What can be expected from the surgical treatment of craniopharyngiomas in children. Report of 92 cases.

Original Papers Child’s Brain 5: 433-449 (1979)

What can be Expected from the Surgical Treatment of Craniopharyngiomas in Children Report of 92 Cases

J. Rougerie Department of Neurosurgery, Centre médico-chirurgical Foch, Suresnes

Key Words. Craniopharyngiomas • Children ■Surgical treatment • Functional results Abstract. 92 cases of craniopharyngiomas have been operated, 63 with a follow-up extending from 30 months to 20 years. The possibilities of the surgical treatment are mainly discussed on the basis of the anatomical location of the tumors. Simple intrasellar craniopharyngiomas can be operated by a transphenoidal approach with, sometimes, spectacular improvement (case of spontaneous normal pregnancy). The tumors most frequently seen are those with predominantly prechiasmatic expansion. A subfrontal approach is always required, aiming at radical excision. Microscopy can offer interesting possibilities for selective surgery. However, if the vital prognosis is excellent and recur rences rare, functional results remain very poor. In retrochiasmatic tumors involving the posterior hypothalamus, the principle of radical excision must be seriously discussed and sometimes challenged. The various combined approaches are described with their own limits and risks. Other therapeutic techniques are suggested. In many cases, selective neurosurgical dissection may raise some hope for better improvement of functional results.

In 1973, commenting on the excellent article of Kahn et al. [9]. Bucy pointed out ‘how rare it was to find a man who will voluntarily acknowledge

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This paper was written in 1974, but could not be published at that time due to the author's severe illness. Much has happened in 4 years: the essential part played by CTT scanning in diagnosis, assessment of excision and follow-up; that of nonsurgical techniques (external and interstitial irradiation) and, eventually, the results achieved by hormonal therapy. Nevertheless, in the author’s opinion the indications and the possibilities provided by surgery still hold good, as well as the trend towards a selective excision as the only way to improve the functional result. The best proof is afforded by a case [19], reported in the ‘Results’ section, who carried out a spontaneous normal pregnancy and delivery. Further more, this 4-year delay and the possibilities of control provided by CTT scanning fully bear out the statements made pertaining to the possibilities of excision of the growths and their evolutivity.

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his error in print'. This paper, similarly, is concerned with clarification and revision of our thoughts. In 1962, we tried to demonstrate the technical possibility of radical excision [17]; this seemed to us all the more imperative since minimal treatment of these benign extra cerebral tumors had consistently failed. With more experience and, above all, with a better appreciation of the postoperative course and long-term results, we have been led to reassess the basis of certain operative decisions, rejecting some old ones and proposing some new ones. Our research for the last 6 years has been resolutely turned towards the best possible functional result. This important idea, especially in young patients, has prompted us to reexamine our excessively systematic approach.

We have operated, with Guiot. 140 craniopharyngiomas. 92 patients were between the ages of 2 and 17 years (64.28%). In addition, there are of course cases of craniopharyngio mas which escape diagnosis until adult age or in which operation was not deemed justifiable. If one adds the age group 17-20 years the figure becomes nearly 68%. 10% were under 5 years at operation; 30% were between 5 and 10 years; 40% were between 10 and 15 years, and 20% were between 15 and 17 years. The separation of infantile and adolescent craniopharyngiomas is significant. It corresponds in fact to a clinical and anatomic entity. The clinical signs and evolution of the adult form of tumors are often characteristically different from the juvenile forms. Some authors [1, 7, 9] describe an anatomic and pathologic duality based on different embryological origin. In fact, endocrinologic problems comprise the major interest of craniopharyngiomas in young people. Appreciation of evolutionary potential and functional results has led us in this review to consider only the 63 cases with more than 30 months follow-up. It is clear that after this delay the frequency of recurrences has significantly diminished and the clinical results have become objectively verified. Among all intra- and suprasellar tumors in young patients, craniopharyngioma is the most frequent. In a previous report [18] of 113 cases we found: (a) 63 craniopharyngiomas; (b) IS pituitary adenomas, rare under 12 years of age, 11 additionally between 17 and 20; (c) 15 optic gliomas (an additional 11 were not operated); (d) 6 ectopic pinealomas (supra sellar germinomas); (e) 3 astrocytomas, and (f) 4 unclassifiable cases (hamartomas). Another consideration of fundamental importance is the anatomical location of the tumor which affects all the following: (a) clinical course and neuroradiologic appearance; (b) possibility of total or partial surgical removal, and (c) ultimate prognosis and, to a certain extent, functional result. There are now three classic forms: (1) simple intrasellar; (2) intra- and suprasellar with primarily anterior expansion (prechiasmatic), and (3) retrochiasmatic with expansion into the posterior hypothalamus.


Material

What can be Expected from the Surgical Treatment of Craniopharyngiomas

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We might add that there are rare giants forms, both pre- and retrochiasmatic, which result in puzzling clinical and neuroradiological patterns, which offer limited operative possibilities and which frequently have a grave prognosis. We shall discuss these 4 varieties. Intrasellar Craniopharyngiomas Simple intrasellar craniopharyngiomas are not rare. We have 10 cases (10.86%). This number is reduced by those patients who escape diagnosis or were not operated. We unfortunately propagated the nonsurgical approach by writing 12 years ago ‘there is no purely endocrinologic indication for operation’. We had 2 cases of craniopharyngiomas diagnosed in childhood, who required operation at the ages of 27 and 30 years because of new clinical signs secondary to suprasellar expansion. In this series no patient was less than 10 years old and half were between 10 and 15. It appears that late diagnosis and treatment may worsen the prognosis. Among simple intrasellar tumors with purely endocrine manifestations, cranio pharyngiomas occupy the first place. The only differential diagnosis is pituitary adenoma (6 cases) in which the average age is a little higher (none before 12 years, 3 between 12 and 15). Practically speaking, two facts are of prime importance: (1) simple intrasellar craniopharyngiomas usually present with panhypopituitarism; certain adenomas can present with symptoms of pituitary excess (galactorrhea, acromegaly, gigantism), and (2) calcifications which are pathognomonic of craniopharyngiomas were present in 7 of our 10 cases. But in 4 cases, diagnosis was made only at operation. Previously, we operated only adenomas because endocrine insufficiency secondary to craniopharyngiomas was con sidered irreversible. Now we believe that viable pituitary tissue may exist in the presence of craniopharyngiomas: There are cases in which craniopharyngiomas were found in adults devoid of endocrine defects; we have found pituitary tissue in 3 suprasellar cranio pharyngiomas in which radical excision was performed. We discovered the possibility of a transphenoidal approach in a case which we thought to be an adenoma. The microscope allows us: (1) a better dissection, although it is impossible to be certain of a perfect excision, and (2) to realize our primary goal aimed at selective surgery and the conservation of residual pituitary tissue.

The treatment of most of these intrasellar tumors is benign: operative and postoperative mortality is 0; morbidity was limited to 3 diabetes insipidus (1 required long-term treatment) and 1 case of CSF leakage which responded to temporary shunting ; there were no infections or major endocrine problems. The endocrine results are interesting, although inferior to those obtained in adenomas. Some pituitary tissue was identified in 3 of our 10 cases. 2 had spectacular improvement: one, with secondary amenorrhea, recovered spon taneous menstruation [19], another regained a growth curve although puberty was late and incomplete. None of these cases completely normalized the STH and FSH.


Results

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Intra- und Suprasellar Tumor with Predominantly Prechiasmatic Expansion This form was most frequent in our series comprising 47 cases (51%). This percentage is somewhat reduced by the addition in the last 3 years of an excess of cases with posterior extension which had been referred to us after rejection by other neurosurgeons or after failure of other treatment. Diagnosis is usually early: 19% were less than 5 years of age and 26% were between 5 and 10 years of age. Signs of hypothalamic pituitary insuffi ciency, optic chiasmatic compression and, rarely, intracranial hypertension (6%), facilitated diagnosis. Differential diagnosis is rarely difficult because of different presenting signs and lesser frequency of other lesions: (a) 6 pituitary adenomas: one was a giant tumor invading the cavernous sinus; (b) 2 ectopic pinealomas with anterior expansion; (c) 1 astrocytoma, and (d) 8 explored chiasmatic gliomas. With the exception of adenomas, these tumors do not enlarge the sella turcica. Therefore, radiotomography is sufficient to exclude them. The discovery of calcification is always important since, as we have already stressed, the operative approach for an expanding suprasellar adenoma differs significantly from that for craniopharyngioma. Despite technical progress, we found calcification in only 70% (some authors report


These are indeed encouraging operative results and can perhaps be improved with earlier operative intervention. Despite the uncertain character of some excisions, we have had no recurrence in the 8 cases that we have followed for more than 30 months. With our current technique, we operate transphenoidaly only in the absence of suprasellar expansion, in contrast to the practice of Hardy. If the tumor is an adenoma and its expansion widely opens the sella turcica with grade 1 or 2 extension, then a transphenoidal approach is effective [5]. This approach is dangerous for a craniopharyngioma: there is a tough often calcified capsule and its close adherence to the optic nerves and the major vessels makes dissection difficult, dangerous and often inadequate. In addi tion, complementary irradiation which is useful for partially excised adeno mas, has no effect on residual craniopharyngioma tissue. This points to the absolute necessity of confirming the intrasellar nature of the tumor, which can only be done by pneumoencephalocistemography. With the proper technique this usually permits visualization of the optic chiasm and optic nerves. The role of CTT scanning is promising but as yet uncertain.


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Therapeutic Modality Operative intervention is required in all cases. One must never wait fo r the appearance o f visual disturbances which are completely unpredictable. In


higher yields) even though they are usually histologically demonstrable. Linear eggshell calcifications indicate the presence of a cyst, as Lindgren and Di Chiro [13] have long contended, and facilitate stereotactic localization for puncture. CTT scanning should be of considerable help in identifying calcifi cations. Besides simple diagnosis, neuroradiologic procedures offer two other benefits. (1) precise determination of tum or size and shape, particularly the number, location and size of discrete extensions, and (2) definition of proximity to essential structures (optic chiasm, cavernous sinus, circle of Willis). The circle of Willis is best studied by angiography [12]. The value of the Hirtz position has been demonstrated by Raimondi [16]. But regardless of available procedures, we still value most highly ventriculocisternographic tomography. This can be achieved in the following ways: (1) frontal burr hole; (2) routinely indicated when clinical examination, supported by angiography, demonstrates dilated ventricles; (3) desirable when a large tumor with a cystic component is suggested by X-rays, echo B and brain scan. When available CTT scanning can replace these three procedures. The location of the frontal burr hole must be carefully selected in order to permit a stereotactic puncture through the foramen of Monro. Lumbar or suboccipital pneumotomoencephalography can visualize simultaneously the ventricular system (chiefly the anterior floor of the third ventricle and occasionally the frontal and temporal horns) and the individual basilar cistern. Localization of the chiasm is usually easy but visualization of the optic nerves is only possible with small tumors which comprise less than half of our cases. A good view of the floor of the third ventricle is essential in order to determine the superior limit of the tum or and its relation to the anterior hypothalamus. A complementary image can be obtained by injecting, near the foramen of Monro, a reabsorbable positive contrast material (dimer X) under image intensification. We performed simultaneous stereotactic puncture only three times: twice for intracranial hypertension and once for severe deterioration in the patient’s general condition.

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many cases, especially the calcified forms, the optic nerves and chiasm are not only stretched and displaced but actually encrusted in tumor so that their blood supply can be significantly compromised. This explains acute compli cations in some cases: in one, visual acuity fell from normal to 30/100 of normal in 3 months. Unfortunately, the resulting optic atrophy is always irreversible. We cannot overemphasize the necessity of early intervention. This is a good reason for early operation in the simple intrasellar forms, before visual signs of tumor expansion appear. The operative approach is always subfrontal, facilitated by cerebral retraction; we customarily add lumbar CSF drainage, mannitol and con trolled negative pressure ventilation. We have never had to resect any cerebral tissue. The expansion of the tumor between the posteriorly displaced chiasm and the stretched elongated optic nerves creates an anatomic situation which facilitates radical excision in many cases. There is general agreement on this point [7, 15, 17]. Use of visual magnification techniques can improve our treatment. We have had few operative failures: (a) 2 calcified lesions (one very large, one of modest size) in which dissection of the carotid and anterior cerebral arteries was impossible; (b) 4 cases in which small capsular frag ments adhered to the carotids and were cauterized in situ; (c) 1 incomplete excision secondary to short optic nerves (there has been not recurrence in the last 5 cases mentioned; (d) finally, in 4 cases, it was difficult to distinguish between the inferior portion of the tumor capsule, the meningeal lining of the sella, and the diaphragma sella which was perhaps invaginated by the tumor: this latter phenomenon could explain our 2 intrasellar recurrences. Of our 44 attempts at complete excision, we succeeded in 33 (75%). Of these 44, we have had between 30 months and 20 years follow-up in 32 with only 4 recurrences (12.5%). Of these 4, the original excision had appeared complete in only 2. All were reoperated: 3 transphenoidale, 1 twice (once from below, once from above). The number of recurrences is underestimated since it includes no case in which excision was obviously incomplete or certain unusual forms to be discussed later. The remote possibility of late recurrence does not seem to warrant a change in surgical approach. This conclusion is supported by our favorable operative statistics. We have had no operative death in 12 years and only one postoperative death in a patient already seriously ill before surgery.


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These excellent results are due to the quality o f our intensive care unit [2], particularly with regard to the postoperative maintenance of electrolyte balance and long-term endocrine support. Morbidity has been limited to 2 cases of completely calcified tumors with postoperative deterioration of vision, and 4 postoperative occurrences of diabetes insipidus requiring long-term treatment. In contrast we must consider the constant mediocrity o f functional results: Diabetes insipidus (D I): all cases which preoperatively had DI (clinical or subclinical) continued to have it. Operation caused it in some others. We now have significantly less DI because of a particular operative tactic: careful examination of the lesion under visual magnification permitting conservation of the stalk (1 case) or section of its lower portion from the tumor mass (6 cases). This has not resulted in persistent DI. However, such an approach can threaten the adequacy of tumor excision. Incidentally, all our cases of DI are now well controlled, some spontaneously (occasionally even after long delay), most through appropriate medical management. Weight control has been obtained in 4 out of 5 cases although gain has been unavoidable, mostly in the first year, in which a considerable gain can be seen (70% in 1 case). Hypothyroidism, very frequent, is controlled with replacement therapy, followed by Achilles reflexograms. Biologic adrenal insufficiency is also frequent but clinical tolerance is variable. Since there is evidence that cortisone can inhibit growth, we have tried to avoid replacement, even in some cases in which ACTH lack has been demonstrated. This approach assumes that only occasional minor complica tions will occur but requires rigorous follow-up (1 case died 3 years after operation of acute adrenal insufficiency during an intercurrent infection). Two considerations remain most important: (1) Growth: All of our cases retained growth failure to variable degrees. In the best cases, those who received no cortisone, growth was 1 SD (2 cases) or 2 SD (3 cases) below normal. 3 others grew only 2 cm per year; in 1, growth continued past the age of 22 years. These results are only fair and are at present little improved with thera peutic efforts. One always hesitates to use androgens and other anabolic steroids which, despite an initial effect, tend towards premature bony matu ration and consequent growth arrest. Our experience in the use of growth hormone is too short to confirm the favorable impression of other authors. In 3 cases, 2 receiving cortisone, growth in 3 years was 12.5-10 and 6 cm. One of them had DI transiently out of control.



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Retrochiasmatic Craniopharyngiomas with Posterior Hypothalamic Expansion Because of their position behind the chiasm, nesting near the hypo thalamus, obstructing (more or less) the foramen of Monro, compressing the brain stem, and compromising the posterior vasculature of the circle of Willis, these forms of tumor have a character of their own. Their prognosis is poor because excision is difficult. Complete removal is usually impossible, there is always a significant risk to life, functional results are always question able and recurrences are frequent. These forms are much more frequent in our series than in some others in which, however, a clear distinction was not always made between pre- and postchiasmatic tumors. Our 33 cases (35.8%), however, seem to overestimate the true incidence, for reasons previously discussed. There is a clear pre


These discouraging results led to one change in approach over the past 6 years, i.e. towards more selective surgical procedures. The best answer to our problems seems to be to preserve functional pituitary tissue in the course of operation. Unfortunately, our attempts to identify and separate pituitary tissue (after dissection of the stalk) with the aid of visual magnification (especially in cases which had a measurable STH level) were only rarely sucessful: 2 cases. In 1, growth is satisfactory but not normal, with a per sistently low STH level. We believe that in very young patients, it is not necessary to attempt complete removal of the intrasellar portion of tumor: 3 cases, 1 (in an infant 2.5 years of age) with a very good result. This obviously gives rise to an increased risk of recurrence and requires close neuroradiologic follow-up. We believe that this is a legitimate risk to take in order to maximize potential for growth. In addition, the residual intrasellar portion is accessible to a second transphenoidal procedure under microscopic dissection. (2) Finally, gondadal insufficiency is disappointingly common. None of our operated cases had spontaneous puberty, although 2 cases of selective approach are still under 12 years of age. The risk causing further growth delay and the possibility of provoking tumor recurrence have led us to delay replacement therapy until at least the age of 20 years. With regard to this problem, our results with some simple intrasellar tumors led us to be as elective as possible concerning excision of the intrasellar portion of the tumor. We shall see in the future whether this orientation and even earlier operation will yield sufficiently encouraging results to allow continuation of this approach.

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dominance of young patients: two-thirds of the 33 were younger than 20 years of age and a third were under 10. Among extracerebral tumors obstructing the third ventricle, cranio pharyngioma is most common. Other forms in our series include: 2 pituitary adenomas (15 years old or less); 6 ectopic pinealomas (3 purely posterior hypothalamic); 3 chiasmatic gliomas; 2 astrocytomas, and 4 unclassifiable (hamartomas). Despite their numerical predominance clinical differential diagnosis was difficult in almost one-third of our cases: 6 cases had pure intracranial hyper tension, 2 had signs indicating a tumor in the posterior fossa and 2 had aspects of pseudomeningitis. In other forms of tumors, endocrine impairment indicates specific involvement. In the posterior expanding forms characteristic signs of posterior hypothalamic involvement are unusual: obesity, adipsia, hyper natremia, 3 cases; memory loss and Korsakoff psychosis, 1 case; Russell’s syndrome, 1 case. Ectopic pinealomas and, to a lesser extent, astrocytomas, and hamarto mas can present very similar pictures (we have seen 3 cases of precocious puberty among these forms). Neuroradiology assists considerably in the diagnosis, revealing calcifica tions which are present in more postchiasmatic tumors than in other forms: 29 cases (88%). Whether or not there is evident calcification, carefully per formed tomography can find smaller calcific extensions, occasionally at the limit of vision. These are seen sometimes within a sella of normal size, some times above and behind the posterior clinoids. Besides their diagnostic interest, they facilitate choice of operative approach. Arteriography remains an excellent examination which can show: (1) significant ventricular dilatation, and (2) extent of posterior extension: compression of the basilar artery and thalamic perforating branches; stretching of posterior communicating branches. In contrast, the position of the chiasm is less reliable since it may be displaced considerably by a subjacent tumor mass. Also, to a certain extent, the length of the optic nerves determines operative technique. Combined ventricular cisternal study is of fundamental importance in postchiasmatic tumors. To our previous discussion we can add: (a) two frontal burr holes are absolutely necessary, one positioned for a possible puncture; (b) estimation of ventricular size and normalization of intracranial pressure are necessary. When intracranial pressure is elevated, the patency of the foramen of



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Therapeutic Possibility Except in rare cases (2) none of these tumors could be completely re moved in one procedure. Combined approaches, on the other hand, sometimes performed serially, frequently carry a real risk to life and functional state. Subfrontal approach. This remains most common and is usually per formed first because technical considerations offer flexibility with only moderate risk. Despite the primarily retrochiasmatic nature of the tumor, the subchiasmatic part - frequently calcified and intrasellar - can frequently only be successfully excised via a prechiasmatic route. Other approaches would be hazardous if not impossible. We believe that these tumors cannot be removed completely without this initial attempt. When the chiasm is anteriorly displaced, pushed against the jugum, it is necessary to make an artificial prechiasmatic triangular osteotomy. A lateral approach to the tumor is performed laterally between the right


Monro must be assessed. A diffusible, reabsorbable contrast material per mitted visualization in all but 3 cases of the superior posterior of the third ventricle and the aqueduct. Thus, it is possible to estimate precisely the superior and posterior limits of tumor. Air injection from below, with a catheter in the frontal horn, has never caused a problem. This permits accurate localization of tumor inferiorly and laterally. In contrast, the anterior limit is difficult to define, and we have never localized the chiasm with certainty. An attempt at puncture is routine. Trajectory is controlled by image intensification. This examination was positive in 14 cases. In 2 others the cysts were inaccessible, hidden by large calcified masses. Evacuation of the cyst frequently allows good decompression, demon strated by the appearance of the third ventricle. But in some cases there is no change, the calcified shell resting in place. It is the patency of the foramen of Monro that is always improved. Cystography allows, by superimposition over other films, estimation of location and exact volume of the tumor itself. It is often necessary to perform a repeat cisternal injection of air. There is double utility in this technique: (1) to determine whether the tumor is operable and what the best operative approach is, and (2) to be able to put off operations to await favorable conditions: this delay must be relatively short; we have seen a cyst reform in less than 3 weeks.

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optic nerve and carotid artery. Opening of the Sylvian fissure considerably increases the possibility of exposure in all cases. This maneuver, after dissection of the large vessels (carotid and/or posterior communicating), allows the tumor to be turned laterally, then downwards. It is hoped in this way to reach the posterior-inferior pole which obstructs the interpeduncular cistern. The size of the cisterns in the child, accentuated by their dilatation, in front of the obstruction, affords or pro vides appreciable aid. Nevertheless, in the case of large craniopharyngiomas, solid and calcified, contralateral removal is very delicate. Above all, it is very rarely possible (1 case) to expose the superior pole and to retract it anteriorly without risk of major hypothalamic trauma. The subtemporal route, with good cerebral retraction and section of the tentorium cerebelli, gives direct access to posterior tumor extension. After isolation of the third and fourth cranial nerves, dissection of the posterior communicating and basilar arteries and their branches can be performed under direct visual control. Tumor rarely adheres to the brain stem and can be easily separated. Its sparsely vascularized character allows piecemeal removal of the exposed portions. There remains only, except in special cases, important contralateral vascular-nervous connections remaining hidden by tumor, and above all superior freeing of the hypothalamus is completely blind. Magnification is rarely a real help. We prefer, by intraoperative radiologic control, to try to define the progress of dissection in relation to the floor of the third ventricle. The ideal would be to be able to tell at what point piecemeal removal becomes too dangerous to continue. We finally note that a contralateral subtemporal procedure can be con sidered at a later time. The transventricular route: this is performed either through the foramen of Monro enlarged by section of the anterior pillar of the trigone [17] or past the corpus callosum through the anterior portion of the roof of the third ventricle [4, 8, 14]. It is the only approach that gives direct access to the superior pole. We have already stressed that this approximates a truly intra ventricular approach, in the majority of cases sacrifiing no nervous tissue, and that there is a thin glial lamina next to the tumor which permits lateral retraction and extension of dissection basally. Thus, we can hope for complete tumor removal, especially if an asso ciated subfrontal approach has already allowed dissection of the inferior portion. In fact, despite the aid of microscopy, it is difficult to avoid hypo thalamic damage during surgery. Even if limited anterior lesions are com patible with life, it is not the same when they extend rearward.



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Thus, there is an unacceptable mortality approaching 50%, uncertainty of attempted complete excision is confirmed by autopsy, and consistent and often major functional impairment has led us to reserve our direction. The transventricular route, although more logical, is no longer the exclusive initial approach. In contrast, it can represent a complementary maneuver at the time of first surgery or later. In our last 11 cases our procedure has been: subfrontal route, using all methods which widen the approach; neuroradiologic monitoring to define precisely the residual tumor; depending on results, and after more or less delay depending on the clinical state, later subtemporal approach either uni lateral or bilateral (5 cases); transventricular route during the initial proce dure (only 2 cases) or a third procedure. This multiple-procedure approach is clearly troublesome, but seems justified by a significant improvement in results: Operative mortality, after reaching almost 50%, has considerably decreased. In the last 11 cases we have had only one early postoperative death. There were also to late deaths (one after multiple recurrences, included in our previous series). This more conservative attitude can only increase our already high recurrence rate: 8 cases, 5 in this series of 11, and 3 in our previous series (note that one of these was prechiasmatic and easily removed). These are known recurrences, with new objective signs or new neuroradiologic findings. We do not yet know the outcome of several cases in which excision was surely incomplete (5 in the last group of 11). We touch here upon a particularly delicate notion, that of evolutionary potential. This is unpredictable: apparently calcified craniopharyngiomas completely removed with normal radiologic findings, have recurred, some times very rapidly. In contrast, some very incompletely removed tumors remain quite stable. We have even seen stabilisation occur after several recurrences: one after 3 surgical procedures, with 5 years of remission; one after 2 procedures, with 3 years of remission. Histologic comparison of operative specimens shows no significant difference which might suggest structural modification of the tumors. This possibility of spontaneous stabili sation is an additional reason not to attempt, at any costs, complete excision since the risk is too great. But this apparent clinical stability is not synonym ous with cure: the case of late death after two recurrences showed at autopsy completely unresectable mesencephalic tumor infiltration. Endocrine results are particularly misleading: (a) 2 patients had worsen ing of initial visual symptoms, a third initially free of any problem had a


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lateral hemianopsia as evidence of visual damage secondary to an interoptic carotid approach; all patients operated by the subtemporal approach, except one, had transitory 3rd cranial nerve paralysis - only one has a persistent partial paralysis; (b) all had persistent diabetes insipidus requiring corrective treatment; (c) all were significantly obese requiring strict diet control, one had acute pancreatitis; (d) almost all had signs of ACTH deficiency requiring replacement; (e) only one had nearly normal growth: 3 others grew apprecia bly, and (f) only the case with normal growth underwent spontaneous puberty. In contrast, contrary to some opinions, the psyche can be completely normal, except for two cases developing atrophic lesions. There were also 3 cases of poor recent memory, 2 persistent.

Operative intervention, then, must be regarded with circumspection and the principle of radical excision has been seriously questioned during the past years: Certain cases must be challenged: large calcified masses in the posterior hypothalamus, verified by clinical and neuroradiological examina tion. 2 cases had cysts which were nevertheless accessible to puncture, which does not always modify the anatomic conditions at the level of V3. Besides, spontaneous evolution or failure of other therapeutic modalities can force operation. In these 4 cases, 2 were irradiated and 2 had implants; 2 had previous shunts. What can be said about this short series? We do not share certain authors optimism regarding the efficiency of radiation [11], if we add to the 2 cases, 3 others which were irradiated after incomplete excision, we have no proof of improvement, clinical, radiologic or histologic; nor is the simple idea of stabilisation demonstrated since 2 had to be operated. In contrast, 2 cases treated with implants by Talairach appear stable after 4 years of follow-up (but without neuroradiologic or operative moni toring). This reunites the opinion (secondary publications) [3] that an effec tive action is possible at the level of the cyst wall, whether in inhibiting reproduction or in preventing keratinization or calcification. The others have to be explored: preferably after initial cyst evacuation, or after shunting (V-P), using conservative approaches, each determined by a neuroradiological study and limited by technical restraints which we see no reason to transgress.


Indications

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Mixed Forms o f Giant Tumors (pre- and retrochiasmatic) This is an aspect that we have seen in 2 of our 29 patients. Retrospec tively, at least 2 others in the previous series fit this entity. They are, then, rare forms, but interesting and with grave prognosis. We can identify them by the following: (a) Their considerable volumes truly giant forms, (b) Their situation with significant anterior expansion between the optic nerves, long and stretched, which, by definition makes them the prechiasmatic form; but also with retrochiasmatic extension also very large, (c) Their extremely calcified character with one or more principal masses, a bed of fine calcification which appears to infiltrate the neighboring arachnoid and which must have originally been of a completely different evolution, (d) Their clinical nature, defying all classification: one a typical picture of a posterior fossa tumor; the others associated with hypothalamic pituitary signs and intracranial hypertension: visual signs are paramount and have a particularly rapid evolution. Simultaneously, neuroradiology is incapable of localizing these tumors, which almost totally obstruct the third ventricle, masquerade as retrochiasma tic craniopharyngiomas. It is especially the clinical evolution which is characteristic, marked by very rapid recurrences: in 1 case, 1 year, 4 months and 3 months; in the other, 18 months, 12 months and 4 months; 2 months with radiologic appearance of extensive calcifications and a marked tendency to invade the posterior hypothalamus. The limits are difficult to determine: in one recurrence operated under double control of microscope and image intensification radio graphy, immediate postoperative tomography showed calcified areas which had escaped notice at operation. Their prognosis is, therefore, grave. Shunting can rapidly become necessary. Complementary radiotherapy has not seemed to modify signifi cantly their evolution. Is this a confirmation of a special pathologic charac teristic? It is a hypothesis that must be explored.

The role of neurosurgery in the treatment of craniopharyngiomas is incontestable, but we should not attempt invariably to perform a total resection.


Comment

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It is difficult, if not impossible, to assure complete tumor excision and endocrine function may be further impaired. The location of the tumor seems to determine clinical and neuroradiologic appearance. The operative possibilities and implications, and the opera tive and postoperative risks, are directly related to the position of the tumor. We may now begin to entertain some hope of preserving hypothalamopituitary function. Modern serum testing (LH-RH) allows dissociation of ante rior hypothalamic and pituitary dysfunction, enabling us to better assess the necessary operative treatment. This point seems essential in the single intrasellar forms of tumor which are accessible to transphenoidal resection. Operative microscopy permits occasional selective excision with conservation of all or part of the pituitary (3 cases out of 10). Results without supplementary treatment are encouraging and strongly support our current operative approach. Craniopharyngioma, with predominantly prechiasmatic expansion, creates local conditions which render them particularly accessible to subfrontal excision, facilitated greatly by modern anesthesia techniques. Excision may appear to be complete in a large percentage of cases, but this should remain doubtful since we have seen occasional recurrences. Complete excision is not always desirable because it may worsen preoperative problems: dia betes insipidus and other permanent endocrine deficits. The young patients must have every chance to grow normally and achieve sexual maturity. This implies the use of visual magnification techniques for selective surgery, risk ing a certain incidence of recurrence. Judicious combination of subfrontal approach, possibly followed by a transphenoidal operation may facilitate optimal results. In all cases, the unpredictable onset of visual signs, and their irreversibility once present, demands prompt operation. Progress is needed in investigations enabling distinctions of pituitary and anterior hypothalamic dysfunction and also in the quality of supplementary treatment. The posteriorly expanding forms which may obstruct the third ventricle, invade the posterior hypothalamus and compress the brain stem remain most troublesome. Surgery must sometimes be deferred. Unfortunately, tumor evolution may make operation unavoidable. We know that except for certain cases, an attempted complete excision has considerable risk of mortality, with no certainty that recurrence will not occur, and at the price of a consistently poor functional result. The most logical approach is to operate serially, by different routes, guided by clinical evolution and neuroradiologic assessment of expansion. These forms prompt us to consider other therapeutic tech-



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niques (e.g., radioactive implants) which we do not commonly use, or, maybe, to associate them with surgery. It will be interesting to see the histologic appearance after such treatment. One wonders whether it might be possible to impede evolution towards keratinization (and thence calcification), which occurs more frequently in young patients and could partially explain the difference in most of the adult forms. This would also be especially interesting in certain giant forms which seem resistant to every kind of treatment.

Conclusion

An important first step has been taken in showing that a rigorous neuro surgical approach has succeeded in a significant proportion of patients. A new technique of selective neurosurgical dissection promises to facilitate improved functional results. We have thought it important to discuss frankly the errors and risks involved in our previously rigorous approach towards complete tumor excision and thus the reasons for current more conservative attitudes.

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Banna, M. : Craniopharyngioma in adults. Surg. Neurol. 1: 202-204 (1973). Beaufils, F.; Nedcy, R., and Chapman, A.: Postoperative control of water and electrolyte balance after hypothalamo-hypophyseal surgery. 1st Wld Congr. Intensive Care, London 1974, Abstract, p. 90. Blacklund, E.D. : Studies on craniopharyngiomas. Stereotaxic treatment with intracystic Yttrium 90. Acta chir. Scand. 139: 237-247 (1973). Dandy, W.E.: The brain, vol. 1, pp. 596-599 (Prior Company, Hagerstown 1966). Guiot, G. : Transphenoidal approach in surgical treatment of pituitary adenomas. An experience of 504 cases ; in Kohler and Ross, Diagnosis and treatment of pituitary tumors, pp. 159-178 (Excerpta Medica-American Elsevier, Amsterdam 1973). Guiot, G. et Thibaut, B. : L’extirpation des adénomes hypophysaires par voie trans sphénoïdale. Neurochirurgia 1: 133-150 (1959). Hoff, J.T. and Patterson, R.H., jr. : Craniopharyngiomas in children and adults. J. Neurosurg. 36: 299-302 (1972). Kahn, E.A.; Crosby, E.C.; Schneider, R.C., and Taren, J.A.: Correlative neuro surgery, 693 pp. (Thomas, Springfield 1969). Kahn, E.A. ; Gosch, H .H .; Seeger, J.F., and Hicks, S.P. : Forty five years experience with the craniopharyngiomas. Surg. Neurol. 1: 5-12 (1973).


References


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Katz, E.L.: Late results of radical excision of craniopharyngiomas in children. J. Neurosurg. 42: 86-90 (1975). Kramer. S.: Soutard, M., and Mansfield, C.M.: Radiotherapy in the management of craniopharyngiomas. Further experiences and late results. Am. J. Roentg. 103: 44-52 (1968). Kramer, R.A.; Poole, G.J.; Dixon, M., and Newtont, T.H.: Angiography in cranio pharyngiomas. Radiology 109: 99-103 (1973). Lindgren, E. and Di Chiro, G.: Suprasellar tumors with calcification. Acta radiol. 36: 173-195 (1951). Long, D.M. and Chou, S.N.: Transcallosal removal craniopharyngiomas within the third ventricle. J. Neurosurg. 39: 563-567 (1973). Matson, D.D. and Crigler, J.F.: Management of craniopharyngioma in childhood. J. Neurosurg. 30: 377-390 (1969). Raimondi, A.J.: Pediatric neuroradiology, vol. 1, pp. 571-591 (Saunders, Phila delphia 1972). Rougerie, J. et Fardeau. M. : Les craniopharyngiomes, 214 pp. (Masson, Paris 1962). Rougerie, J.: Intra- and para-sellar tumors in children (113 cases) in Proceedings from Gottingen 1973. Thomas, B.; Loflfredo, V. et Merger, R.: Craniopharyngiome avec aménorrhée secondaire. Guérison après exérèse par voie transsphénoïdale. Soc. fr. Gynéc. Obstét., 1972.

Dr Jacques Rougerie, Centre médico-chirurgical Foch, 40, rue Worth, F-92000 Suresnes (France)


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What Should be Expected from Medical Congresses?

What can be expected from antimicrobial de-escalation in the critically ill?

Lifestyle modifications and erectile dysfunction: what can be expected?

[What benefit and harm can be expected from screening and routine examinations and from their omission?].

Aggressive surgical management of craniopharyngiomas in children.

Surgical resection for hepatocellular carcinoma: Moving from what can be done to what is worth doing.

Intraventricular craniopharyngiomas: surgical management and outcome analyses in 24 cases.

What can be learnt from a snail?

Surgical treatment of bronchial obstruction in primary tuberculosis in children: report of seven cases.

The treatment of craniopharyngiomas.

What lessons can be learned about asthma phenotypes in children from cohort studies?

What can be learned from reporting no-treatment effect of distribution of upper limb training?

Suicide--what can be done?

[Surgical treatment of acetabular fractures: report of 22 cases].

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What is to be expected from an ethics audit integrated within the accreditation process of hospitals from romania?

What Should Be the Treatment Protocol in Cases of Second and Third Degree Hemorrhoids?

The role of infection in childhood leukemia: what can be learned from the male predominance?

Practical guidance on immunogenicity to biologic agents used in the treatment of psoriasis: What can be learnt from other diseases?

Endodontic problems resulting from surgical fistulation: report of two cases.

Prenatal diagnosis: can what counts be counted?

Surgical treatment for late-appearing adrenal metastasis from gastric cancer: report of two cases.

Vaccination in the elderly: what can be recommended?

Exploratory spatial analysis of Lyme disease in Texas -what can we learn from the reported cases?