admitted 1 month later with rapidly progressing hearing and vision loss. An EVD was placed, and bilateral optic nerve fenestration was performed. C-section was performed at 34 weeks without complication. Two-stage tumor resection was performed by retrosigmoid craniectomy and a translabyrinthe approach. The patient's vision improved post-op but she continues to experience 6th nerve palsy with diplopia. At 10-month follow-up, MRI demonstrated stable residual tumor with evidence of psuedomeningocele. Overall, the patient and her child are doing well, and she will continue to be monitored annually.

Discussion/Clinical Findings: In both of our cases, EVD alleviated neurological symptoms, and a cesarean section was planned for 34 weeks. Cesarean section (CS) is the recommended mode of delivery for patients with VS, as vaginal delivery is associated with increased intracranial pressure and associated neurological deficit. Patient 2 had a successful CS, as planned, at 34 weeks, while Patient 1 experienced spontaneous rupture of membranes at 33 weeks and had to have an emergent CS. Both deliveries were uncomplicated and the fetuses were transferred to NICU, and surgical resection of the tumors were planned 1-2 weeks postpartum. The delay in resection is recommended to allow time for maternal hemodynamics to stabilize. Patient 1 had their tumor resected successfully 1-week post-delivery, while Patient 2 had a two-stage resection at 2 and 4 weeks postpartum.

Conclusion: In this mini-case series, we presented two cases that underscore the diagnostic and therapeutic challenges surrounding vestibular schwannoma in pregnancy. Multiple specialties were involved in care, including neurosurgery, obstetrics, anesthesia, ophthalmology, and ENT. We emphasize the need for a multidisciplinary approach that prioritizes both maternal and fetal well-being. Our successful outcomes reflect the critical role of timely interventions, such as intracranial pressure management, in preventing irreversible neurological damage. While guidelines recommend conservative management until full-term, these cases reveal that earlier intervention may be vital for preserving maternal health. Serial surveillance and patient-centered care in the postpartum period are crucial to address any residual disease and optimize outcomes for the mother. These cases contribute valuable insights to a limited body of literature on this complex clinical scenario, offering more data for future management strategies in similar cases.