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Sagging Brain Masquerading as a Pituitary Adenoma Jennie Luna, Ila Khanna, Fiona J. Cook, Eric M. Marti, and Regis G. Hoppenot Division of Endocrinology (J.L., I.K., F.J.C.) and Departments of Radiology (E.M.M.) and Neurosurgery (R.G.H.), Brody School of Medicine, East Carolina University, Greenville, North Carolina 27834
55-year-old woman diagnosed with a nonfunctioning pituitary macroadenoma underwent transsphenoidal resection due to orthostatic headache and generalized constriction of visual fields, right eye ⬎ left eye, on formal ophthalmological testing. Magnetic resonance imaging (MRI) showed interval enlargement of a pituitary mass, increasing in inferior to superior dimension from 0.9 cm to 1.3 cm in 6 months, with mass effect on the optic chiasm. Surgical pathology showed normal adenohypophyseal and neurohypophyseal architecture with no features of adenoma. Before surgery, pituitary function was normal; however, after resection, panhypopituitarism and diabetes insipidus developed. Additional findings on preoperative MRI included features characteristic of spontaneous intracranial hypotension (SIH): pachymeningeal thickening with gadolinium enhancement, flattening of the pons, and lowering of the brainstem and cerebellar tonsils, hence the term “sagging brain” (Figure 1). Immediate postoperative pituitary MRI showed intraventricular hemorrhage. Visual fields improved, and MRI performed 6 months after surgery showed improvement in the findings described. To date, the etiology for this patient’s intracranial hypotension is not known. SIH is a rare cause for persistent headaches and is often misdiagnosed (1). To date, there is no definitive etiology for this entity; however, cerebrospinal fluid leak due to trivial trauma or meningeal weakness is commonly entertained as the cause (2). Enlargement of the pituitary may occur due to engorgement of the dural and epidural venous sinuses and can be mistaken for a macroadenoma (3). Increased awareness may avoid misdiagnosis and unnecessary procedures. This is the first reported case of SIH that has resulted in a visual field deficit due to optic chiasm compression.
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Acknowledgments Address all correspondence and requests for reprints to: Jennie Luna, MD, Division of Endocrinology, Brody School of MediISSN Print 0021-972X ISSN Online 1945-7197 Printed in U.S.A. Copyright © 2014 by the Endocrine Society Received February 10, 2014. Accepted June 2, 2014. First Published Online June 20, 2014
doi: 10.1210/jc.2014-1397
Figure 1. A and B, Pituitary mass growth 0.9 cm (A) to 1.3 cm (B, long arrow). B and C, SIH findings, pachymeningeal gadolinium enhancement (B, block arrows), flattened pons (C, block arrow), lowering of brainstem and cerebellar tonsils (C, long arrow). D, Improvement of sagging brain 6 months after surgery.
cine, East Carolina University, 3E-129, 600 Moye Boulevard, Greenville, NC 27834. E-mail: [email protected]. Disclosure Summary: The authors do not have any conflicts of interest that are relevant to the subject matter in this manuscript.
References 1. Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol. 2003;60:1713–1718. 2. Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache. 2013;53:1034 –1053. 3. Mokri B, Atkinson JL. False pituitary tumor in CSF leaks. Neurology. 2000;55:573–575. Abbreviations: MRI, magnetic resonance imaging; SIH, spontaneous intracranial hypotension.
J Clin Endocrinol Metab, September 2014, 99(9):3043
jcem.endojournals.org
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 14 January 2015. at 03:59 For personal use only. No other uses without permission. . All rights reserved.
3043
i n
F E A T U R E
E n d o c r i n o l o g y
Sagging Brain Masquerading as a Pituitary Adenoma Jennie Luna, Ila Khanna, Fiona J. Cook, Eric M. Marti, and Regis G. Hoppenot Division of Endocrinology (J.L., I.K., F.J.C.) and Departments of Radiology (E.M.M.) and Neurosurgery (R.G.H.), Brody School of Medicine, East Carolina University, Greenville, North Carolina 27834
55-year-old woman diagnosed with a nonfunctioning pituitary macroadenoma underwent transsphenoidal resection due to orthostatic headache and generalized constriction of visual fields, right eye ⬎ left eye, on formal ophthalmological testing. Magnetic resonance imaging (MRI) showed interval enlargement of a pituitary mass, increasing in inferior to superior dimension from 0.9 cm to 1.3 cm in 6 months, with mass effect on the optic chiasm. Surgical pathology showed normal adenohypophyseal and neurohypophyseal architecture with no features of adenoma. Before surgery, pituitary function was normal; however, after resection, panhypopituitarism and diabetes insipidus developed. Additional findings on preoperative MRI included features characteristic of spontaneous intracranial hypotension (SIH): pachymeningeal thickening with gadolinium enhancement, flattening of the pons, and lowering of the brainstem and cerebellar tonsils, hence the term “sagging brain” (Figure 1). Immediate postoperative pituitary MRI showed intraventricular hemorrhage. Visual fields improved, and MRI performed 6 months after surgery showed improvement in the findings described. To date, the etiology for this patient’s intracranial hypotension is not known. SIH is a rare cause for persistent headaches and is often misdiagnosed (1). To date, there is no definitive etiology for this entity; however, cerebrospinal fluid leak due to trivial trauma or meningeal weakness is commonly entertained as the cause (2). Enlargement of the pituitary may occur due to engorgement of the dural and epidural venous sinuses and can be mistaken for a macroadenoma (3). Increased awareness may avoid misdiagnosis and unnecessary procedures. This is the first reported case of SIH that has resulted in a visual field deficit due to optic chiasm compression.
A
Acknowledgments Address all correspondence and requests for reprints to: Jennie Luna, MD, Division of Endocrinology, Brody School of MediISSN Print 0021-972X ISSN Online 1945-7197 Printed in U.S.A. Copyright © 2014 by the Endocrine Society Received February 10, 2014. Accepted June 2, 2014. First Published Online June 20, 2014
doi: 10.1210/jc.2014-1397
Figure 1. A and B, Pituitary mass growth 0.9 cm (A) to 1.3 cm (B, long arrow). B and C, SIH findings, pachymeningeal gadolinium enhancement (B, block arrows), flattened pons (C, block arrow), lowering of brainstem and cerebellar tonsils (C, long arrow). D, Improvement of sagging brain 6 months after surgery.
cine, East Carolina University, 3E-129, 600 Moye Boulevard, Greenville, NC 27834. E-mail: [email protected]. Disclosure Summary: The authors do not have any conflicts of interest that are relevant to the subject matter in this manuscript.
References 1. Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol. 2003;60:1713–1718. 2. Mokri B. Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache. 2013;53:1034 –1053. 3. Mokri B, Atkinson JL. False pituitary tumor in CSF leaks. Neurology. 2000;55:573–575. Abbreviations: MRI, magnetic resonance imaging; SIH, spontaneous intracranial hypotension.
J Clin Endocrinol Metab, September 2014, 99(9):3043
jcem.endojournals.org
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 14 January 2015. at 03:59 For personal use only. No other uses without permission. . All rights reserved.
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