J Neurosurg 120:1309–1312, 2014 ©AANS, 2014
Conservative management of cavernous sinus cavernous hemangioma in pregnancy Case report Jessica S. Haber, B.A.,1 Kartik Kesavabhotla, B.S.,1 Malte Ottenhausen, M.D.,1 Imithri Bodhinayake, M.D.,1 Marc J. Dinkin, M.D., 2 Alan Z. Segal, M.D., 3 Young M. Lee, M.D., 4 and John A. Boockvar, M.D.1 Departments of 1Neurological Surgery, 2Ophthalmology, 3Neurology and Neuroscience, and 4Obstetrics and Gynecology, Weill Medical College of Cornell University, NewYork-Presbyterian Hospital, New York, New York Cavernous sinus cavernous hemangiomas in pregnancy are extremely rare lesions. The precise management of these lesions remains unknown. The authors present a case of a cavernous hemangioma in pregnancy, centered within the cavernous sinus that underwent postpartum involution without surgical intervention. A 34-year-old pregnant patient (gravida 1, para 0) presented to an otolaryngologist with persistent headache and left-sided facial pain and numbness in the V1 distribution. While being treated for sinusitis, her symptoms progressed to include a left-sided oculomotor palsy and abducens palsy. Magnetic resonance imaging without contrast revealed an expansile mass within the left cavernous sinus consistent with a cavernous hemangioma. The patient was evaluated by a neurosurgeon who recommended close follow-up and postpartum imaging without surgical intervention. Although the lesion enlarged during pregnancy, the patient was able to undergo an uncomplicated cesarean section at 37 weeks. All facial and ocular symptoms resolved by 9 months postpartum, and MRI showed a decrease in lesion size and reduced mass effect. The authors conclude that nonsurgical management may be a viable approach in patients who have an onset or exacerbation of symptoms associated with cavernous sinus cavernous hemangiomas during pregnancy because postpartum involution may negate the need for surgical intervention. (http://thejns.org/doi/abs/10.3171/2014.3.JNS131756)
C
Key Words • cavernous sinus • cavernous hemangioma • pregnancy • oncology
hemangiomas (CHs) are benign, slowgrowing vascular anomalies. While most CHs are intracranial, rarely they are found in extraaxial locations, including the orbit18 and the cavernous sinus (CS). Cavernous sinus cavernous hemangiomas (CSCHs) typically result in neurological morbidity related to compression of intracavernous cranial nerves.9,12,15–17 Surgery is considered the preferred method of treatment when lesion location allows safe resection. When anatomical location compromises safe resection,6,14 radiosurgery is an option. Radiosurgery has been shown to reduce the size of lesions while avoiding excessive bleeding and cranial nerve damage.6,14,21 More recently, stereotactic fractionated radiotherapy has also been effective in the treatment of orbital CHs.14 In general, there is a female predominance of CHs, with the peak age of incidence around the age of 40 avernous
Abbreviations used in this paper: CH = cavernous hemangioma; CS = cavernous sinus; VEGF = vascular endothelial growth factor.
J Neurosurg / Volume 120 / June 2014
years.13 Several case reports show that both intracerebral CHs15 and extracerebral CHs within the optic dsic,20 retina,19 or optic nerve10 are at an increased risk for hemorrhage during pregnancy, requiring intervention. However, there are no established treatment options for pregnant patients with these vascular lesions. We report a case of extracerebral CSCH that presented during pregnancy and was successfully managed without resection.
Case Report Presentation. A 34-year-old pregnant patient (gravida 1, para 0) developed persistent headache and left-sided facial pain and numbness at 21 weeks of gestation. She consulted an otolaryngologist who treated her with antibiotics and methylprednisolone for presumed sinusitis. When she developed left eye ptosis at 25 weeks of gestaThis article contains some figures that are displayed in color online but in black-and-white in the print edition.
1309
J. S. Haber et al. tion, she was referred to an outside neurologist who found ophthalmoplegia of the left eye and sensory deficit in the left V1 distribution.
Clinical Course. The patient was then referred to the emergency department of the NewYork-Presbyterian Hospital where her examination was notable for 2 mm of ptosis in the left eye, impaired adduction, elevation, and depression of the left eye consistent with a partial left oculomotor nerve palsy and an inability to abduct the left eye, consistent with a complete left abducens palsy. Magnetic resonance imaging showed an expansile mass in the left CS (Fig. 1A) presumed to be a schwanomma. Several weeks later, the patient’s left periorbital pain and numbness as well as the vertical motility of the left eye improved. However, repeat MRI showed a slight increase in lesion size and multiple serpentine T2 hyperintense structures, which together were suggestive of a CSCH (Fig. 1B). A cesarean section was recommended to reduce the risk of enlargement and hemorrhage during delivery. The neurosurgery team recommended no acute surgical treatment given the extent of the lesion within the CS, close follow-up, and postpartum imaging to assess for possible spontaneous involution. At 30 weeks of gestation the patient presented with progressive left orbital pressure, lid edema, worsened ptosis to 5 mm of the left eye and a new 2-mm anisocoria with mydriasis of the left eye. Repeat MRI revealed a significant enlargement of the lesion with extension to the orbital apex (Fig. 1C). There was severe compression of the left internal carotid artery and increased impingement
of the left optic nerve. The patient was offered the option of immediate delivery, but she declined. A cesarean section was scheduled for 37 weeks. At 35 weeks of gestation, the patient was referred to a neuro-ophthalmologist for declining left visual acuity. Neuro-ophthalmic examination confirmed a complete left abducens palsy and partial left oculomotor palsy, including a mild adduction defect, mild ptosis and mydriasis, worse in light. However, there was no relative afferent pupillary defect. Visual acuity in the left eye improved with a +2 lens, consistent with blurry vision from a lens accommodation paresis associated with the oculomotor palsy rather than from optic nerve compression (Fig. 2). Postpartum Course. The patient underwent the scheduled cesarean section without complication. Two weeks postpartum, she developed complete ptosis and decreased supra- and infra-adduction of the left eye, consistent with worsened oculomotor nerve function. Furthermore, in addition to left mydriasis in the light, a relative miosis in dim light had developed, consistent with a new left Horner’s syndrome. Magnetic resonance imaging demonstrated a further increase in size of the lesion (Fig. 1D). The neurosurgery team recommended a steroid treatment course. Two weeks into this treatment, the patient had improved vision and decreased ptosis, but she continued to have diplopia with leftward gaze and left eye pressure exacerbated by recumbence. Magnetic resonance imaging showed a decrease in lesion size (2.7 × 1.8 × 1.4 cm compared with 3.4 × 2.7 × 2.0 cm) and a reduced mass effect on the left internal carotid artery. Optic nerve
Fig. 1. Axial T2-weighted FLAIR images arranged and labeled in chronological order from top left to bottom right. A: Mass in the left cavernous sinus on presentation. B: Increase in lesion size after several weeks. The lesion’s appearance is consistent with a CH. C: Significant enlargement at 30 weeks of gestation after symptoms progressed. D: Further enlargement 2 weeks postpartum associated with progression of symptoms. E: Decrease in size 4 months postpartum. F: Nine months postpartum after symptoms resolved.
1310
J Neurosurg / Volume 120 / June 2014
Conservative management of cavernous sinus cavernous hemangioma
Fig. 2. Accommodation paresis due to left oculomotor palsy with mydriasis in light. CN = cranial nerve.
compression had resolved (Fig. 1E). By approximately 4 months postpartum, the left oculomotor palsy had resolved and there was 50% left abducens function. Notably, by 9 months postpartum, the patient’s neurological examination had returned to normal, and MRI showed a significant decrease in size of the CH (Fig. 1F).
Discussion
Cavernous hemangiomas are benign vascular anomalies that contain a characteristic honeycomb network of vascular spaces lined with endothelial cells and sparse amounts of loose connective tissue.11,13,16 It is important to note that extracerebral CHs such as those within the CS, optic nerve, optic disc, or retina should be differentiated from intracerebral CHs because of existing differences in clinical behavior, imaging, and response to treatment.7 Various studies have noted a concomitance of pregnancy and the onset of symptoms associated with CH,9,13,14 but the number of cases reported during pregnancy remains small. Our patient first experienced symptoms in the 2nd trimester of her pregnancy. While the link between pregnancy and the formation of lesions remains unclear, several hypotheses concerning changes in angiogenic signals and hemodynamic parameters have been proposed. Pregnant women are known to express increased levels of both vascular endothelial growth factor5 (VEGF) and progesterone. Cavernous hemangiomas express proangiogenic Flk-1 VEGF receptors12 and progesterone receptors4 on both endothelial and smooth muscle cells. Upregulation of these two endocrine signals may be associated with the growth of these lesions. Pregnancy also brings about a number of hemodynamic changes including the expansion of plasma volume and estrogen-induced increase in vascular compliance3 either through direct interaction with vascular smooth muscle or induction of nitric oxide release by the endothelium.1,3,23 Ohata et al. described cavernous sinus hemangiomas as a “cluster of small balloons” that can change in size according to blood pressure.13 According to this model, the vasodilaJ Neurosurg / Volume 120 / June 2014
tion, increased heart rate, and hypervolumia that are part of the physiological state of pregnancy may increase the size of a CSCH as seen in this case. Surgical treatment of CH has been associated with low risk and effective symptom alleviation.2,6,13,22 However, lesions that are close to the arteries and nerves of the orbit, as in this case, are difficult to resect safely14 and can make complete removal complicated.11 Surgical alternatives such as stereotactic radiosurgery have been used successfully to treat CHs.13,21 In our pregnant patient, both surgical and radiosurgical treatment were considered lastline treatment. The biggest threat to the patient’s safety was hemorrhage from the CSCH. The morbidity from hemorrhage during pregnancy and/or labor is notable in intracerebral CHs15 as well as those within the optic disc20 and retina,19 which require surgical intervention. The rupture of a CH is less likely than rupture of a carotid-cavernous aneurysm, which has an incidence of 1.3%.8 Therefore, we felt that the best course of action would be to see whether symptoms improved postpartum, as has been reported elsewhere.17 If the CSCH ruptured, the hemorrhage would likely be contained within the dural sinus. The patient’s symptoms did not resolve soon after delivery. The CSCH enlarged during the first 2 weeks following delivery before it started to decrease in size around 4 weeks postpartum. The patient’s net gain of approximately 800 ml of fluid during the cesarean section may account for the modest increase in CSCH size seen immediately postpartum. In conclusion, nonsurgical treatment is a potential option for CSCH in pregnant women. Postpartum involution may negate the need for surgical intervention. Disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. Author contributions to the study and manuscript preparation include the following. Conception and design: Boockvar. Acquisition of data: Haber, Dinkin, Segal, Lee. Drafting the article: Haber, Kesavabhotla, Ottenhausen, Bodhinayake. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Boockvar. Study supervision: Boockvar. References 1. Austin CE: Chronic and acute effects of oestrogens on vascular contractility. J Hypertens 18:1365–1378, 2000 2. Boari N, Gagliardi F, Castellazzi P, Mortini P: Surgical treatment of orbital cavernomas: clinical and functional outcome in a series of 20 patients. Acta Neurochir (Wien) 153:491–498, 2011 3. Dhawan V, Brookes ZL, Kaufman S: Long-term effects of repeated pregnancies (multiparity) on blood pressure regulation. Cardiovasc Res 64:179–186, 2004 4. Di Tommaso L, Scarpellini F, Salvi F, Ragazzini T, Foschini MP: Progesterone receptor expression in orbital cavernous hemangiomas. Virchows Arch 436:284–288, 2000 5. Evans P, Wheeler T, Anthony F, Osmond C: Maternal serum vascular endothelial growth factor during early pregnancy. Clin Sci (Lond) 92:567–571, 1997
1311
J. S. Haber et al. 6. Fraser JF, Mass AY, Brown S, Anand VK, Schwartz TH: Transnasal endoscopic resection of a cavernous sinus hemangioma: technical note and review of the literature. Skull Base 18:309–315, 2008 7. Gonzalez LF, Lekovic GP, Eschbacher J, Coons S, Porter RW, Spetzler RF: Are cavernous sinus hemangiomas and cavernous malformations different entities? Neurosurg Focus 21(1): E6, 2006 8. International Study of Unruptured Intracranial Aneurysms Investigators: Unruptured intracranial aneurysms—risk of rupture and risks of surgical intervention. N Engl J Med 339:1725– 1733, 1998 (Erratum in N Engl J Med 340:744, 1999) 9. Kitzmann AS, Moore EJ, Salomão DR, Woog JJ: Cavernous hemangioma involving the lacrimal outflow system. Ophthal Plast Reconstr Surg 23:488–490, 2007 10. Maruoka N, Yamakawa Y, Shimauchi M: Cavernous hemangioma of the optic nerve. Case report. J Neurosurg 69:292– 294, 1988 11. Meyer FB, Lombardi D, Scheithauer B, Nichols DA: Extraaxial cavernous hemangiomas involving the dural sinuses. J Neurosurg 73:187–192, 1990 12. Nagasaka M, Naganuma H, Satoh E: Growth potential of orbital cavernous hemangioma suggested by vascular endothelial growth factor and its receptor flk-1. Neurol Med Chir (Tokyo) 47:5–10, 2007 13. Ohata K, El-Naggar A, Takami T, Morino M, El-Adawy Y, ElSheik K, et al: Efficacy of induced hypotension in the surgical treatment of large cavernous sinus cavernomas. J Neurosurg 90:702–708, 1999 14. Rootman DB, Rootman J, Gregory S, Feldman KA, Ma R: Stereotactic fractionated radiotherapy for cavernous venous malformations (hemangioma) of the orbit. Ophthal Plast Reconstr Surg 28:96–102, 2012 15. Safavi-Abbasi S, Feiz-Erfan I, Spetzler RF, Kim L, Dogan S, Porter RW, et al: Hemorrhage of cavernous malformations during pregnancy and in the peripartum period: causal or co-
1312
incidence? Case report and review of the literature. Neurosurg Focus 21(1):E12, 2006 16. Sepehrnia A, Tatagiba M, Brandis A, Samii M, Prawitz RH: Cavernous angioma of the cavernous sinus: case report. Neurosurgery 27:151–155, 1990 17. Shi J, Wang H, Hang C, Pan Y, Liu C, Zhang Z: Cavernous hemangiomas in the cavernous sinus. Case reports. Surg Neurol 52:473–479, 1999 18. Shinder R, Al-Zubidi N, Esmaeli B: Survey of orbital tumors at a comprehensive cancer center in the United States. Head Neck 33:610–614, 2011 19. Smith BT, Joseph DP: Labor-induced hemorrhage of a retinal cavernous hemangioma. Ophthalmic Surg Lasers Imaging 40:419–420, 2009 20. Vela JI, Garcia-Vilaró M, Buil JA: Haemorrhage of a cavernous haemangioma of the optic disc in pregnancy. BMJ Case Rep 2010:bcr0320102806, 2010 21. Yamamoto M, Kida Y, Fukuoka S, Iwai Y, Jokura H, Akabane A, et al: Gamma Knife radiosurgery for hemangiomas of the cavernous sinus: a seven-institute study in Japan. Clinical article. J Neurosurg 112:772–779, 2010 22. Yan J, Wu Z: Cavernous hemangioma of the orbit: analysis of 214 cases. Orbit 23:33–40, 2004 23. Zygmunt M, Herr F, Münstedt K, Lang U, Liang OD: Angiogenesis and vasculogenesis in pregnancy. Eur J Obstet Gynecol Reprod Biol 110 Suppl 1:S10–S18, 2003 Manuscript submitted August 14, 2013. Accepted March 11, 2014. Please include this information when citing this paper: published online April 11, 2014; DOI: 10.3171/2014.3.JNS131756. Address correspondence to: John A. Boockvar, M.D., Weill Cornell Medical College of Cornell University, NewYork-Presbyterian Hospital, 525 E. 68th St., Box 99, New York, NY 10065. email: [email protected].
J Neurosurg / Volume 120 / June 2014
Conservative management of cavernous sinus cavernous hemangioma in pregnancy Case report Jessica S. Haber, B.A.,1 Kartik Kesavabhotla, B.S.,1 Malte Ottenhausen, M.D.,1 Imithri Bodhinayake, M.D.,1 Marc J. Dinkin, M.D., 2 Alan Z. Segal, M.D., 3 Young M. Lee, M.D., 4 and John A. Boockvar, M.D.1 Departments of 1Neurological Surgery, 2Ophthalmology, 3Neurology and Neuroscience, and 4Obstetrics and Gynecology, Weill Medical College of Cornell University, NewYork-Presbyterian Hospital, New York, New York Cavernous sinus cavernous hemangiomas in pregnancy are extremely rare lesions. The precise management of these lesions remains unknown. The authors present a case of a cavernous hemangioma in pregnancy, centered within the cavernous sinus that underwent postpartum involution without surgical intervention. A 34-year-old pregnant patient (gravida 1, para 0) presented to an otolaryngologist with persistent headache and left-sided facial pain and numbness in the V1 distribution. While being treated for sinusitis, her symptoms progressed to include a left-sided oculomotor palsy and abducens palsy. Magnetic resonance imaging without contrast revealed an expansile mass within the left cavernous sinus consistent with a cavernous hemangioma. The patient was evaluated by a neurosurgeon who recommended close follow-up and postpartum imaging without surgical intervention. Although the lesion enlarged during pregnancy, the patient was able to undergo an uncomplicated cesarean section at 37 weeks. All facial and ocular symptoms resolved by 9 months postpartum, and MRI showed a decrease in lesion size and reduced mass effect. The authors conclude that nonsurgical management may be a viable approach in patients who have an onset or exacerbation of symptoms associated with cavernous sinus cavernous hemangiomas during pregnancy because postpartum involution may negate the need for surgical intervention. (http://thejns.org/doi/abs/10.3171/2014.3.JNS131756)
C
Key Words • cavernous sinus • cavernous hemangioma • pregnancy • oncology
hemangiomas (CHs) are benign, slowgrowing vascular anomalies. While most CHs are intracranial, rarely they are found in extraaxial locations, including the orbit18 and the cavernous sinus (CS). Cavernous sinus cavernous hemangiomas (CSCHs) typically result in neurological morbidity related to compression of intracavernous cranial nerves.9,12,15–17 Surgery is considered the preferred method of treatment when lesion location allows safe resection. When anatomical location compromises safe resection,6,14 radiosurgery is an option. Radiosurgery has been shown to reduce the size of lesions while avoiding excessive bleeding and cranial nerve damage.6,14,21 More recently, stereotactic fractionated radiotherapy has also been effective in the treatment of orbital CHs.14 In general, there is a female predominance of CHs, with the peak age of incidence around the age of 40 avernous
Abbreviations used in this paper: CH = cavernous hemangioma; CS = cavernous sinus; VEGF = vascular endothelial growth factor.
J Neurosurg / Volume 120 / June 2014
years.13 Several case reports show that both intracerebral CHs15 and extracerebral CHs within the optic dsic,20 retina,19 or optic nerve10 are at an increased risk for hemorrhage during pregnancy, requiring intervention. However, there are no established treatment options for pregnant patients with these vascular lesions. We report a case of extracerebral CSCH that presented during pregnancy and was successfully managed without resection.
Case Report Presentation. A 34-year-old pregnant patient (gravida 1, para 0) developed persistent headache and left-sided facial pain and numbness at 21 weeks of gestation. She consulted an otolaryngologist who treated her with antibiotics and methylprednisolone for presumed sinusitis. When she developed left eye ptosis at 25 weeks of gestaThis article contains some figures that are displayed in color online but in black-and-white in the print edition.
1309
J. S. Haber et al. tion, she was referred to an outside neurologist who found ophthalmoplegia of the left eye and sensory deficit in the left V1 distribution.
Clinical Course. The patient was then referred to the emergency department of the NewYork-Presbyterian Hospital where her examination was notable for 2 mm of ptosis in the left eye, impaired adduction, elevation, and depression of the left eye consistent with a partial left oculomotor nerve palsy and an inability to abduct the left eye, consistent with a complete left abducens palsy. Magnetic resonance imaging showed an expansile mass in the left CS (Fig. 1A) presumed to be a schwanomma. Several weeks later, the patient’s left periorbital pain and numbness as well as the vertical motility of the left eye improved. However, repeat MRI showed a slight increase in lesion size and multiple serpentine T2 hyperintense structures, which together were suggestive of a CSCH (Fig. 1B). A cesarean section was recommended to reduce the risk of enlargement and hemorrhage during delivery. The neurosurgery team recommended no acute surgical treatment given the extent of the lesion within the CS, close follow-up, and postpartum imaging to assess for possible spontaneous involution. At 30 weeks of gestation the patient presented with progressive left orbital pressure, lid edema, worsened ptosis to 5 mm of the left eye and a new 2-mm anisocoria with mydriasis of the left eye. Repeat MRI revealed a significant enlargement of the lesion with extension to the orbital apex (Fig. 1C). There was severe compression of the left internal carotid artery and increased impingement
of the left optic nerve. The patient was offered the option of immediate delivery, but she declined. A cesarean section was scheduled for 37 weeks. At 35 weeks of gestation, the patient was referred to a neuro-ophthalmologist for declining left visual acuity. Neuro-ophthalmic examination confirmed a complete left abducens palsy and partial left oculomotor palsy, including a mild adduction defect, mild ptosis and mydriasis, worse in light. However, there was no relative afferent pupillary defect. Visual acuity in the left eye improved with a +2 lens, consistent with blurry vision from a lens accommodation paresis associated with the oculomotor palsy rather than from optic nerve compression (Fig. 2). Postpartum Course. The patient underwent the scheduled cesarean section without complication. Two weeks postpartum, she developed complete ptosis and decreased supra- and infra-adduction of the left eye, consistent with worsened oculomotor nerve function. Furthermore, in addition to left mydriasis in the light, a relative miosis in dim light had developed, consistent with a new left Horner’s syndrome. Magnetic resonance imaging demonstrated a further increase in size of the lesion (Fig. 1D). The neurosurgery team recommended a steroid treatment course. Two weeks into this treatment, the patient had improved vision and decreased ptosis, but she continued to have diplopia with leftward gaze and left eye pressure exacerbated by recumbence. Magnetic resonance imaging showed a decrease in lesion size (2.7 × 1.8 × 1.4 cm compared with 3.4 × 2.7 × 2.0 cm) and a reduced mass effect on the left internal carotid artery. Optic nerve
Fig. 1. Axial T2-weighted FLAIR images arranged and labeled in chronological order from top left to bottom right. A: Mass in the left cavernous sinus on presentation. B: Increase in lesion size after several weeks. The lesion’s appearance is consistent with a CH. C: Significant enlargement at 30 weeks of gestation after symptoms progressed. D: Further enlargement 2 weeks postpartum associated with progression of symptoms. E: Decrease in size 4 months postpartum. F: Nine months postpartum after symptoms resolved.
1310
J Neurosurg / Volume 120 / June 2014
Conservative management of cavernous sinus cavernous hemangioma
Fig. 2. Accommodation paresis due to left oculomotor palsy with mydriasis in light. CN = cranial nerve.
compression had resolved (Fig. 1E). By approximately 4 months postpartum, the left oculomotor palsy had resolved and there was 50% left abducens function. Notably, by 9 months postpartum, the patient’s neurological examination had returned to normal, and MRI showed a significant decrease in size of the CH (Fig. 1F).
Discussion
Cavernous hemangiomas are benign vascular anomalies that contain a characteristic honeycomb network of vascular spaces lined with endothelial cells and sparse amounts of loose connective tissue.11,13,16 It is important to note that extracerebral CHs such as those within the CS, optic nerve, optic disc, or retina should be differentiated from intracerebral CHs because of existing differences in clinical behavior, imaging, and response to treatment.7 Various studies have noted a concomitance of pregnancy and the onset of symptoms associated with CH,9,13,14 but the number of cases reported during pregnancy remains small. Our patient first experienced symptoms in the 2nd trimester of her pregnancy. While the link between pregnancy and the formation of lesions remains unclear, several hypotheses concerning changes in angiogenic signals and hemodynamic parameters have been proposed. Pregnant women are known to express increased levels of both vascular endothelial growth factor5 (VEGF) and progesterone. Cavernous hemangiomas express proangiogenic Flk-1 VEGF receptors12 and progesterone receptors4 on both endothelial and smooth muscle cells. Upregulation of these two endocrine signals may be associated with the growth of these lesions. Pregnancy also brings about a number of hemodynamic changes including the expansion of plasma volume and estrogen-induced increase in vascular compliance3 either through direct interaction with vascular smooth muscle or induction of nitric oxide release by the endothelium.1,3,23 Ohata et al. described cavernous sinus hemangiomas as a “cluster of small balloons” that can change in size according to blood pressure.13 According to this model, the vasodilaJ Neurosurg / Volume 120 / June 2014
tion, increased heart rate, and hypervolumia that are part of the physiological state of pregnancy may increase the size of a CSCH as seen in this case. Surgical treatment of CH has been associated with low risk and effective symptom alleviation.2,6,13,22 However, lesions that are close to the arteries and nerves of the orbit, as in this case, are difficult to resect safely14 and can make complete removal complicated.11 Surgical alternatives such as stereotactic radiosurgery have been used successfully to treat CHs.13,21 In our pregnant patient, both surgical and radiosurgical treatment were considered lastline treatment. The biggest threat to the patient’s safety was hemorrhage from the CSCH. The morbidity from hemorrhage during pregnancy and/or labor is notable in intracerebral CHs15 as well as those within the optic disc20 and retina,19 which require surgical intervention. The rupture of a CH is less likely than rupture of a carotid-cavernous aneurysm, which has an incidence of 1.3%.8 Therefore, we felt that the best course of action would be to see whether symptoms improved postpartum, as has been reported elsewhere.17 If the CSCH ruptured, the hemorrhage would likely be contained within the dural sinus. The patient’s symptoms did not resolve soon after delivery. The CSCH enlarged during the first 2 weeks following delivery before it started to decrease in size around 4 weeks postpartum. The patient’s net gain of approximately 800 ml of fluid during the cesarean section may account for the modest increase in CSCH size seen immediately postpartum. In conclusion, nonsurgical treatment is a potential option for CSCH in pregnant women. Postpartum involution may negate the need for surgical intervention. Disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. Author contributions to the study and manuscript preparation include the following. Conception and design: Boockvar. Acquisition of data: Haber, Dinkin, Segal, Lee. Drafting the article: Haber, Kesavabhotla, Ottenhausen, Bodhinayake. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Boockvar. Study supervision: Boockvar. References 1. Austin CE: Chronic and acute effects of oestrogens on vascular contractility. J Hypertens 18:1365–1378, 2000 2. Boari N, Gagliardi F, Castellazzi P, Mortini P: Surgical treatment of orbital cavernomas: clinical and functional outcome in a series of 20 patients. Acta Neurochir (Wien) 153:491–498, 2011 3. Dhawan V, Brookes ZL, Kaufman S: Long-term effects of repeated pregnancies (multiparity) on blood pressure regulation. Cardiovasc Res 64:179–186, 2004 4. Di Tommaso L, Scarpellini F, Salvi F, Ragazzini T, Foschini MP: Progesterone receptor expression in orbital cavernous hemangiomas. Virchows Arch 436:284–288, 2000 5. Evans P, Wheeler T, Anthony F, Osmond C: Maternal serum vascular endothelial growth factor during early pregnancy. Clin Sci (Lond) 92:567–571, 1997
1311
J. S. Haber et al. 6. Fraser JF, Mass AY, Brown S, Anand VK, Schwartz TH: Transnasal endoscopic resection of a cavernous sinus hemangioma: technical note and review of the literature. Skull Base 18:309–315, 2008 7. Gonzalez LF, Lekovic GP, Eschbacher J, Coons S, Porter RW, Spetzler RF: Are cavernous sinus hemangiomas and cavernous malformations different entities? Neurosurg Focus 21(1): E6, 2006 8. International Study of Unruptured Intracranial Aneurysms Investigators: Unruptured intracranial aneurysms—risk of rupture and risks of surgical intervention. N Engl J Med 339:1725– 1733, 1998 (Erratum in N Engl J Med 340:744, 1999) 9. Kitzmann AS, Moore EJ, Salomão DR, Woog JJ: Cavernous hemangioma involving the lacrimal outflow system. Ophthal Plast Reconstr Surg 23:488–490, 2007 10. Maruoka N, Yamakawa Y, Shimauchi M: Cavernous hemangioma of the optic nerve. Case report. J Neurosurg 69:292– 294, 1988 11. Meyer FB, Lombardi D, Scheithauer B, Nichols DA: Extraaxial cavernous hemangiomas involving the dural sinuses. J Neurosurg 73:187–192, 1990 12. Nagasaka M, Naganuma H, Satoh E: Growth potential of orbital cavernous hemangioma suggested by vascular endothelial growth factor and its receptor flk-1. Neurol Med Chir (Tokyo) 47:5–10, 2007 13. Ohata K, El-Naggar A, Takami T, Morino M, El-Adawy Y, ElSheik K, et al: Efficacy of induced hypotension in the surgical treatment of large cavernous sinus cavernomas. J Neurosurg 90:702–708, 1999 14. Rootman DB, Rootman J, Gregory S, Feldman KA, Ma R: Stereotactic fractionated radiotherapy for cavernous venous malformations (hemangioma) of the orbit. Ophthal Plast Reconstr Surg 28:96–102, 2012 15. Safavi-Abbasi S, Feiz-Erfan I, Spetzler RF, Kim L, Dogan S, Porter RW, et al: Hemorrhage of cavernous malformations during pregnancy and in the peripartum period: causal or co-
1312
incidence? Case report and review of the literature. Neurosurg Focus 21(1):E12, 2006 16. Sepehrnia A, Tatagiba M, Brandis A, Samii M, Prawitz RH: Cavernous angioma of the cavernous sinus: case report. Neurosurgery 27:151–155, 1990 17. Shi J, Wang H, Hang C, Pan Y, Liu C, Zhang Z: Cavernous hemangiomas in the cavernous sinus. Case reports. Surg Neurol 52:473–479, 1999 18. Shinder R, Al-Zubidi N, Esmaeli B: Survey of orbital tumors at a comprehensive cancer center in the United States. Head Neck 33:610–614, 2011 19. Smith BT, Joseph DP: Labor-induced hemorrhage of a retinal cavernous hemangioma. Ophthalmic Surg Lasers Imaging 40:419–420, 2009 20. Vela JI, Garcia-Vilaró M, Buil JA: Haemorrhage of a cavernous haemangioma of the optic disc in pregnancy. BMJ Case Rep 2010:bcr0320102806, 2010 21. Yamamoto M, Kida Y, Fukuoka S, Iwai Y, Jokura H, Akabane A, et al: Gamma Knife radiosurgery for hemangiomas of the cavernous sinus: a seven-institute study in Japan. Clinical article. J Neurosurg 112:772–779, 2010 22. Yan J, Wu Z: Cavernous hemangioma of the orbit: analysis of 214 cases. Orbit 23:33–40, 2004 23. Zygmunt M, Herr F, Münstedt K, Lang U, Liang OD: Angiogenesis and vasculogenesis in pregnancy. Eur J Obstet Gynecol Reprod Biol 110 Suppl 1:S10–S18, 2003 Manuscript submitted August 14, 2013. Accepted March 11, 2014. Please include this information when citing this paper: published online April 11, 2014; DOI: 10.3171/2014.3.JNS131756. Address correspondence to: John A. Boockvar, M.D., Weill Cornell Medical College of Cornell University, NewYork-Presbyterian Hospital, 525 E. 68th St., Box 99, New York, NY 10065. email: [email protected].
J Neurosurg / Volume 120 / June 2014
