- Email: [email protected]
Pituitary apoplexy and inappropriate ADH secretion
260 Agrawal and Mahapatra
24. Mokri B, Sundt Jr TM, Houser OW, Piepgras DG. Spontaneous dissection of the cervical internal carotid artery. Ann Neurol 1986; 19: 126–138. 25. Lucas C, Moulin T, Deplanque D, Tatu L, Chavot D. Stroke patterns of internal carotid artery dissection in 40 patients. Stroke 1998; 29: 2646–2648. 26. Sturzenegger M. Spontaneous internal carotid artery dissection: early diagnosis and management in 44 patients. J Neurol 1995; 242: 231–238. 27. Macaya C, Serruys PW, Ruygrok P, Suryapranata H, Mast G, Klugmann S, Urban P, den Heijer P, Koch K, Simon R, Morice MC, Crean P, Bonnier H, Wijns W, Danchin N, Bourdonnec C, Morel MA. Continued benefit of coronary stenting versus balloon angioplasty: one-year clinical follow-up of Benestent trial. Benestent Study Group. J Am Coll Cardiol 1996; 27: 255–261. 28. Yadav JS, Roubin GS, King P, Iyer S, Vitek J. Angioplasty and stenting for restenosis after carotid endarterectomy. Initial experience. Stroke 1996; 27: 2075–2079. 29. Dorros G, Cohn JM, Palmer LE. Stent deployment resolves a petrous carotid artery angioplasty dissection. AJNR 1998; 19: 392–394. 30. Matsuura JH, Rosenthal D, Jerius H, Clark MD, Owens DS. Traumatic carotid artery dissection and pseudoaneurysm treated with endovascular coils and stent. J Endovasc Surg 1997; 4: 339–343.
Received 31 January 2002 Accepted 18 March 2002 Correspondence to: A.K. Mahapatra, Department of Neurosurgery, CN Centre, All India Institute of Medical Sciences, New Delhi 110029, India. Tel.: +91-11-6594577; Fax: +91-11-6862663; E-mail: [email protected]
INTRODUCTION Pituitary adenomas may present clinically for the first time as pituitary apoplexy, in which patients may have a sudden onset of headache, often accompanied by vomiting, meningism and a varying degree of visual deterioration. Management modalities have covered the spectrum from emergent surgery to conservative management. We report a case of pituitary apoplexy presenting with SIADH and visual deterioration, where the vision became normal bilaterally following surgery. The electrolyte and water disturbances in this patient, as well as the need for correct initial diagnosis and early surgery are highlighted. CASE REPORT
Pituitary apoplexy and inappropriate ADH secretion Deepak Agrawal, A.K. Mahapatra Department of Neurosurgery, CN Centre, All India Institute of Medical Sciences, New Delhi, India
Summary Pituitary apoplexy may be associated with visual deterioration that may be severe in some cases. Misdiagnosis of this condition is not uncommon, resulting in delayed treatment, which may adversely affect the outcome and visual prognosis. We present a case of pituitary apoplexy, who presented with features of syndrome of inappropriate anti-diuretic hormone secretion (SIADH) and monocular blindness. He had remarkable improvement to normal vision along with normalisation of serum sodium following emergency trans-sphenoidal surgery. c 2003 Elsevier Science Ltd. All rights reserved.
Journal of Clinical Neuroscience (2003) 10(2), 260–261 0967-5868/03/$ - see front matter ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00002-X
Keywords: pituitary apoplexy, trans-sphenoidal surgery, good visual outcome, SIADH
A 46 year old male presented with a history of sudden onset of headache and vomiting followed by bilateral visual deterioration and altered sensorium. He had been recently diagnosed as hypertensive and diabetic, but was not taking any treatment for the same. He was admitted to the medical ward of another hospital and was referred to our department after NCCT of head showed features of pituitary tumour with bleed. The patient presented to us 6 days after the initial episode. On examination the patient was disoriented. Visual acuity test revealed complete blindness in the left eye and perception of light present only in right nasal quadrant. Fundi showed bilateral primary optic atrophy. Ocular movements were normal except for a right sixth nerve paresis. The remainder of the neurological examination was normal. Magnetic resonance imaging (MRI) scan of the brain revealed a large pituitary adenoma extending up to the floor of the third ventricle which contained multiple hypodense areas within it, suggestive of pituitary apoplexy (Figs. 1a and b). Laboratory investigations revealed severe hyponatremia with initial sodium of 110 meq/L. Urinary specific gravity was 1010. However, as he had polyuria and urinary spot sodium was 33 meq/ L. A diagnosis of SIADH was made. The hyponatremia was corrected by fluid restriction and slow infusion of hypertonic (3%) saline. A hormone profile showed normal thyroid status and serum growth hormone. Serum prolactin and serum cortisol were also within normal limits.
Fig. 1
Journal of Clinical Neuroscience (2003) 10(2)
ª 2003 Elsevier Science Ltd. All rights reserved.
Pituitary apoplexy and ADH secretion
After correction for hyponatremia the patient underwent an emergency decompression of the pituitary adenoma, via sublabial trans-sphenoidal route. The patient had improvement of vision in the immediate post-operative period and clinical progress continued during hospital stay. Visual acuity tested on post-operative day 5 was found to be 6/6 bilaterally, though bitemporal field cuts persisted. At follow-up 2 months after admission the patient was asymptomatic and the visual field charting revealed a significant improvement in temporal field cuts.
261
however, decompression undertaken in partially blind eyes even months after the injury may have gratifying results.20 Although these findings are not strictly comparable in view of different aetiologies, we believe that the fundamental pathology, the sudden compression of the optic nerves with resulting ischaemia, remains the same in a large majority of the cases, traumatic or otherwise.
CONCLUSIONS DISCUSSION Pituitary apoplexy is a term used to describe acute infarction of the pituitary along with haemorrhage in pre-existing pituitary tumours.1 It has also been used to describe sudden infarction of nontumourous pituitary glands.2 Clinically, pituitary apoplexy manifests as sudden onset headache, often associated with vomiting and followed by visual deterioration and ophthalmoplegia.1 Bleibtreu in 1905,3 described this condition for the first time in an acromegalic man. Since then, though more than 200 cases of pituitary apoplexy have been reported in the literature, the aetiopathogenesis is still far from clear. Brougham et al.1 hypothesized the possibility of a sudden spurt in tumour growth so that it outstripped its blood supply. Microvascular abnormalities have also been demonstrated in pituitary adenomas, but their role in pituitary apoplexy has not yet been clarified.4 Others have suggested precipitating factors, including trauma,5 pregnancy,6 nasal catarrh,7 angiography6 and thrombocytopenia.8 Its association with diabetes mellitus9 and hypertension10 is also well known, and is presumably due to the coexisting vascular disease in such patients.11 Our case had both the above risk factors. Although there is a wealth of information available, controversy still surrounds the management of pituitary apoplexy. Treatment protocols have included permutations and combinations of steroids,12 bromocriptine therapy, radiotherapy and surgery.13;14 Consensus is veering towards surgery as the modality of choice in view of its multifaceted benefits. Surgical decompression may not only relieve the visual deficit but also prevents a recurrent apoplectic episode and additional tumour growth,1 besides being the definitive treatment for most patients. Although hypopituitarism has been reported to develop after apoplexy,6–11–13 urgent surgical decompression may improve pituitary function.6–9 Clinically significant neurohypophyseal dysfunction is also uncommon in pituitary apoplexy,15 though diabetes insipidus15 and SIADH16;17 has been described. Our patient did not have the classical features of SIADH, as the urine specific gravity was maintained at 1010. The hyponatremia and polyuria corrected spontaneously following transsphenoidal decompressive surgery. Morinaga et al.17 have described a similar case in which there was polyuria without hypotonicity. They measured serum ADH and ANP levels in a case of pituitary apoplexy and found that although serum ADH was elevated preoperatively, the ANP level was abnormally high at 140 pg/m. In polyuria due to abnormally increased serum ANP, the osmotic pressure of the urine is maintained relatively well and may explain the clinical findings. The excellent visual outcome in our patient could be due to the unique ability of optic nerves to withstand ischaemia for prolonged periods. Previous reports have shown return of normal vision if optic nerves are decompressed within a week.12 Delay beyond this period has proved detrimental for the visual outcome. Our own experience in treating over 800 cases of traumatic optic nerve injuries has shown that early optic nerve decompression is associated with best visual outcome,18;19 ª 2003 Elsevier Science Ltd. All rights reserved.
Pituitary apoplexy still remains a much misdiagnosed entity resulting in avoidable delay in neurosurgical consultation. Patients may present with water-electrolyte abnormalities which may not fit into the rigid definitions of SIADH or diabetes insipidus. The management is also controversial in patients without marked visual loss, but consensus is emerging towards trans-sphenoidal decompression of the pituitary adenoma and optic apparatus, in cases where visual deterioration is significant. Vision may return to normal in previously blind eyes if operative decompression is undertaken within a week. Further studies are needed to elucidate the pathophysiology of injury to optic nerves and the reasons behind its ability to withstand ischaemia for a prolonged period of time.
REFERENCES 1. Brougham M, Heusner AP, Adams RD. Acute degenerative changes in adenomas of pituitary body; with special reference to pituitary apoplexy. J Neurosurg 1950; 7: 421–439. 2. Conomy JP, Feruson JH, Brodkey JS et al. Spontaneous infarction in pituitary tumors. Neurologic and therapeutic aspects. Neurology 1975; 25: 580–587. 3. Bleibtreu L. Ein Fall von akromgalie (Zerst€orung der Hypophysis durch Blutung). Much Med Wochensch 1905; 52: 2079–2080. 4. Rovit RL, Fein JM. Pituitary apoplexy: a review and reappraisal. J Neurosurg 1972; 37: 280–288. 5. Van Wagenen WP. Hemorrhage into pituitary tumor following trauma. Ann Surg 1932; 95: 625–628. 6. Nagulesparan M, Roper J. Hemorrhage into the anterior pituitary during pregnancy after induction of ovulation with clomiphene. A case report. Br J Obstet Gynaecol 1978; 85: 153–155. 7. Dawson BH, Kothandaram P. Acute massive infarction of pituitary adenomas. A study of five patients. J Neurosurg 1972; 37: 275–279. 8. Nouizadeh AR, Pitts FW. Hemorrhage into pituitary adenoma during anticoagulant therapy. JAMA 1965; 193: 623–625. 9. Lufkin EG, Reagan TJ, Doan DH et al. Acute cerebral dysfunction in diabetes ketoacidosis: survival followed by pan hypopituitarism. Metabolism 1977; 26: 363–369. 10. Sussman EB, Poro RS. Pituitary apoplexy: the role of atheromatous emboli. Stroke 1974; 5: 318–323. 11. Wakai S, Fukushima T, Teramoto A et al. Pituitary apoplexy: its incidence and clinical significance. J Neurosurg 1981; 55: 187–193. 12. Maccagnan P, Macedo CLD, Kayath MJ et al. Conservative management of pituitary apoplexy: a prospective study. J Clin Endocrinol Metab 1995; 80: 2190–2197. 13. McFadzean RM, Doyle D, Rampling R et al. Pituitary apoplexy and its effect on vision. Neurosurgery 1991; 29: 669–675. 14. Ibersold MJ, Laws ER, Scheithauer BW et al. Pituitary apoplexy treated by trans-sphenoidal surgery. J Neurosurg 1983; 58: 315–320. 15. Reid RL, Quigley ME, Yen SS. Pituitary apoplexy. A review. Arch Neurol 1985; 42: 712–719. 16. Chen CC, Wang K, Jap TS et al. Pituitary apoplexy diagnosed by magnetic resonance imaging: a case report. Chung Hua I Hsueh Tsa Chih (Taipei) 1995; 55: 180–184. 17. Morinaga K, Inoue Y, Ueda M et al. An interesting case of pituitary apoplexy showing abnormality in water-electrolyte before and after surgery. No To Shinkei 1990; 42: 907–911. 18. Mahapatra AK. Does optic nerve injury require decompression? Ind J Med Assoc 1990; 88: 822–884. 19. Mahapatra AK, Tandon DA, Bhatia R et al. Prospective study of 100 patients with optic nerve injury. Neurol India 1992; 40: 17–21. 20. Tandon DA, Thakar A, Mahapatra AK. Transethmoidal optic nerve decompression. Clin Otolaryngol 1994; 19: 98–104.
Journal of Clinical Neuroscience (2003) 10(2)
24. Mokri B, Sundt Jr TM, Houser OW, Piepgras DG. Spontaneous dissection of the cervical internal carotid artery. Ann Neurol 1986; 19: 126–138. 25. Lucas C, Moulin T, Deplanque D, Tatu L, Chavot D. Stroke patterns of internal carotid artery dissection in 40 patients. Stroke 1998; 29: 2646–2648. 26. Sturzenegger M. Spontaneous internal carotid artery dissection: early diagnosis and management in 44 patients. J Neurol 1995; 242: 231–238. 27. Macaya C, Serruys PW, Ruygrok P, Suryapranata H, Mast G, Klugmann S, Urban P, den Heijer P, Koch K, Simon R, Morice MC, Crean P, Bonnier H, Wijns W, Danchin N, Bourdonnec C, Morel MA. Continued benefit of coronary stenting versus balloon angioplasty: one-year clinical follow-up of Benestent trial. Benestent Study Group. J Am Coll Cardiol 1996; 27: 255–261. 28. Yadav JS, Roubin GS, King P, Iyer S, Vitek J. Angioplasty and stenting for restenosis after carotid endarterectomy. Initial experience. Stroke 1996; 27: 2075–2079. 29. Dorros G, Cohn JM, Palmer LE. Stent deployment resolves a petrous carotid artery angioplasty dissection. AJNR 1998; 19: 392–394. 30. Matsuura JH, Rosenthal D, Jerius H, Clark MD, Owens DS. Traumatic carotid artery dissection and pseudoaneurysm treated with endovascular coils and stent. J Endovasc Surg 1997; 4: 339–343.
Received 31 January 2002 Accepted 18 March 2002 Correspondence to: A.K. Mahapatra, Department of Neurosurgery, CN Centre, All India Institute of Medical Sciences, New Delhi 110029, India. Tel.: +91-11-6594577; Fax: +91-11-6862663; E-mail: [email protected]
INTRODUCTION Pituitary adenomas may present clinically for the first time as pituitary apoplexy, in which patients may have a sudden onset of headache, often accompanied by vomiting, meningism and a varying degree of visual deterioration. Management modalities have covered the spectrum from emergent surgery to conservative management. We report a case of pituitary apoplexy presenting with SIADH and visual deterioration, where the vision became normal bilaterally following surgery. The electrolyte and water disturbances in this patient, as well as the need for correct initial diagnosis and early surgery are highlighted. CASE REPORT
Pituitary apoplexy and inappropriate ADH secretion Deepak Agrawal, A.K. Mahapatra Department of Neurosurgery, CN Centre, All India Institute of Medical Sciences, New Delhi, India
Summary Pituitary apoplexy may be associated with visual deterioration that may be severe in some cases. Misdiagnosis of this condition is not uncommon, resulting in delayed treatment, which may adversely affect the outcome and visual prognosis. We present a case of pituitary apoplexy, who presented with features of syndrome of inappropriate anti-diuretic hormone secretion (SIADH) and monocular blindness. He had remarkable improvement to normal vision along with normalisation of serum sodium following emergency trans-sphenoidal surgery. c 2003 Elsevier Science Ltd. All rights reserved.
Journal of Clinical Neuroscience (2003) 10(2), 260–261 0967-5868/03/$ - see front matter ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00002-X
Keywords: pituitary apoplexy, trans-sphenoidal surgery, good visual outcome, SIADH
A 46 year old male presented with a history of sudden onset of headache and vomiting followed by bilateral visual deterioration and altered sensorium. He had been recently diagnosed as hypertensive and diabetic, but was not taking any treatment for the same. He was admitted to the medical ward of another hospital and was referred to our department after NCCT of head showed features of pituitary tumour with bleed. The patient presented to us 6 days after the initial episode. On examination the patient was disoriented. Visual acuity test revealed complete blindness in the left eye and perception of light present only in right nasal quadrant. Fundi showed bilateral primary optic atrophy. Ocular movements were normal except for a right sixth nerve paresis. The remainder of the neurological examination was normal. Magnetic resonance imaging (MRI) scan of the brain revealed a large pituitary adenoma extending up to the floor of the third ventricle which contained multiple hypodense areas within it, suggestive of pituitary apoplexy (Figs. 1a and b). Laboratory investigations revealed severe hyponatremia with initial sodium of 110 meq/L. Urinary specific gravity was 1010. However, as he had polyuria and urinary spot sodium was 33 meq/ L. A diagnosis of SIADH was made. The hyponatremia was corrected by fluid restriction and slow infusion of hypertonic (3%) saline. A hormone profile showed normal thyroid status and serum growth hormone. Serum prolactin and serum cortisol were also within normal limits.
Fig. 1
Journal of Clinical Neuroscience (2003) 10(2)
ª 2003 Elsevier Science Ltd. All rights reserved.
Pituitary apoplexy and ADH secretion
After correction for hyponatremia the patient underwent an emergency decompression of the pituitary adenoma, via sublabial trans-sphenoidal route. The patient had improvement of vision in the immediate post-operative period and clinical progress continued during hospital stay. Visual acuity tested on post-operative day 5 was found to be 6/6 bilaterally, though bitemporal field cuts persisted. At follow-up 2 months after admission the patient was asymptomatic and the visual field charting revealed a significant improvement in temporal field cuts.
261
however, decompression undertaken in partially blind eyes even months after the injury may have gratifying results.20 Although these findings are not strictly comparable in view of different aetiologies, we believe that the fundamental pathology, the sudden compression of the optic nerves with resulting ischaemia, remains the same in a large majority of the cases, traumatic or otherwise.
CONCLUSIONS DISCUSSION Pituitary apoplexy is a term used to describe acute infarction of the pituitary along with haemorrhage in pre-existing pituitary tumours.1 It has also been used to describe sudden infarction of nontumourous pituitary glands.2 Clinically, pituitary apoplexy manifests as sudden onset headache, often associated with vomiting and followed by visual deterioration and ophthalmoplegia.1 Bleibtreu in 1905,3 described this condition for the first time in an acromegalic man. Since then, though more than 200 cases of pituitary apoplexy have been reported in the literature, the aetiopathogenesis is still far from clear. Brougham et al.1 hypothesized the possibility of a sudden spurt in tumour growth so that it outstripped its blood supply. Microvascular abnormalities have also been demonstrated in pituitary adenomas, but their role in pituitary apoplexy has not yet been clarified.4 Others have suggested precipitating factors, including trauma,5 pregnancy,6 nasal catarrh,7 angiography6 and thrombocytopenia.8 Its association with diabetes mellitus9 and hypertension10 is also well known, and is presumably due to the coexisting vascular disease in such patients.11 Our case had both the above risk factors. Although there is a wealth of information available, controversy still surrounds the management of pituitary apoplexy. Treatment protocols have included permutations and combinations of steroids,12 bromocriptine therapy, radiotherapy and surgery.13;14 Consensus is veering towards surgery as the modality of choice in view of its multifaceted benefits. Surgical decompression may not only relieve the visual deficit but also prevents a recurrent apoplectic episode and additional tumour growth,1 besides being the definitive treatment for most patients. Although hypopituitarism has been reported to develop after apoplexy,6–11–13 urgent surgical decompression may improve pituitary function.6–9 Clinically significant neurohypophyseal dysfunction is also uncommon in pituitary apoplexy,15 though diabetes insipidus15 and SIADH16;17 has been described. Our patient did not have the classical features of SIADH, as the urine specific gravity was maintained at 1010. The hyponatremia and polyuria corrected spontaneously following transsphenoidal decompressive surgery. Morinaga et al.17 have described a similar case in which there was polyuria without hypotonicity. They measured serum ADH and ANP levels in a case of pituitary apoplexy and found that although serum ADH was elevated preoperatively, the ANP level was abnormally high at 140 pg/m. In polyuria due to abnormally increased serum ANP, the osmotic pressure of the urine is maintained relatively well and may explain the clinical findings. The excellent visual outcome in our patient could be due to the unique ability of optic nerves to withstand ischaemia for prolonged periods. Previous reports have shown return of normal vision if optic nerves are decompressed within a week.12 Delay beyond this period has proved detrimental for the visual outcome. Our own experience in treating over 800 cases of traumatic optic nerve injuries has shown that early optic nerve decompression is associated with best visual outcome,18;19 ª 2003 Elsevier Science Ltd. All rights reserved.
Pituitary apoplexy still remains a much misdiagnosed entity resulting in avoidable delay in neurosurgical consultation. Patients may present with water-electrolyte abnormalities which may not fit into the rigid definitions of SIADH or diabetes insipidus. The management is also controversial in patients without marked visual loss, but consensus is emerging towards trans-sphenoidal decompression of the pituitary adenoma and optic apparatus, in cases where visual deterioration is significant. Vision may return to normal in previously blind eyes if operative decompression is undertaken within a week. Further studies are needed to elucidate the pathophysiology of injury to optic nerves and the reasons behind its ability to withstand ischaemia for a prolonged period of time.
REFERENCES 1. Brougham M, Heusner AP, Adams RD. Acute degenerative changes in adenomas of pituitary body; with special reference to pituitary apoplexy. J Neurosurg 1950; 7: 421–439. 2. Conomy JP, Feruson JH, Brodkey JS et al. Spontaneous infarction in pituitary tumors. Neurologic and therapeutic aspects. Neurology 1975; 25: 580–587. 3. Bleibtreu L. Ein Fall von akromgalie (Zerst€orung der Hypophysis durch Blutung). Much Med Wochensch 1905; 52: 2079–2080. 4. Rovit RL, Fein JM. Pituitary apoplexy: a review and reappraisal. J Neurosurg 1972; 37: 280–288. 5. Van Wagenen WP. Hemorrhage into pituitary tumor following trauma. Ann Surg 1932; 95: 625–628. 6. Nagulesparan M, Roper J. Hemorrhage into the anterior pituitary during pregnancy after induction of ovulation with clomiphene. A case report. Br J Obstet Gynaecol 1978; 85: 153–155. 7. Dawson BH, Kothandaram P. Acute massive infarction of pituitary adenomas. A study of five patients. J Neurosurg 1972; 37: 275–279. 8. Nouizadeh AR, Pitts FW. Hemorrhage into pituitary adenoma during anticoagulant therapy. JAMA 1965; 193: 623–625. 9. Lufkin EG, Reagan TJ, Doan DH et al. Acute cerebral dysfunction in diabetes ketoacidosis: survival followed by pan hypopituitarism. Metabolism 1977; 26: 363–369. 10. Sussman EB, Poro RS. Pituitary apoplexy: the role of atheromatous emboli. Stroke 1974; 5: 318–323. 11. Wakai S, Fukushima T, Teramoto A et al. Pituitary apoplexy: its incidence and clinical significance. J Neurosurg 1981; 55: 187–193. 12. Maccagnan P, Macedo CLD, Kayath MJ et al. Conservative management of pituitary apoplexy: a prospective study. J Clin Endocrinol Metab 1995; 80: 2190–2197. 13. McFadzean RM, Doyle D, Rampling R et al. Pituitary apoplexy and its effect on vision. Neurosurgery 1991; 29: 669–675. 14. Ibersold MJ, Laws ER, Scheithauer BW et al. Pituitary apoplexy treated by trans-sphenoidal surgery. J Neurosurg 1983; 58: 315–320. 15. Reid RL, Quigley ME, Yen SS. Pituitary apoplexy. A review. Arch Neurol 1985; 42: 712–719. 16. Chen CC, Wang K, Jap TS et al. Pituitary apoplexy diagnosed by magnetic resonance imaging: a case report. Chung Hua I Hsueh Tsa Chih (Taipei) 1995; 55: 180–184. 17. Morinaga K, Inoue Y, Ueda M et al. An interesting case of pituitary apoplexy showing abnormality in water-electrolyte before and after surgery. No To Shinkei 1990; 42: 907–911. 18. Mahapatra AK. Does optic nerve injury require decompression? Ind J Med Assoc 1990; 88: 822–884. 19. Mahapatra AK, Tandon DA, Bhatia R et al. Prospective study of 100 patients with optic nerve injury. Neurol India 1992; 40: 17–21. 20. Tandon DA, Thakar A, Mahapatra AK. Transethmoidal optic nerve decompression. Clin Otolaryngol 1994; 19: 98–104.
Journal of Clinical Neuroscience (2003) 10(2)