Pediatric Neurology 50 (2014) 648e651
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Clinical Observations
Pituitary Apoplexy in a TeenagerdCase Report Chen-Cheng Chao MD a, Chun-Ju Lin MD a, b, c, * a
Department of Ophthalmology, Changhua Christian Hospital, Changhua, Taiwan Department of Ophthalmology, China Medical University Hospital, Taichung, Taiwan c School of Medicine, China Medical University, Taichung, Taiwan b
abstract BACKGROUND: Pituitary apoplexy is a rare clinical emergency which results from hemorrhage or infarction in the pituitary gland. PATIENT: We present a 14-year-old girl with pituitary apoplexy and review the literature. RESULTS: Our patient experienced blurred vision, nausea, and headache. Her best-corrected visual acuity was 20/200 and 20/20. Confrontation test visual field testing revealed bitemporal hemianopsia. Brain imaging demonstrated a suprasellar mass. The microscopic endonasal transsphenoidal approach only found 5-10 mL brownish fluid-like material. Pathology confirmed no malignancy. Pituitary apoplexy was diagnosed. Her nausea and headache gradually improved. Six months after operation, her best-corrected visual acuity had improved to 20/30 and 20/20. CONCLUSIONS: Although pituitary apoplexy is rare in pediatric patients, prompt evaluation including detailed ophthalmic examination, biochemical evaluation, endocrine workup, and image study are very important. Keywords: bitemporal hemianopsia, hemorrhage in the pituitary gland, pituitary apoplexy, teenager
Pediatr Neurol 2014; 50: 648-651 Ó 2014 Elsevier Inc. All rights reserved.
Introduction
Pituitary apoplexy is a rare clinical emergency that results from hemorrhage or infarction in the pituitary gland.13 It may arise from pituitary tumors,4 physiological morphologic changes during pregnancy, hypotension after delivery,5 or medical treatment.6 This form of apoplexy usually occurs in individuals between 20 to 50 years of age. It affects males more often than females and is particularly rare in the pediatric population. We describe a teenager with pituitary apoplexy without pituitary tumor. Patient description A 14-year-old girl experienced blurred vision in the right eye for two weeks accompanied by nausea and headache. She gained The authors report no financial conflict of interest in the subject matter in the manuscript. The authors do not have commercial or proprietary interests related to a relevant product or company. The material was presented at the 28th Congress of the Asia-Pacific Academy of Ophthalmology (APAO) Congress, January 17- 20, 2013, Hyderabad, India.
Article History: Received November 21, 2013; Accepted in final form February 10, 2014 * Communications should be addressed to: Dr. Chun-Ju Lin; Department of Ophthalmology; 2 Yuh-Der Road; Taichung City 40447, Taiwan. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2014.02.004
approximately 7 kg of weight within 4 days, and polymenorrhea was also noted. Her best-corrected visual acuity was 20/200 in the right eye and 20/20 in the left eye. No elevated intraocular pressure, relative afferent pupillary defect, or disc swelling was observed. Confrontation test revealed bitemporal hemianopsia. A pituitary gland tumor was suspected. Brain magnetic resonance imaging (MRI) revealed a sellar-suprasellar mass, which appeared as a heterogeneous hypo- and isointense signal on T1-weighted images (Fig 1) and hyperintense contents on T2-weighted images (Fig 2). Brain computed tomography (CT) revealed a 1.5 1.8 1.8 cm sellarsuprasellar mass with heterogeneous isointense signal (Fig 3). Although craniopharyngioma was suspected initially, results from laboratory examinations including free T4, prolactin, human chorionic gonadotropin, growth hormone, cortisol, a-fetoprotein, and insulin-like growth factor 1 were within normal limits. Therefore, a neurosurgical consultation was sought. Endoscopic transsphenoidal approach of pituitary tumor resection under navigation assistance was performed but only 5-10 mL brownish fluid-like material was found. Pathology confirmed the absence of malignant cells. Pituitary hemorrhage with apoplexy was diagnosed. Her nausea and headache improved postoperatively. Six months after surgery, her best-corrected visual acuity improved to 20/30 and 20/20.
Discussion
Pituitary apoplexy is potentially life threatening because of acute ischemic infarction or hemorrhage of the pituitary gland.1,4 Sheehan,7 in 1938, first described this syndrome in
C.-C. Chao, C.-J. Lin / Pediatric Neurology 50 (2014) 648e651
649
FIGURE 1. Brain magnetic resonance imaging revealed a sellar-suprasellar mass (arrows) presenting as heterogeneous hypo- and isointense signal on T2-weighted image.
obstetric cases. Brougham,8 in 1950, coined the term “pituitary apoplexy.” It may present as severe headache, visual deterioration, ophthalmoplegia, partial or complete pituitary failure, with or without altered consciousness.2 Pituitary apoplexy is often misdiagnosed before surgery, and the condition may range from asymptomatic to critical when presenting with visual loss and subarachnoid hemorrhage. Most cases of pituitary apoplexy present in individuals in their 50s and 60s with a slight male predominance.9 Pituitary apoplexy is rare in the pediatric population, mostly happening in individuals with invasive pituitary adenomas.4 Our patient, however, is a 14-year-old teenager, and no pituitary tumor was found. Ranabir and Baruah reported that nearly 80% of patients with pituitary apoplexy have a deficiency of one or more anterior pituitary hormones at presentation, most
commonly adrenocorticotropic hormone deficiency.1 Other abnormalities include growth hormone deficit, hypothyroidism, and hypogonadotropic hypogonadism. Our patient’s laboratory examinations were normal, including free T4, prolactin, human chorionic gonadotropin, growth hormone, cortisol, a-fetoprotein, and insulin-like growth factor 1. Timely surgical intervention was considered to prevent the possible deficiency of pituitary hormones. Typical manifestations of childhood craniopharyngioma are headache, visual impairment, polyuria and/or polydypsia, growth retardation, puberty development disturbances, and significant weight gain.10 Our patient presented with blurred vision, headache, and weight gain of about 7 kg. Moreover, given that pituitary apoplexy is rare in teenagers, a craniopharyngioma was suspected initially.
FIGURE 2. Brain magnetic resonance imaging depicts a sellar-suprasellar heterogeneous hyperintense mass (arrows) on the T1-weighted sequences.
650
C.-C. Chao, C.-J. Lin / Pediatric Neurology 50 (2014) 648e651
FIGURE 3. Brain computed tomography depicts an isodense 1.8-cm sellar-suprasellar mass (arrows).
A recent hemorrhage can appear as a single or multiple hyperdense lesions with little or no contrast enhancement on CT. Lesion detection is better with MRI than with CT in the subacute and chronic stages.5 T1-weighted MRI images are also more useful than CT both for tumoral volume determination and detection of intratumoral hemorrhage.11 T1- and T2-weighted images may demonstrate hypo- or isointense lesions in the first 7 days of acute attack. In the subacute stage, MRI may reveal marginal signal reinforcement. In the chronic stage, overall increase on T1- and T2-signal images may be noted. Pituitary apoplexy may present with different magnetic resonance features, including hemorrhagic and nonhemorrhagic characteristics on T1-weighted images. The T2-weighted studies demonstrated areas of low- and high-signal intensities in keeping with the presence of blood degradation contents.12 Brain MRI of our case presented as heterogeneous signal both on T1- and T2-weighted images revealing different stages of hemorrhage. Surgical management is a consideration in individuals with deteriorating consciousness, severely reduced visual acuity, and visual field defect.13-15 Turgut et al.2 reported no difference in visual recovery between patients who underwent surgery within 3 days of symptom onset versus those who underwent surgery within 4-7 days of the apoplexy. And there was also no significant difference in the visual recovery in patients undergoing surgery within the first day of the apoplexy compared with those undergoing surgery within 27 days of apoplectic event. However, Randeva et al.15 reported that early surgical decompression within 8 days could lead to significant improvement. As for surgical method, endonasal transsphenoidal resection has been advocated as a treatment option for the pituitary adenomas in children.16 Despite the technical challenges associated with this approach in the pediatric population, these tumors can be effectively managed with minimal morbidity.16 Although our patient underwent surgery 2 weeks after the onset of signs, her vision fortunately improved postoperatively. Although pituitary apoplexy is uncommon in pediatric patients, prompt evaluation, including detailed
ophthalmic examination, biochemical evaluation, endocrine testing, and imaging studies, is important. Early surgical intervention is indicated in individuals with deteriorating consciousness and severe visual loss or visual field defect. Although significant improvement of visual acuity and visual field is possible after timely and successful surgery, additional studies with more patients and a longer follow-up period are warranted.
References 1. Ranabir S, Barush MP. Pituitary apoplexy. Indian J Endocrinol Metab. 2011;3(15 Suppl):S188-S196. 2. Turgut M, Özsunar Y, Bas¸ak S. Pituitary apoplexy: an overview of 186 cases published during the last century. Acta Neurochir. 2010; 152:749-761. 3. Vicente A, Lecumberri B, Gálvez MA, en nombre del Grupo de Trabajo de Neuroendocrinología. Clinical practice guideline for the diagnosis and treatment of pituitary apoplexy. Endocrinol Nutr. 2013;60(10):582.e1-582.e12. 4. Mehrazin M. Pituitary tumors in children: clinical analysis of 21 cases. Childs Nerv Syst. 2007;23:391-398. 5. Alvis JS, Hicks RJ. Pregnancy-induced acute neurologic emergencies and neurologic conditions encountered in pregnancy. Semin Ultrasound CT MR. 2012;33:46-54. 6. Hands KE, Alvarez A, Bruder JM. Gonadotropin-releasing hormone agonist-induced pituitary apoplexy in treatment of prostate cancer: case report and review of literature. Endocr Pract. 2007;13: 642-646. 7. Sheehan HL. Postpartum necrosis of the anterior pituitary. J Pathol Bact. 1937;45:189-214. 8. Brougham M, Heusner AP, Adams RD. Acute degenerative changes in adenomas of the pituitary bodyewith special reference to pituitary apoplexy. J Neurosurg. 1950;7:421-439. 9. Gsponer J, De Tribolet N, Déruaz JP, Janzer R, Uské A, Mirimanoff RO, et al. Diagnosis, treatment, and outcome of pituitary tumors and other abnormal intrasellar masses. Retrospective analysis of 353 patients. Medicine (Baltimore). 1999;78: 236-269. 10. Müller HL. Childhood craniopharyngioma. Pituitary. 2013;16: 56-67. 11. L’Huillier F, Combes C, Martin N, Leclerc X, Pruvo JP, Gaston A. MRI in the diagnosis of so-called pituitary apoplexy: seven cases. J Neuroradiol. 1989;16:221-237. [Article in English, French]. 12. Piotin M, Tampieri D, Rüfenacht DA, et al. The various MRI patterns of pituitary apoplexy. Eur Radiol. 1999;9:918-923.
C.-C. Chao, C.-J. Lin / Pediatric Neurology 50 (2014) 648e651 13. Ayuk J, McGregor EJ, Mitchell RD, Gittoes NJL. Acute management of pituitary apoplexydsurgery or conservative management? Clin Endocrinol (Oxf). 2004;61:747-752. 14. Sally Murad-Kejbou, Eric Eggenberger. Pituitary apoplexy: evaluation, management, and prognosis. Current Opinion in Ophthalmology. 2009;20:456-461.
651
15. Randeva HS, Schoebel J, Byrne J, Esiri M, Adams CB, Wass JA. Classical pituitary apoplexy: clinical features, management and outcome. Clin Endocrinol (Oxf). 1999;51:181-188. 16. Tarapore PE, Sughrue ME, Blevins L, Auguste KI, Gupta N, Kunwar S. Microscopic endonasal transsphenoidal pituitary adenomectomy in the pediatric population. J Neurosurg Pediatr. 2011;7:501-509.
The history of medicine is, in fact, the history of humanity itself, with its ups and downs, its brave aspirations after truth and finality, and its pathetic failures. The subject may be treated variously as a pageant, an array of books, a procession of characters, a succession of theories, an exposition of human ineptitudes, or as the very bone and marrow of human culture. Fielding H. Garrison
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Clinical Observations
Pituitary Apoplexy in a TeenagerdCase Report Chen-Cheng Chao MD a, Chun-Ju Lin MD a, b, c, * a
Department of Ophthalmology, Changhua Christian Hospital, Changhua, Taiwan Department of Ophthalmology, China Medical University Hospital, Taichung, Taiwan c School of Medicine, China Medical University, Taichung, Taiwan b
abstract BACKGROUND: Pituitary apoplexy is a rare clinical emergency which results from hemorrhage or infarction in the pituitary gland. PATIENT: We present a 14-year-old girl with pituitary apoplexy and review the literature. RESULTS: Our patient experienced blurred vision, nausea, and headache. Her best-corrected visual acuity was 20/200 and 20/20. Confrontation test visual field testing revealed bitemporal hemianopsia. Brain imaging demonstrated a suprasellar mass. The microscopic endonasal transsphenoidal approach only found 5-10 mL brownish fluid-like material. Pathology confirmed no malignancy. Pituitary apoplexy was diagnosed. Her nausea and headache gradually improved. Six months after operation, her best-corrected visual acuity had improved to 20/30 and 20/20. CONCLUSIONS: Although pituitary apoplexy is rare in pediatric patients, prompt evaluation including detailed ophthalmic examination, biochemical evaluation, endocrine workup, and image study are very important. Keywords: bitemporal hemianopsia, hemorrhage in the pituitary gland, pituitary apoplexy, teenager
Pediatr Neurol 2014; 50: 648-651 Ó 2014 Elsevier Inc. All rights reserved.
Introduction
Pituitary apoplexy is a rare clinical emergency that results from hemorrhage or infarction in the pituitary gland.13 It may arise from pituitary tumors,4 physiological morphologic changes during pregnancy, hypotension after delivery,5 or medical treatment.6 This form of apoplexy usually occurs in individuals between 20 to 50 years of age. It affects males more often than females and is particularly rare in the pediatric population. We describe a teenager with pituitary apoplexy without pituitary tumor. Patient description A 14-year-old girl experienced blurred vision in the right eye for two weeks accompanied by nausea and headache. She gained The authors report no financial conflict of interest in the subject matter in the manuscript. The authors do not have commercial or proprietary interests related to a relevant product or company. The material was presented at the 28th Congress of the Asia-Pacific Academy of Ophthalmology (APAO) Congress, January 17- 20, 2013, Hyderabad, India.
Article History: Received November 21, 2013; Accepted in final form February 10, 2014 * Communications should be addressed to: Dr. Chun-Ju Lin; Department of Ophthalmology; 2 Yuh-Der Road; Taichung City 40447, Taiwan. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2014.02.004
approximately 7 kg of weight within 4 days, and polymenorrhea was also noted. Her best-corrected visual acuity was 20/200 in the right eye and 20/20 in the left eye. No elevated intraocular pressure, relative afferent pupillary defect, or disc swelling was observed. Confrontation test revealed bitemporal hemianopsia. A pituitary gland tumor was suspected. Brain magnetic resonance imaging (MRI) revealed a sellar-suprasellar mass, which appeared as a heterogeneous hypo- and isointense signal on T1-weighted images (Fig 1) and hyperintense contents on T2-weighted images (Fig 2). Brain computed tomography (CT) revealed a 1.5 1.8 1.8 cm sellarsuprasellar mass with heterogeneous isointense signal (Fig 3). Although craniopharyngioma was suspected initially, results from laboratory examinations including free T4, prolactin, human chorionic gonadotropin, growth hormone, cortisol, a-fetoprotein, and insulin-like growth factor 1 were within normal limits. Therefore, a neurosurgical consultation was sought. Endoscopic transsphenoidal approach of pituitary tumor resection under navigation assistance was performed but only 5-10 mL brownish fluid-like material was found. Pathology confirmed the absence of malignant cells. Pituitary hemorrhage with apoplexy was diagnosed. Her nausea and headache improved postoperatively. Six months after surgery, her best-corrected visual acuity improved to 20/30 and 20/20.
Discussion
Pituitary apoplexy is potentially life threatening because of acute ischemic infarction or hemorrhage of the pituitary gland.1,4 Sheehan,7 in 1938, first described this syndrome in
C.-C. Chao, C.-J. Lin / Pediatric Neurology 50 (2014) 648e651
649
FIGURE 1. Brain magnetic resonance imaging revealed a sellar-suprasellar mass (arrows) presenting as heterogeneous hypo- and isointense signal on T2-weighted image.
obstetric cases. Brougham,8 in 1950, coined the term “pituitary apoplexy.” It may present as severe headache, visual deterioration, ophthalmoplegia, partial or complete pituitary failure, with or without altered consciousness.2 Pituitary apoplexy is often misdiagnosed before surgery, and the condition may range from asymptomatic to critical when presenting with visual loss and subarachnoid hemorrhage. Most cases of pituitary apoplexy present in individuals in their 50s and 60s with a slight male predominance.9 Pituitary apoplexy is rare in the pediatric population, mostly happening in individuals with invasive pituitary adenomas.4 Our patient, however, is a 14-year-old teenager, and no pituitary tumor was found. Ranabir and Baruah reported that nearly 80% of patients with pituitary apoplexy have a deficiency of one or more anterior pituitary hormones at presentation, most
commonly adrenocorticotropic hormone deficiency.1 Other abnormalities include growth hormone deficit, hypothyroidism, and hypogonadotropic hypogonadism. Our patient’s laboratory examinations were normal, including free T4, prolactin, human chorionic gonadotropin, growth hormone, cortisol, a-fetoprotein, and insulin-like growth factor 1. Timely surgical intervention was considered to prevent the possible deficiency of pituitary hormones. Typical manifestations of childhood craniopharyngioma are headache, visual impairment, polyuria and/or polydypsia, growth retardation, puberty development disturbances, and significant weight gain.10 Our patient presented with blurred vision, headache, and weight gain of about 7 kg. Moreover, given that pituitary apoplexy is rare in teenagers, a craniopharyngioma was suspected initially.
FIGURE 2. Brain magnetic resonance imaging depicts a sellar-suprasellar heterogeneous hyperintense mass (arrows) on the T1-weighted sequences.
650
C.-C. Chao, C.-J. Lin / Pediatric Neurology 50 (2014) 648e651
FIGURE 3. Brain computed tomography depicts an isodense 1.8-cm sellar-suprasellar mass (arrows).
A recent hemorrhage can appear as a single or multiple hyperdense lesions with little or no contrast enhancement on CT. Lesion detection is better with MRI than with CT in the subacute and chronic stages.5 T1-weighted MRI images are also more useful than CT both for tumoral volume determination and detection of intratumoral hemorrhage.11 T1- and T2-weighted images may demonstrate hypo- or isointense lesions in the first 7 days of acute attack. In the subacute stage, MRI may reveal marginal signal reinforcement. In the chronic stage, overall increase on T1- and T2-signal images may be noted. Pituitary apoplexy may present with different magnetic resonance features, including hemorrhagic and nonhemorrhagic characteristics on T1-weighted images. The T2-weighted studies demonstrated areas of low- and high-signal intensities in keeping with the presence of blood degradation contents.12 Brain MRI of our case presented as heterogeneous signal both on T1- and T2-weighted images revealing different stages of hemorrhage. Surgical management is a consideration in individuals with deteriorating consciousness, severely reduced visual acuity, and visual field defect.13-15 Turgut et al.2 reported no difference in visual recovery between patients who underwent surgery within 3 days of symptom onset versus those who underwent surgery within 4-7 days of the apoplexy. And there was also no significant difference in the visual recovery in patients undergoing surgery within the first day of the apoplexy compared with those undergoing surgery within 27 days of apoplectic event. However, Randeva et al.15 reported that early surgical decompression within 8 days could lead to significant improvement. As for surgical method, endonasal transsphenoidal resection has been advocated as a treatment option for the pituitary adenomas in children.16 Despite the technical challenges associated with this approach in the pediatric population, these tumors can be effectively managed with minimal morbidity.16 Although our patient underwent surgery 2 weeks after the onset of signs, her vision fortunately improved postoperatively. Although pituitary apoplexy is uncommon in pediatric patients, prompt evaluation, including detailed
ophthalmic examination, biochemical evaluation, endocrine testing, and imaging studies, is important. Early surgical intervention is indicated in individuals with deteriorating consciousness and severe visual loss or visual field defect. Although significant improvement of visual acuity and visual field is possible after timely and successful surgery, additional studies with more patients and a longer follow-up period are warranted.
References 1. Ranabir S, Barush MP. Pituitary apoplexy. Indian J Endocrinol Metab. 2011;3(15 Suppl):S188-S196. 2. Turgut M, Özsunar Y, Bas¸ak S. Pituitary apoplexy: an overview of 186 cases published during the last century. Acta Neurochir. 2010; 152:749-761. 3. Vicente A, Lecumberri B, Gálvez MA, en nombre del Grupo de Trabajo de Neuroendocrinología. Clinical practice guideline for the diagnosis and treatment of pituitary apoplexy. Endocrinol Nutr. 2013;60(10):582.e1-582.e12. 4. Mehrazin M. Pituitary tumors in children: clinical analysis of 21 cases. Childs Nerv Syst. 2007;23:391-398. 5. Alvis JS, Hicks RJ. Pregnancy-induced acute neurologic emergencies and neurologic conditions encountered in pregnancy. Semin Ultrasound CT MR. 2012;33:46-54. 6. Hands KE, Alvarez A, Bruder JM. Gonadotropin-releasing hormone agonist-induced pituitary apoplexy in treatment of prostate cancer: case report and review of literature. Endocr Pract. 2007;13: 642-646. 7. Sheehan HL. Postpartum necrosis of the anterior pituitary. J Pathol Bact. 1937;45:189-214. 8. Brougham M, Heusner AP, Adams RD. Acute degenerative changes in adenomas of the pituitary bodyewith special reference to pituitary apoplexy. J Neurosurg. 1950;7:421-439. 9. Gsponer J, De Tribolet N, Déruaz JP, Janzer R, Uské A, Mirimanoff RO, et al. Diagnosis, treatment, and outcome of pituitary tumors and other abnormal intrasellar masses. Retrospective analysis of 353 patients. Medicine (Baltimore). 1999;78: 236-269. 10. Müller HL. Childhood craniopharyngioma. Pituitary. 2013;16: 56-67. 11. L’Huillier F, Combes C, Martin N, Leclerc X, Pruvo JP, Gaston A. MRI in the diagnosis of so-called pituitary apoplexy: seven cases. J Neuroradiol. 1989;16:221-237. [Article in English, French]. 12. Piotin M, Tampieri D, Rüfenacht DA, et al. The various MRI patterns of pituitary apoplexy. Eur Radiol. 1999;9:918-923.
C.-C. Chao, C.-J. Lin / Pediatric Neurology 50 (2014) 648e651 13. Ayuk J, McGregor EJ, Mitchell RD, Gittoes NJL. Acute management of pituitary apoplexydsurgery or conservative management? Clin Endocrinol (Oxf). 2004;61:747-752. 14. Sally Murad-Kejbou, Eric Eggenberger. Pituitary apoplexy: evaluation, management, and prognosis. Current Opinion in Ophthalmology. 2009;20:456-461.
651
15. Randeva HS, Schoebel J, Byrne J, Esiri M, Adams CB, Wass JA. Classical pituitary apoplexy: clinical features, management and outcome. Clin Endocrinol (Oxf). 1999;51:181-188. 16. Tarapore PE, Sughrue ME, Blevins L, Auguste KI, Gupta N, Kunwar S. Microscopic endonasal transsphenoidal pituitary adenomectomy in the pediatric population. J Neurosurg Pediatr. 2011;7:501-509.
The history of medicine is, in fact, the history of humanity itself, with its ups and downs, its brave aspirations after truth and finality, and its pathetic failures. The subject may be treated variously as a pageant, an array of books, a procession of characters, a succession of theories, an exposition of human ineptitudes, or as the very bone and marrow of human culture. Fielding H. Garrison
