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The neurological abnormalities and operative findingsin the transcallosal approach for large juxtasellar–ventricular craniopharyngiomas
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Journal of Clinical Neuroscience (2002) 9(2), 159–163 © 2002, Elsevier Science Ltd. All rights reserved. DOI: 10.1054/jocn.2001.0942, available online at http: //www.idealibrary.com on
Clinical study
The neurological abnormalities and operative findings in the transcallosal approach for large juxtasellar–ventricular craniopharyngiomas Han-Jung Chen MD PHD Department of Neurosurgery, Chang Gung University and Medical Center at Kaohsiung, Taiwan
Summary From February 1994 to June 1999, there were 36 patients who underwent microsurgical resection for craniopharyngiomas. Of 6 adult patients, large tumours ([4 cm) with extension from intrasellar or parasellar space to the foramen of Monro were found. There were 4 male and 2 female patients with ages from 18 to 44 years (mean 32.4 years). All of them underwent one-stage interhemispheric transcallosal total excision of tumours. This approach affords good overhead view of tumours and surrounding structures. Preoperatively, decreased visual acuity and restriction in the visual field was found in 5 patients. Studies of anterior pituitary function revealed far below the normal range in all patients. Polyuria was noted in 4 patients. All of 6 patients showed abnormally high levels of blood cholesterol and triglyceride and 4 patients were obese. Tumours with extension into the pituitary fossa were found in 2 cases. A relatively caudal location in the third Ventricle was found in case 6, who showed better preoperative visual function and no polyuria. There were no major surgical complications encountered. One patient with high cholesterol (731 mg%) died of acute pancreatitis 6 months after surgery. For large craniopharyngiomas without pre-chiasmatic extension, proper treatment could be achieved under substitution therapy and one stage transcallosal total excision of tumours with preservation of important neural structures. © 2002, Elsevier Science Ltd. All rights reserved. Keywords: Corpus callosum, craniopharyngioma, juxtasellar tumour
INTRODUCTION Since Harvey Cushing described the management of craniopharyngiomas as a ‘baffling problem to neurosurgeons’, many improvements in the surgical treatment in this tumour has been developed in addition to hormone substitution therapy, radiotherapy and chemotherapy.1–7 On the basis of improvement of radiological studies, especially magnetic resonance imaging (MRI), microdissection of anatomical structures and microsurgical techniques, radical excision can be achieved with surgical approaches.8–12 The operative findings will be presented in detail under operative microscope. Most craniopharyngiomas are involved with vital brain structures: hypothalamic–pituitary axis, visual pathway, peduncular area and limbic system.13,14 Clinically, a patient with craniopharyngioma has a different prognosis, depending on whether the important areas are involved or not.13,15 The preferred approach and the extent of surgery vary, depending on the size and involvement of the tumour and the neurosurgeon’s experience.12,16–20 For small tumours, surgical treatment will achieve a high cure rate, but for a large or giant tumour, the associated visual and endocrino-metabolic deficits are still devastating.7,15,21 We report the surgical findings on the large craniopharyngiomas and the associated neuro-endocrinological deficits.
with contrast medium. Conventional cerebral angiography or MRI angiography were performed in selected patients. For ophthalmological investigation, visual acuity and perimetry examination were performed before and 3 months after surgery. Endocrinological assessment was done in all patients including dynamic tests with adreno-corticotropic hormone, thyrotropinreleasing hormone, gonadotropin hormone, growth hormone and prolactin before and 1 week after surgery. The hormone studies were also repeated in the follow-up period. Preoperative blood chemistry including serum cholesterol and triglyceride was examined and repeated in the follow-up period. Three patients aged under 15 years were treated by pediatric neurosurgeons. For 27 patients with smaller tumours (diameter \4 cm), the pterional trans-sylvian approach was performed to excise the tumours. The surgical result was good in these 27 patients. There was no surgical mortality and no major neurological deficits encountered. Major neurological deficits were present in 6 patients including visual dysfunction, severe headache, vomiting and mental change. Radiological studies revealed the sizes of tumours were over 4 cm in diameter in all six patients. The tumours extended from the pituitary fossa to the foramen of Monro and interpeduncular space. Obesity was found in four patients. All six patients underwent craniotomy with transcallosal approach for tumour excision.
METHODS AND PATIENTS
SURGICAL TECHNIQUES FOR TUMOURS
From January 1994 to June 1999, 36 patients were admitted to our department for treatment of craniopharyngiomas. Preoperatively, all patients were evaluated by magnetic resonance imaging (MRI)
The interhemispheric, anterior transcallosal, transforaminal approach has been performed in all cases.2,24 A bicoronal skin incision with a right unilateral, para-median, 5 cm frontal craniotomy was done. The craniotomy was about 1.5 cm to the left of the superior sagittal sinus. The retraction of the brain was minimal, about 1.5 cm away from the falx and one third of the craniotomy was behind the coronary suture. A self-retaining retractor with one 3/5 inch spatula was used. Some space was usually allowed with retraction of the falx. The dissection plan of the corpus callosum was along the space between the pericallosal arteries. A small
Received 28 December 2000 Accepted 7 March 2001 Correspondence to: Han-Jung Chen MD, PhD, Department of Neurosurgery, Chang Gung Memorial Hospital at Kaohsiung, Taiwan, 123 Ta-Pei Road, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan. Tel.: ]886 7 7317123; Fax: ]886 7 7318762; E-mail: [email protected]
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callosal incision about 2 cm was made. Because of hydrocephalus, the corpus callosum was usually very thin. The septum pellucidum and the fornix should be carefully protected from injury to avoid memory impairment. The dilated foramen of Monro afforded a space enough for removal of the intraventricular portion of the tumour. The choroid plexus was usually visible from the foramen of Monro to the lateral ventricles. This structure, striothalamic and internal cerebral veins were well protected. Aspiration of a motor oil-like cystic component and piecemeal extirpation of a calcified mass were useful for the dissection of the tumour capsule away from the ventricular wall. Pieces of cottonoid were used for protection of the normal tissue. The choroidal arteries and septal, striothalamic and internal cerebral veins should be carefully protected from injury. Meanwhile, close attention was paid to the protection of the fornix, anterior commissure, pre-septal nucleus and infundibular area. A perforation through the base of the third Ventricle was observed in 6 cases; the tumours were not purely intraventricular in location. Adhesion was noted between the tumour and hypothalamic tissue, mamillary bodies and optic tract. This was one of the important points for dissection. Delicate protection of these important structures and the perforators of the posterior communicating artery was absolutely indicated. Another important point was preservation of the perforators from the basilar tip and proximal segment of the posterior cerebral arteries, surrounding venous structure and the cerebral peduncule. Transcallosal approach afforded a better overview for dissection of this area than pterional approach or the route through the lamina terminalis. RESULTS Clinical presentation The major preoperative symptoms for each patient are listed in Table 1. The duration of symptoms ranged from 3 months to years. Visual acuity deterioration and visual field restriction were found in all patients except in Case 6 who had a normal perimetry study. Headache was also one of the major complaints. All patients presented with drowsiness and unsteady gait. Preoperative neuroradiological findings MRI studies revealed marked homogenous contrast enhancement of the tumour in Case 1. In other five cases, MRI showed mixed
components and in Case 5, cystic component presented the major portion of the tumour. All tumours extended from the skull base up to the foramen of Monro. In Case 4, 5, tumours in the intrasellar or infra-diaphragmatic space were well visualized from MRI. Hydrocephalus was found in all cases. The pituitary gland and stalk were poorly demonstrated in MRI studies. Endocrinological findings Pan-hypopituitarism was observed in all patients. The endocrinological studies included gonadal axis, thyroid axis, adrenal axia, growth hormone and prolactin. The data, except prolactin in 6 patients, were abnormally low (Table 1). The body height in Case 5 was 165 cm but the secondary sexual characteristics did not develop. Irregular menstruation after menarche was found in Case 6. About 6 months before admission, hypo-menorrhea or amenorrhea was noted. Diabetes insipidus presented in 4 patients. The daily urine amount of Cases 3 and 6 was around 3000 cc without complaints of polyuria. Hyperlipemia with abnormal high levels of serum cholesterol and triglyceride was found in all patients. Four of them were obese and one (Case 3) had serum cholesterol level up to 731 mg%. Operative findings and outcome The operative findings and outcome are summarized in Table 2. In Cases 3 and 6, polyuria was not the major complaint. The operative findings revealed a small adhesion band of tumour capsule to the diaphragm (Case 3), or dorsum sellae (Case 6). The destructive effect of tumours on the infundibulo-posterior pituitary axis was probably not disastrous. Therefore, pitressin was not always necessary after surgery. Because of relatively caudal location of tumour in the skull base, the destructive effect of the tumour on the optic pathway in Case 6 was relatively mild. The visual acuity and visual field examination revealed no deficit. Endocrine dysfunction persisted after surgery and postoperative substitution therapy continued in all patients. Because of polyuria, three patients needed long-term use of pitressin. Postoperatively, they made good neurological recovery. The visual acuity and visual field gradually improved in all cases. In Cases 1, 3, 4 and 5, the temporal hemianopia recovered after a 6-month follow-up period. The higher function of the brain and memory also improved in these six cases. Cases 2 and 4 could enjoy their easy jobs, Case 5 returned to his junior college and Case 6 got
Table 1 Preoperative ophthalmological and endocrinological findings Patient No.
Sex
Age
Visual acuity
Visual field
Thyroid axis
Gonadal axis
Adrenal axis
GH
Posterior pituitary function
N (low margin)
↓
↓
↓
Polyuria
Chol: 199 TG: 430
–
↓
↓
↓
↓
Polyria
Chol: 235
]
Temporal hemianopia –
N (low margin)
↓
↓
↓
(–)
Chol: 731 TG: 350
]
No defect Temporal hemianopia Bitemporal hemianopia
↓
↓
↓
↓
Polyuria
]
N (low margin)
↓ No Secondary sexual develop ↓
↓
↓
Polyuria
Chol: 238 TG: 338 Chol: 250
↓
↓
(–)
1
M
44
R: FC L: 0.02
– Temporal hemianopia
2
F
38
R: 0.2 L: 0.5
Restriction in both sides
3
M
31
4
M
35
5
M
18
R: 02 L: hand movement R: 02 L: 0.08 R: 0.01 L: 0.01
6
F
31
R: 0.5 L: 0.1
Both sides: no restriction
Anterior pituitary function
N
Blood lipid (mg%)
Obesity
Chol: 270 TG: 232
]
[
R, right side; L, left side; N, normal, low margin: normal but close to low margin, ↓: impaired, Chol: cholesterol, TG: triglyceride.
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Transcallosal approach for large juxtasellar–ventricular craniopharyngiomas 161 Table 2 Surgical findings and outcome of six patients with big craniopharyngiomas Patient No.
Appearance
1
All solid with fibrous band and adhesion with the diaphragm and tuberculum Major portion was calcified solid mass, fibrous adhesion with the retrochiasmatic tuberculum Major part of tumor was solid mass, a small fibrous adhesion with the tuberculum and diaphragm Major portion of tumor was solid, extension into the intrasellar space Major portion was cystic component, tumor inside the whole pituitary fossa Major part of tumor was solid, fibrous adhesion with the dorsum sellae
2
3
4
5
6
Resection (tumor size, cm) Complete (4.2) Complete (4.0) Complete (5.0)
Complete (5.0) Complete (4.5 cm) Complete (5.0)
Complication
None (continuous pitressin usage at night time) None (No more pitressin now) None (No pitressin after surgery) None (continuous pitressin usage at night time) None (Continuous pitressin usage at night time) Mild left hemiparesis, pitressin was needed for 4 months
Follow-up (months)
Outcome
40
Independent living
46
Return to work
6
24
Died of acute pancreatitis and associated hepatorenal failure Independent living
15
Continuing school work
8
Independent living
a job as a clerk in a store. Case 3 enjoyed a good living after surgery. Pitressin was only temporarily necessary for him. Unfortunately, he died of acute pancreatitis 6 months after surgery. He was found to have abnormally high serum cholesterol and triglyceride.
ILLUSTRATIVE CASES Case No.4 This 35 year old man had suffered from polyuria and bilateral blurred vision for more than 3 months. He was admitted to our department in March, 1997. Review of history revealed general weakness, drowsiness and loss of libido for more than 6 months. He was the father of two children. Ophthalmological examination revealed left visual acuity 0.08 with temporal hemianopia and right visual acuity 0.2. Hormone studies, including gonadal, thyroid, adrenal axes and growth hormone were below normal range. MRI revealed a retro-chiasmatic mass with extension from the pituitary fossa up to the foramen of Monro (Fig.1). The major portion of the tumour was contrast enhanced solid tissue with a diameter of 5 cm. He underwent an uneventful craniotomy with transcallosal approach of total excision of tumour. Drainage of the cystic component and piecemeal extirpation of calcified solid mass were performed for internal decompression. The capsule of the tumour was carefully dissected away from the normal tissue, especially around the hypothalamus, optic tract and other important structures. The tip of the basilar artery and its perforators were well protected. The normal pituitary gland was replaced by the tumour mass (Fig. 2). This tumour was totally excised through the transcallosal approach in one stage. He recovered soon with full consciousness and good motor function. Hormone substitution treatment and desmopressin acetate (DDAVP, Rhône-Poulenc Rorer Pharmaceuticals, Inc., PA, USA) 0.025 cc was administered before sleep for polyuria. He returned to work. The follow-up MRI 2 years after surgery revealed no recurrence (Fig. 3). Case No.6 This 31-year-old unmarried woman has been found to have irregular menstruation since menarche. About 6 months prior to this admission, hypomenorrhea or amenorrhea was noted. The daily activities had been normal until one month before admission when intermittent headache and vomiting occurred. On admission, she was found to be very drowsy. Physical examination revealed © 2002, Elsevier Science Ltd. All rights reserved.
Fig.1 MRI (T1-weighted image with contrast enhancement) in Case 4 reveals a large heterogenous tumour with downward extension to the pitutitary fossa and upward extension to the foramen of Monro.
isocoric pupils with prompt light reflex. The visual acuity in the right was 0.5 and the left, 0.1. She wore spectacles for years and no definite vision change was noted. Perimetry demonstrated no defect in the visual field. Hormone studies showed most values were far below the normal range except prolactin (57.8 ng/ml). Besides normal perimetry, she did not complain of polyuria. The daily total urine amount was less than 3000 cc, and MRI revealed a big suprasellar irregular contrast enhanced tumour with extension to the foramen of Monro and compression to the midbrain and cerebral peduncles. Multiple cystic components were noted (Fig. 4). She underwent right frontal craniotomy with transcallosal approach in June 1999. Internal decompression by drainage of the cystic component and piecemeal extirpation of calcified solid tumour was performed through the dilated foramen of Morno. The normal hypothalamic and optic pathway were protected with cottonoid while dissecting the tumour capsule. This tumour could be separated away from the infundibulo-pituitary axis. A fibrous adhesion to the dorsum sellae was found. This adhesion band was coagulated and then divided. Compared with other large craniopharyngiomas, the anatomical location of this tumour was relatively caudal to chiasma Journal of Clinical Neuroscience (2002) 9(2), 159–163
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Fig. 4 MRI (T1-weighted image with contrast enhancement) of Case 6 reveals a large extra- and intra-ventricular tumor with compression to the cerebral peduncles.
Fig. 2 The pituitary fossa is empty after removal of tumor. The dorsum Sellae (arrow) is shown.
Fig. 3
Follow-up MRI 2 years after surgery reveals no recurrence.
and hypothalamus. This is the reason why the perimetry showed no defect and she did not complain of polyuria. This tumour showed tight adhesion with the cerebral peduncles and impaction in the interpeduncular space. Delicate dissection and preservation of all perforators from the basilar tip and the proximal segment of both posterior cerebral arteries were achieved (Fig. 5). Finally, the tumour was totally excised. Mild polyuria (daily urine amount less than 4000 cc) developed after surgery. Only mild left hemiparesis was found. This might be caused by the dissection between the tumour capsule and cerebral peduncles. She recovered gradually after a programmed rehabilitation and got work as a clerk in a store one year after surgery. Her memory showed good recovery. Journal of Clinical Neuroscience (2002) 9(2), 159–163
Fig. 5 Operative picture of Case 6 reveals the basilar artery, p1 (arrow heads) and perforators (arrows). The tumor was totally removed.
DISCUSSION Craniopharyngiomas usually affect not only the hypothalamic– pituitary axis, but also the visual pathway, frontal lobes, striothalamic areas, limbic system and caudally midbrain and cerebral peducular area.9,14,22 In more than 100 previous cases, mental disturbance, visual deficits and endocrine dysfunction affected more than half of our patients. The neurosurgeon always tries his utmost to treat this tumour by employing microsurgical techniques to separate the normal structures from the tumour, preserve the normal function and totally remove the tumour. Of course, we should consider the balance between the risk of recurrence and injury of important structures © 2002, Elsevier Science Ltd. All rights reserved.
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Transcallosal approach for large juxtasellar–ventricular craniopharyngiomas 163
such as the hypothalamus and visual pathway.6,12,13,21,23 Hormone substitution therapy available at present is a dispensable treatment for patients before and after surgery.12,14 Damage of the visual pathway by the tumour is another disastrous effect in most cases. There is no substitution therapy for this.7,12,13,22 Conventional radiotherapy or stereotactic radiosurgery cannot assure long-term preservation of visual function.6,13,22,24 For small juxtasellar or parachiasmatic tumours, the pterional trans-sylvian approach usually accomplishes complete removal of tumours. The symptoms and signs may be mild or limited. For large extra- and intraventricular tumours, the strategy of the author is total excision of the tumour, followed by appropriate substitution therapy instead of repeated surgical procedures and/or radiotherapy.1,11,12 A certain numbers of neurosurgeons choose trans-lamina terminalis approach for a large intraventricular tumours. This approach is, however, suitable for tumours in the anterior part of the third ventricle, especially for those that develop anteriorly from the line joining the anterior ridge of the foramen of Monro and cerebral aqueduct. For tumours developing to the posterior part of the third ventricle, complete excision by opening the lamina terminalis only is not possible.25–27 The disadvantages of this approach also include the possibility of infection from the frontal sinus, division of the sagittal sinus and injury of the olfactory nerves. For large craniopharyngiomas like these 6 cases, the foramen of Monro was large enough for removal of the tumour using the transcallosal, transforaminal approach. The septum pellucidum and the fornix could be carefully protected from injury to avoid memory impairment. After internal decompression, the capsule of tumours could be carefully dissected from the anterior commissure, pre-septal nucleus and infundibular area. This approach affords a good overhead view of removal of tumours inside the sella and dissection of tumours from the normal structures such as both internal carotid arteries, the posterior communicating arteries and their perforators and perforators from the P1 segment of the posterior cerebral arteries and the tip of the basilar artery. It would be difficult to remove the tumour around the area of the tip of the basilar artery using translamina terminalis approach in Case 6. Of course, the transcallosal, transforminal approach shows its limitation in removal of tumour at the pre-chiasmatic space.2,4,5,12 The destructive effects of tumours on the optic pathway are usually disastrous in the patients and were especially pronounced in the children. There is still no substitutional therapy for the damaged vision. Surgery can release the destructive effect of the tumours and preserve the residual vision or even improve the vision. A good-quality MRI can provide the surgeons with information such as size, location and attachment of the tumour.8 Operative findings prove the imaging studies. In case 6, we found the tumour capsule was attached to the dorsum sellae. The pressure or destructive effects of the tumour was probably not high on the chiasma or optic radiation, so the visual function was preserved. The anterior pituitary function of all six patients was impaired. The data about the gonadal axis, adrenal axis and growth hormone were far below the normal level. However, in the thyroid axis, it was better, in low margin of normal range for 3 patient and normal for one patient. Diabetes insipidus was found in 4 patients except Case 3 and 6. The abnormality of endocrinological function showed the correlation of location of tumour in MRI studies and operative findings. The destructive or pressure effects of the tumours on the midbrain and cerebral peduncle will increase if the tumours grow toward this area. It needs delicate microsurgical technique to separate the tumour capsule from the normal neural tissue and perforators from the basilar tip or P1 segment. In the author’s opinion, transcallosal approach provides a better overhead view for this area. There is a blind spot for dissection of the basilar tip and peripeduncular area if approach is through the lamina terminals, © 2002, Elsevier Science Ltd. All rights reserved.
and the blind spot is also for dissection of the perforators of the opposite posterior communicating artery with pterional transsylvian approach. Of course, the pure transcallosal approach has its limitation in the excision of pre-chiasmatic mass, especially tumour encasing the anterior communicating artery and its branches.1,2,9,12 Obesity and hyperlipemia is one of the major problems for patients. Because of destruction of the hypothalamus, unlimited intake will induce obesity and hyperlipemia. This may be the cause of acute pancreatitis found in Case 3. Appropriate medical therapy and diet control is indispensable for achieving a good surgical result. REFERENCES 1.
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26. 27.
Apuzzo MLJ, Levy ML, Tung H. Surgical strategies and technical methodologies in optimal management of craniopharyngioma and masses affecting the third ventricular chamber. Acta Neurochir Suppl (Wien) 1991; 53: 77–88. Apuzzo MLJ, Zee CS, Breeze RE. Anterior and mid-third ventricular lesions: a surgical overview. In: Apuzzo MLJ (ed) Surgery of the Third Ventricle. Baltimore: Williams & Wilkins, 1987: 518–520. Backlund EO. Role of stereotaxis in the management of midline cerebral lesions. In: Apuzzo MLJ (ed) Surgery of the Third Ventricle. Baltimore: Williams & Wilkins, 1987: 802–805. Baskin DS, Wilson CB. Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg 1986; 65: 22–27. Carmel PW. Craniopharyngioma. In: Wilkins RH, Rengachary SS (eds) Neurosurgery. New York: McGraw Hill, 1985: 905–916. Richmond IL, Wara WM, Wilson CB. Role of radiation therapy in the management of craniopharyngiomas in children. Neurosurgery 1980; 6: 513–517. Wara WM, Gutin PH. Radiotherapy of pineal and suprasellar tumours. In : Apuzzo MLJ (ed) Surgery of the Third Ventricle. Baltimore: Williams & Wilkins, 1987: 831–837. Pusey E, Kortman KE, Flannigan BD, Tsuruda J, Bradley WG. MR of craniopharyngiomas: tumour delineation and characterization. AJR 1987; 149: 383–388. Steno J. Microsurgical topography of craniopharyngiomas. Acta Neurochir Suppl 1985; 35: 94–100. Sweet WH. Radical surgical treatment of craniopharyngioma. Clin Neurosurg 1975; 23: 52–79. Symon L, Sprich W. Radical excision of craniopharyngioma. Results in 20 patients. J Neurosurg 1985; 62: 174–181. Yasargil MG, Curcic M, Kis M, Siegenthaler G, Teddy PJ, Roth P. Total removal of craniopharyngiomas, approaches and long-term results in 144 patients. J Neurosurg 1990; 73: 3–11. Hoffman HJ. Craniopharyngioma: the continuing controversy on management. Concepts Pediatr Neurosurg 1982; 2: 14–28. Paja M, Lucas T, Garcia-Uria J, Salome F, Barcelo B, Estrada J. Hypothalamiopituitary dysfunction in patients with craniopharyngioma. Clin Endocrinol (Oxf) 1995; 42: 467–73. Rougerie J. What can be expected from the surgical treatment of craniopharyngiomas in children. Report of 92 cases. Child’s Brain 1979; 5: 433–449. Ciric IS, Cozzens JW. Craniopharyngioma: trans-sphenoid method of approach for the virtuous only? Clin Neurosurg 1980; 27: 169–187. King TT. Removal of intraventricular craniopharyngiomas through the lamina terminalis. Acta Neurochir 1979; 45: 277–286. Long DM, Chou SN. Transcallosal removal of craniopharyngiomas within the third ventricle. J Neurosurg 1973; 39: 563–567. Patterson RH Jr, Danylevich A. Surgical removal of craniopharyngiomas by transcranial approach through the lamina terminalis and sphenoid sinus. Neurosurgery 1980; 7: 111–117. Suzuki J, Katakura R, Mori T. Interhemispheric approach through the lamina terminalis to tumours of the anterior part of the third ventricle. Surg Neurol 1984; 22: 157–163. Caldarelli M, di Rocco C, Papacci F, Colosimo C Jr. Management of recurrent craniopharyngiomas. Acta Neurochir (Wien) 1998; 140: 447–454. Abrams LS, Repka MX. Visual outcome of craniopharyngioma in children. J Pediatr Ophthalmol Strabismus 1997; 34: 223–228. Sweet WH. Recurrent craniopharyngiomas: therapeutic alternatives. Clin Neurosurg 1980; 27: 206–229. Rajan B, Ashley S, Thomas DG, Marsh H, Britton J, Brada M. Craniopharyngioma: improving outcome by early recognition and treatment of acute complications. Int J Radiat Oncol Biol Phys 1997; 37: 517–521. Suzuki J, Katakura R, Mori T. Interhemispheric approach through the Lamina terminalis to tumours of the anterior part of the third Ventricle. Surg Neurol 1984; 22: 157–163. Suzuki J. Bifrontal anterior interhemispheric approach In: Apuzzo MLJ (ed) Surgery of the Third Ventricle. Baltimore: Williams & Wilkins, 1987: 413–439. Shillito J. Craniopharyngioma: the subfrontal approach, or none at all Clin Neurosurg 1980; 27: 188–205.
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Journal of Clinical Neuroscience (2002) 9(2), 159–163 © 2002, Elsevier Science Ltd. All rights reserved. DOI: 10.1054/jocn.2001.0942, available online at http: //www.idealibrary.com on
Clinical study
The neurological abnormalities and operative findings in the transcallosal approach for large juxtasellar–ventricular craniopharyngiomas Han-Jung Chen MD PHD Department of Neurosurgery, Chang Gung University and Medical Center at Kaohsiung, Taiwan
Summary From February 1994 to June 1999, there were 36 patients who underwent microsurgical resection for craniopharyngiomas. Of 6 adult patients, large tumours ([4 cm) with extension from intrasellar or parasellar space to the foramen of Monro were found. There were 4 male and 2 female patients with ages from 18 to 44 years (mean 32.4 years). All of them underwent one-stage interhemispheric transcallosal total excision of tumours. This approach affords good overhead view of tumours and surrounding structures. Preoperatively, decreased visual acuity and restriction in the visual field was found in 5 patients. Studies of anterior pituitary function revealed far below the normal range in all patients. Polyuria was noted in 4 patients. All of 6 patients showed abnormally high levels of blood cholesterol and triglyceride and 4 patients were obese. Tumours with extension into the pituitary fossa were found in 2 cases. A relatively caudal location in the third Ventricle was found in case 6, who showed better preoperative visual function and no polyuria. There were no major surgical complications encountered. One patient with high cholesterol (731 mg%) died of acute pancreatitis 6 months after surgery. For large craniopharyngiomas without pre-chiasmatic extension, proper treatment could be achieved under substitution therapy and one stage transcallosal total excision of tumours with preservation of important neural structures. © 2002, Elsevier Science Ltd. All rights reserved. Keywords: Corpus callosum, craniopharyngioma, juxtasellar tumour
INTRODUCTION Since Harvey Cushing described the management of craniopharyngiomas as a ‘baffling problem to neurosurgeons’, many improvements in the surgical treatment in this tumour has been developed in addition to hormone substitution therapy, radiotherapy and chemotherapy.1–7 On the basis of improvement of radiological studies, especially magnetic resonance imaging (MRI), microdissection of anatomical structures and microsurgical techniques, radical excision can be achieved with surgical approaches.8–12 The operative findings will be presented in detail under operative microscope. Most craniopharyngiomas are involved with vital brain structures: hypothalamic–pituitary axis, visual pathway, peduncular area and limbic system.13,14 Clinically, a patient with craniopharyngioma has a different prognosis, depending on whether the important areas are involved or not.13,15 The preferred approach and the extent of surgery vary, depending on the size and involvement of the tumour and the neurosurgeon’s experience.12,16–20 For small tumours, surgical treatment will achieve a high cure rate, but for a large or giant tumour, the associated visual and endocrino-metabolic deficits are still devastating.7,15,21 We report the surgical findings on the large craniopharyngiomas and the associated neuro-endocrinological deficits.
with contrast medium. Conventional cerebral angiography or MRI angiography were performed in selected patients. For ophthalmological investigation, visual acuity and perimetry examination were performed before and 3 months after surgery. Endocrinological assessment was done in all patients including dynamic tests with adreno-corticotropic hormone, thyrotropinreleasing hormone, gonadotropin hormone, growth hormone and prolactin before and 1 week after surgery. The hormone studies were also repeated in the follow-up period. Preoperative blood chemistry including serum cholesterol and triglyceride was examined and repeated in the follow-up period. Three patients aged under 15 years were treated by pediatric neurosurgeons. For 27 patients with smaller tumours (diameter \4 cm), the pterional trans-sylvian approach was performed to excise the tumours. The surgical result was good in these 27 patients. There was no surgical mortality and no major neurological deficits encountered. Major neurological deficits were present in 6 patients including visual dysfunction, severe headache, vomiting and mental change. Radiological studies revealed the sizes of tumours were over 4 cm in diameter in all six patients. The tumours extended from the pituitary fossa to the foramen of Monro and interpeduncular space. Obesity was found in four patients. All six patients underwent craniotomy with transcallosal approach for tumour excision.
METHODS AND PATIENTS
SURGICAL TECHNIQUES FOR TUMOURS
From January 1994 to June 1999, 36 patients were admitted to our department for treatment of craniopharyngiomas. Preoperatively, all patients were evaluated by magnetic resonance imaging (MRI)
The interhemispheric, anterior transcallosal, transforaminal approach has been performed in all cases.2,24 A bicoronal skin incision with a right unilateral, para-median, 5 cm frontal craniotomy was done. The craniotomy was about 1.5 cm to the left of the superior sagittal sinus. The retraction of the brain was minimal, about 1.5 cm away from the falx and one third of the craniotomy was behind the coronary suture. A self-retaining retractor with one 3/5 inch spatula was used. Some space was usually allowed with retraction of the falx. The dissection plan of the corpus callosum was along the space between the pericallosal arteries. A small
Received 28 December 2000 Accepted 7 March 2001 Correspondence to: Han-Jung Chen MD, PhD, Department of Neurosurgery, Chang Gung Memorial Hospital at Kaohsiung, Taiwan, 123 Ta-Pei Road, Niao-Sung Hsiang, Kaohsiung Hsien, Taiwan. Tel.: ]886 7 7317123; Fax: ]886 7 7318762; E-mail: [email protected]
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callosal incision about 2 cm was made. Because of hydrocephalus, the corpus callosum was usually very thin. The septum pellucidum and the fornix should be carefully protected from injury to avoid memory impairment. The dilated foramen of Monro afforded a space enough for removal of the intraventricular portion of the tumour. The choroid plexus was usually visible from the foramen of Monro to the lateral ventricles. This structure, striothalamic and internal cerebral veins were well protected. Aspiration of a motor oil-like cystic component and piecemeal extirpation of a calcified mass were useful for the dissection of the tumour capsule away from the ventricular wall. Pieces of cottonoid were used for protection of the normal tissue. The choroidal arteries and septal, striothalamic and internal cerebral veins should be carefully protected from injury. Meanwhile, close attention was paid to the protection of the fornix, anterior commissure, pre-septal nucleus and infundibular area. A perforation through the base of the third Ventricle was observed in 6 cases; the tumours were not purely intraventricular in location. Adhesion was noted between the tumour and hypothalamic tissue, mamillary bodies and optic tract. This was one of the important points for dissection. Delicate protection of these important structures and the perforators of the posterior communicating artery was absolutely indicated. Another important point was preservation of the perforators from the basilar tip and proximal segment of the posterior cerebral arteries, surrounding venous structure and the cerebral peduncule. Transcallosal approach afforded a better overview for dissection of this area than pterional approach or the route through the lamina terminalis. RESULTS Clinical presentation The major preoperative symptoms for each patient are listed in Table 1. The duration of symptoms ranged from 3 months to years. Visual acuity deterioration and visual field restriction were found in all patients except in Case 6 who had a normal perimetry study. Headache was also one of the major complaints. All patients presented with drowsiness and unsteady gait. Preoperative neuroradiological findings MRI studies revealed marked homogenous contrast enhancement of the tumour in Case 1. In other five cases, MRI showed mixed
components and in Case 5, cystic component presented the major portion of the tumour. All tumours extended from the skull base up to the foramen of Monro. In Case 4, 5, tumours in the intrasellar or infra-diaphragmatic space were well visualized from MRI. Hydrocephalus was found in all cases. The pituitary gland and stalk were poorly demonstrated in MRI studies. Endocrinological findings Pan-hypopituitarism was observed in all patients. The endocrinological studies included gonadal axis, thyroid axis, adrenal axia, growth hormone and prolactin. The data, except prolactin in 6 patients, were abnormally low (Table 1). The body height in Case 5 was 165 cm but the secondary sexual characteristics did not develop. Irregular menstruation after menarche was found in Case 6. About 6 months before admission, hypo-menorrhea or amenorrhea was noted. Diabetes insipidus presented in 4 patients. The daily urine amount of Cases 3 and 6 was around 3000 cc without complaints of polyuria. Hyperlipemia with abnormal high levels of serum cholesterol and triglyceride was found in all patients. Four of them were obese and one (Case 3) had serum cholesterol level up to 731 mg%. Operative findings and outcome The operative findings and outcome are summarized in Table 2. In Cases 3 and 6, polyuria was not the major complaint. The operative findings revealed a small adhesion band of tumour capsule to the diaphragm (Case 3), or dorsum sellae (Case 6). The destructive effect of tumours on the infundibulo-posterior pituitary axis was probably not disastrous. Therefore, pitressin was not always necessary after surgery. Because of relatively caudal location of tumour in the skull base, the destructive effect of the tumour on the optic pathway in Case 6 was relatively mild. The visual acuity and visual field examination revealed no deficit. Endocrine dysfunction persisted after surgery and postoperative substitution therapy continued in all patients. Because of polyuria, three patients needed long-term use of pitressin. Postoperatively, they made good neurological recovery. The visual acuity and visual field gradually improved in all cases. In Cases 1, 3, 4 and 5, the temporal hemianopia recovered after a 6-month follow-up period. The higher function of the brain and memory also improved in these six cases. Cases 2 and 4 could enjoy their easy jobs, Case 5 returned to his junior college and Case 6 got
Table 1 Preoperative ophthalmological and endocrinological findings Patient No.
Sex
Age
Visual acuity
Visual field
Thyroid axis
Gonadal axis
Adrenal axis
GH
Posterior pituitary function
N (low margin)
↓
↓
↓
Polyuria
Chol: 199 TG: 430
–
↓
↓
↓
↓
Polyria
Chol: 235
]
Temporal hemianopia –
N (low margin)
↓
↓
↓
(–)
Chol: 731 TG: 350
]
No defect Temporal hemianopia Bitemporal hemianopia
↓
↓
↓
↓
Polyuria
]
N (low margin)
↓ No Secondary sexual develop ↓
↓
↓
Polyuria
Chol: 238 TG: 338 Chol: 250
↓
↓
(–)
1
M
44
R: FC L: 0.02
– Temporal hemianopia
2
F
38
R: 0.2 L: 0.5
Restriction in both sides
3
M
31
4
M
35
5
M
18
R: 02 L: hand movement R: 02 L: 0.08 R: 0.01 L: 0.01
6
F
31
R: 0.5 L: 0.1
Both sides: no restriction
Anterior pituitary function
N
Blood lipid (mg%)
Obesity
Chol: 270 TG: 232
]
[
R, right side; L, left side; N, normal, low margin: normal but close to low margin, ↓: impaired, Chol: cholesterol, TG: triglyceride.
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Transcallosal approach for large juxtasellar–ventricular craniopharyngiomas 161 Table 2 Surgical findings and outcome of six patients with big craniopharyngiomas Patient No.
Appearance
1
All solid with fibrous band and adhesion with the diaphragm and tuberculum Major portion was calcified solid mass, fibrous adhesion with the retrochiasmatic tuberculum Major part of tumor was solid mass, a small fibrous adhesion with the tuberculum and diaphragm Major portion of tumor was solid, extension into the intrasellar space Major portion was cystic component, tumor inside the whole pituitary fossa Major part of tumor was solid, fibrous adhesion with the dorsum sellae
2
3
4
5
6
Resection (tumor size, cm) Complete (4.2) Complete (4.0) Complete (5.0)
Complete (5.0) Complete (4.5 cm) Complete (5.0)
Complication
None (continuous pitressin usage at night time) None (No more pitressin now) None (No pitressin after surgery) None (continuous pitressin usage at night time) None (Continuous pitressin usage at night time) Mild left hemiparesis, pitressin was needed for 4 months
Follow-up (months)
Outcome
40
Independent living
46
Return to work
6
24
Died of acute pancreatitis and associated hepatorenal failure Independent living
15
Continuing school work
8
Independent living
a job as a clerk in a store. Case 3 enjoyed a good living after surgery. Pitressin was only temporarily necessary for him. Unfortunately, he died of acute pancreatitis 6 months after surgery. He was found to have abnormally high serum cholesterol and triglyceride.
ILLUSTRATIVE CASES Case No.4 This 35 year old man had suffered from polyuria and bilateral blurred vision for more than 3 months. He was admitted to our department in March, 1997. Review of history revealed general weakness, drowsiness and loss of libido for more than 6 months. He was the father of two children. Ophthalmological examination revealed left visual acuity 0.08 with temporal hemianopia and right visual acuity 0.2. Hormone studies, including gonadal, thyroid, adrenal axes and growth hormone were below normal range. MRI revealed a retro-chiasmatic mass with extension from the pituitary fossa up to the foramen of Monro (Fig.1). The major portion of the tumour was contrast enhanced solid tissue with a diameter of 5 cm. He underwent an uneventful craniotomy with transcallosal approach of total excision of tumour. Drainage of the cystic component and piecemeal extirpation of calcified solid mass were performed for internal decompression. The capsule of the tumour was carefully dissected away from the normal tissue, especially around the hypothalamus, optic tract and other important structures. The tip of the basilar artery and its perforators were well protected. The normal pituitary gland was replaced by the tumour mass (Fig. 2). This tumour was totally excised through the transcallosal approach in one stage. He recovered soon with full consciousness and good motor function. Hormone substitution treatment and desmopressin acetate (DDAVP, Rhône-Poulenc Rorer Pharmaceuticals, Inc., PA, USA) 0.025 cc was administered before sleep for polyuria. He returned to work. The follow-up MRI 2 years after surgery revealed no recurrence (Fig. 3). Case No.6 This 31-year-old unmarried woman has been found to have irregular menstruation since menarche. About 6 months prior to this admission, hypomenorrhea or amenorrhea was noted. The daily activities had been normal until one month before admission when intermittent headache and vomiting occurred. On admission, she was found to be very drowsy. Physical examination revealed © 2002, Elsevier Science Ltd. All rights reserved.
Fig.1 MRI (T1-weighted image with contrast enhancement) in Case 4 reveals a large heterogenous tumour with downward extension to the pitutitary fossa and upward extension to the foramen of Monro.
isocoric pupils with prompt light reflex. The visual acuity in the right was 0.5 and the left, 0.1. She wore spectacles for years and no definite vision change was noted. Perimetry demonstrated no defect in the visual field. Hormone studies showed most values were far below the normal range except prolactin (57.8 ng/ml). Besides normal perimetry, she did not complain of polyuria. The daily total urine amount was less than 3000 cc, and MRI revealed a big suprasellar irregular contrast enhanced tumour with extension to the foramen of Monro and compression to the midbrain and cerebral peduncles. Multiple cystic components were noted (Fig. 4). She underwent right frontal craniotomy with transcallosal approach in June 1999. Internal decompression by drainage of the cystic component and piecemeal extirpation of calcified solid tumour was performed through the dilated foramen of Morno. The normal hypothalamic and optic pathway were protected with cottonoid while dissecting the tumour capsule. This tumour could be separated away from the infundibulo-pituitary axis. A fibrous adhesion to the dorsum sellae was found. This adhesion band was coagulated and then divided. Compared with other large craniopharyngiomas, the anatomical location of this tumour was relatively caudal to chiasma Journal of Clinical Neuroscience (2002) 9(2), 159–163
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Fig. 4 MRI (T1-weighted image with contrast enhancement) of Case 6 reveals a large extra- and intra-ventricular tumor with compression to the cerebral peduncles.
Fig. 2 The pituitary fossa is empty after removal of tumor. The dorsum Sellae (arrow) is shown.
Fig. 3
Follow-up MRI 2 years after surgery reveals no recurrence.
and hypothalamus. This is the reason why the perimetry showed no defect and she did not complain of polyuria. This tumour showed tight adhesion with the cerebral peduncles and impaction in the interpeduncular space. Delicate dissection and preservation of all perforators from the basilar tip and the proximal segment of both posterior cerebral arteries were achieved (Fig. 5). Finally, the tumour was totally excised. Mild polyuria (daily urine amount less than 4000 cc) developed after surgery. Only mild left hemiparesis was found. This might be caused by the dissection between the tumour capsule and cerebral peduncles. She recovered gradually after a programmed rehabilitation and got work as a clerk in a store one year after surgery. Her memory showed good recovery. Journal of Clinical Neuroscience (2002) 9(2), 159–163
Fig. 5 Operative picture of Case 6 reveals the basilar artery, p1 (arrow heads) and perforators (arrows). The tumor was totally removed.
DISCUSSION Craniopharyngiomas usually affect not only the hypothalamic– pituitary axis, but also the visual pathway, frontal lobes, striothalamic areas, limbic system and caudally midbrain and cerebral peducular area.9,14,22 In more than 100 previous cases, mental disturbance, visual deficits and endocrine dysfunction affected more than half of our patients. The neurosurgeon always tries his utmost to treat this tumour by employing microsurgical techniques to separate the normal structures from the tumour, preserve the normal function and totally remove the tumour. Of course, we should consider the balance between the risk of recurrence and injury of important structures © 2002, Elsevier Science Ltd. All rights reserved.
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Transcallosal approach for large juxtasellar–ventricular craniopharyngiomas 163
such as the hypothalamus and visual pathway.6,12,13,21,23 Hormone substitution therapy available at present is a dispensable treatment for patients before and after surgery.12,14 Damage of the visual pathway by the tumour is another disastrous effect in most cases. There is no substitution therapy for this.7,12,13,22 Conventional radiotherapy or stereotactic radiosurgery cannot assure long-term preservation of visual function.6,13,22,24 For small juxtasellar or parachiasmatic tumours, the pterional trans-sylvian approach usually accomplishes complete removal of tumours. The symptoms and signs may be mild or limited. For large extra- and intraventricular tumours, the strategy of the author is total excision of the tumour, followed by appropriate substitution therapy instead of repeated surgical procedures and/or radiotherapy.1,11,12 A certain numbers of neurosurgeons choose trans-lamina terminalis approach for a large intraventricular tumours. This approach is, however, suitable for tumours in the anterior part of the third ventricle, especially for those that develop anteriorly from the line joining the anterior ridge of the foramen of Monro and cerebral aqueduct. For tumours developing to the posterior part of the third ventricle, complete excision by opening the lamina terminalis only is not possible.25–27 The disadvantages of this approach also include the possibility of infection from the frontal sinus, division of the sagittal sinus and injury of the olfactory nerves. For large craniopharyngiomas like these 6 cases, the foramen of Monro was large enough for removal of the tumour using the transcallosal, transforaminal approach. The septum pellucidum and the fornix could be carefully protected from injury to avoid memory impairment. After internal decompression, the capsule of tumours could be carefully dissected from the anterior commissure, pre-septal nucleus and infundibular area. This approach affords a good overhead view of removal of tumours inside the sella and dissection of tumours from the normal structures such as both internal carotid arteries, the posterior communicating arteries and their perforators and perforators from the P1 segment of the posterior cerebral arteries and the tip of the basilar artery. It would be difficult to remove the tumour around the area of the tip of the basilar artery using translamina terminalis approach in Case 6. Of course, the transcallosal, transforminal approach shows its limitation in removal of tumour at the pre-chiasmatic space.2,4,5,12 The destructive effects of tumours on the optic pathway are usually disastrous in the patients and were especially pronounced in the children. There is still no substitutional therapy for the damaged vision. Surgery can release the destructive effect of the tumours and preserve the residual vision or even improve the vision. A good-quality MRI can provide the surgeons with information such as size, location and attachment of the tumour.8 Operative findings prove the imaging studies. In case 6, we found the tumour capsule was attached to the dorsum sellae. The pressure or destructive effects of the tumour was probably not high on the chiasma or optic radiation, so the visual function was preserved. The anterior pituitary function of all six patients was impaired. The data about the gonadal axis, adrenal axis and growth hormone were far below the normal level. However, in the thyroid axis, it was better, in low margin of normal range for 3 patient and normal for one patient. Diabetes insipidus was found in 4 patients except Case 3 and 6. The abnormality of endocrinological function showed the correlation of location of tumour in MRI studies and operative findings. The destructive or pressure effects of the tumours on the midbrain and cerebral peduncle will increase if the tumours grow toward this area. It needs delicate microsurgical technique to separate the tumour capsule from the normal neural tissue and perforators from the basilar tip or P1 segment. In the author’s opinion, transcallosal approach provides a better overhead view for this area. There is a blind spot for dissection of the basilar tip and peripeduncular area if approach is through the lamina terminals, © 2002, Elsevier Science Ltd. All rights reserved.
and the blind spot is also for dissection of the perforators of the opposite posterior communicating artery with pterional transsylvian approach. Of course, the pure transcallosal approach has its limitation in the excision of pre-chiasmatic mass, especially tumour encasing the anterior communicating artery and its branches.1,2,9,12 Obesity and hyperlipemia is one of the major problems for patients. Because of destruction of the hypothalamus, unlimited intake will induce obesity and hyperlipemia. This may be the cause of acute pancreatitis found in Case 3. Appropriate medical therapy and diet control is indispensable for achieving a good surgical result. REFERENCES 1.
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