Author information
Dwivedi JS *, Gupta AS **
(* Third Year Resident, ** Professor, Department of Obstetrics and
Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India)
Abstract
Vestibular
schwannoma occurs more commonly in females. Though it is infrequently seen
during pregnancy the symptoms might be worsened. The early diagnosis followed
by prompt treatment is very important. We present a case of multigravida with
full term gestation referred to us with a large vestibular schwannoma.
Introduction
Sir
Charles Ballance performed the first successful surgery for a vestibular schwannoma in 1894.[1] Since
then many technological advancements have been made in the diagnosis and
management of this tumor with varied symptoms. However the algorithm for management of cases detected in
pregnancy still remains at a naive stage and needs to be studied further.
Case report
A
30 yr old G5P3L3A1 with 41 weeks of gestation with previous full term normal deliveries
was referred to us from a private practitioner in view of recently diagnosed
acoustic schwannoma for emergency cesarean section and further management. The
patient was registered with the referring private practitioner from third month
of gestation and had a regular follow up. The patient gave history of headache,
blurring of vision, gait imbalance since fifth month of gestation which she did
not consider important and did not inform the treating doctor. Fifteen days prior
to presenting to us, patient had a fall due to imbalance for which she was
investigated and an MRI from a private center diagnosed a 6.5 cm x 5.5 cm x 4
cm mass occupying the left cerebellopontine angle with compression of fourth
ventricle and evidence of obstructive hydrocephalus. A neurosurgery opinion was
taken at that hospital. She was advised to follow up after six weeks of
delivery and the decision regarding mode of delivery was left to the
obstetrician. A neurologist, neurosurgeon, and an ophthalmologist from our
tertiary care center evaluated the patient clinically. She was diagnosed with bilateral papilledema
and central nystagmus. She was started on injection mannitol 100 mg 6 hourly,
and tablet acetazolamide twice daily. All other parameters were normal.
Initially the neurosurgeon decided to operate and excise the tumor urgently and
then allow the patient to go into spontaneous labor; however, the anesthetist
denied fitness and recommended neurosurgery after delivery. The patient went
into spontaneous, precipitate labor and uneventfully delivered a female child
of 3.4 kg baby weight. There was no evidence of any coning. Post delivery the
course in ward was uneventful. Subsequently the neurosurgeon decided to wait
for 6 weeks after child birth for definitive surgery.
Discussion
Cerebellopontine
angle (CPA) is an area of neurological tissue. Tumor in this area might present
with varied signs and symptoms. Any patient with unilateral sensorineural
hearing loss or tinnitus should be evaluated for cerebellopontine angle tumor.
Although the differential diagnosis for CPA tumors is quite large, the vast
majority are vestibular schwannomas. Vestibular or acoustic schwannoma form 8 -
10% of the total intracranial tumors.[2] These are encapsulated, benign, slow
growing tumors with a growing rate of approximately 2 mm/ year. Symptoms are
mostly produced due to the pressure on the surrounding neural tissue. Acoustic
schwannoma seldom presents during pregnancy, however the symptoms of the same
can be exaggerated or worsened during the pregnancy.[3] The symptoms include
tinnitus, hearing abnormalities and
symptoms due to compression of the cerebellum and brain stem. These are
mainly reported with large tumors as was also seen in our case. MRI imaging is
the gold standard for vestibular schwannomas.[4] Large tumors are difficult to
manage. Surgical management of the tumor and delivery in the same sitting have
not been reported in the literature. The recent treatment options include
microsurgical excision, stereotactic radiosurgery or fractionated
radiotherapy.[2] In 1917, accelerated growth of vestibular schwannoma during
pregnancy was first reported by Harvey Cushing.[1] The correlation of increase
in size of the tumor with the pregnancy led to research of presence of estrogen
receptor (ER) on the tumor. ER α expression is increased in cases of sporadic
vestibular schwannoma.[2] There have been reported cases of operative
management of acoustic schwannomas in pregnancy, however the preferred time is
the second trimester.[5] In our case the patient presented late at 41 weeks, and
the difference of opinion between neurosurgeons and anesthetist delayed the
neurosurgery. Patient soon went into spontaneous labor, hence operative
management of the tumor was deferred till end of puerperium by the
neurosurgeons. Patient did not develop any neurological complication during delivery
or immediately postpartum leaving a point of thought, that these patients at
full term pregnancy where dilemma exists about the mode of delivery, can be
given a trial of labor in an institute where combined obstetric and
neurosurgical emergency services are available. However to conclude that such
modality can be of benefit requires further study.
References
- Machinis TG, Fountas KN, Dimopoulos V, Robinson JS. History of Acoustic Neurinoma Surgery. Neurosurg Focus. 2005;18(4) Available from: http://www.medscape.com/viewarticle/503952_2
- Brown CM, Ahmad ZK, Ryan AF, Doherty JK. Estrogen receptor expression in sporadic vestibular schwannomas. Otol Neurotol. 2011 Jan; 32(1): 158-62.
- Beni- Adani L, Pomeranz S, Flores I, Shoshan Y, Ginosar Y, Ben- Shachar I. Huge acoustic neurinomas presenting in the late stage of pregnancy. Treatment options and review of literature. Acta Obstet Gynecol Scand. 2001 Feb;80(2):179-84.
- Kutz JW, Roland PS. Acoustic Neuroma Workup. Available from: http://emedicine.medscape.com/article/882876
- Shah KJ, Chamoun RB. Large Vestibular Schwannomas Presenting during Pregnancy: Management Strategies. J Neurol Surg B Skull Base. 2014 Jun;75(3):214-20.
Dwivedi JS, Gupta AS. Acoustic Schwannoma In a Full Term Pregnant Patient JPGO 2015. Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/acoustic-schwannoma-in-full-term.html