Favorable management of symptomatic cerebellar hemangioblastoma presenting with obstructive hydrocephalus during pregnancy: A case report and literature review

Mechanism of increase in the size of hemangioblastoma during pregnancy

Cerebellar hemangioblastoma is a benign vascular tumor and accounts for approximately 3% of all central nervous system tumors.[7,20] The combination of hemangioblastoma and pregnancy is rare because of its low incidence. The previous publications have focused on the effect of pregnancy on this tumor and there was a hypothesis that pregnancy could potentially accelerate hemangioblastoma progression through fluid retention, increasing venous pressure, and the occurrence of hormone receptors on tumor cell in patients with Von Hippel-Lindau disease.[1,6,22] However, it was also reported that the progression of symptoms in hemangioblastoma was simply a result of the natural course of the disease rather than the effects of pregnancy because gestational age is consistent with the peak age for the presence of hemangioblastoma symptoms.[21] Although there is currently no direct evidence of a relationship between pregnancy and hemangioblastoma progression, managing these patients to safely control the tumor and protect the fetus has been a major challenge.

Favorable management for hemangioblastoma during pregnancy

A variety of management options for hemangioblastoma occurring during pregnancy has been reported in the relevant literature, including conservative management with close observation, spinal fluid detour, and direct surgery. Conservative management with close observation is not a viable option for symptomatic patients because these conditions can deteriorate during pregnancy.[15]

To date, there are a total of 12 English reports regarding direct surgical treatment of symptomatic hemangioblastomas during pregnancy in 16 cases, primarily in case reports or case series [Table 1]. In these reports, 14 of the 16 patients underwent urgent tumor resection (UTR) during gestation, and the pregnancies were continued; in 12 patients, the infant was delivered at full term with no problems.[2,3,5,8,9,12-15,19] Naidoo and Bhigjee performed UTR in two patients at 21 and 33 weeks of gestation, achieving good results.[13] Similarly, Nathan et al. reported that a 28-year-old woman underwent UTR without spinal fluid detour during the second trimester of pregnancy with good results and continued pregnancy resulting in a full-term delivery.[14] Spinal fluid detour may also be an option for temporary symptomatic improvement; however, patients may deteriorate after shunting and may experience shunt-related complications.[17] Kasarskis et al. reported neurological deterioration after shunting in a patient with symptomatic cerebellar hemangioblastoma and described the need for direct emergency surgery on the hemangioblastoma.[8] The causes of deterioration after shunting include rapid tumor growth due to intravascular re-expansion of the tumor and direct pressure on the brainstem due to loss of the cushioning effect between the spinal fluid and the lesion. Ma et al. reported in 24 hemangioblastoma in pregnancy, four patients had external ventricular draining as an initial treatment; two patients had abortion at 6 weeks and 16 weeks, and other two patients had emergent cesarean section.[12] From this result, external ventricular draining has not shown positive results in maternal as well as fetal outcomes and is not recommended as a first choice of treatment. To the best of our knowledge, there are no case reports of endoscopic third ventriculostomy for hemangioblastoma during pregnancy.

Table 1:: Summary of direct surgical treatment of symptomatic hemangioblastomas during pregnancy as reported in the past literature.

Based on the previous literature, six cases were reported with brainstem compression and three cases were reported without brainstem compression [Table 1]. There was no difference in treatment strategy between patients with and without brainstem compression. Further case reports are needed, and at this point, it is not considered to be an indicator of decision-making.

Overall, these reports suggest that direct surgery is a better option, especially in early pregnancy. Repeated general anesthesia and shunt-related complications during pregnancy can both be avoided. Moreover, in patients who present after 30–32 weeks of gestation, as in this case, combined of cesarean section and surgical resection of the tumor can be planned if fetal lung maturity is confirmed.[17,18] Once obstetric criteria are met, an emergency cesarean section with epidural anesthesia should be employed to avoid prolonged labor and increased cerebrospinal fluid pressure.[16] A cesarean delivery should be preferred if there are any signs of increased intracranial pressure.[12] A multidisciplinary discussion between neurosurgeons, anesthetists, and obstetricians is necessary before surgery.[11] In this case, intracranial hypertension due to cerebellar hemangioblastoma in the mother was observed, and the delayed venous return associated with pregnancy contributed to the intracranial hypertension was considered. Since there was a possibility that the tumor resection would not improve the intracranial pressure quickly, an emergency cesarean section was chosen after confirming fetal lung maturity.

Prognosis

The previous publications reported that most pregnant patients with hemangioblastoma during pregnancy experienced an uneventful gestational course [Table 1]. In our literature review of perinatal case reports, there were no maternal deaths due to neurological deterioration, and both maternal and fetal outcomes were quite favorable; regarding fetal prognoses, only one pregnancy ended in spontaneous miscarriage.[12] Other neonates, including preterm infants, were in good condition with no congenital malformations. Thus, reported maternal and fetal outcomes have been good in the past literature. Unfortunately, there are few case reports on the difference in outcome between early term delivery and full-term delivery in terms of fetal prognosis. Further case accumulation and investigation are needed.