The endoscopic endonasal approach for the management of ......solari THe enDoscoPic enDonasal...

454 Journal of neurosurgical sciences December 2016

anno: 2016Mese: DecemberVolume: 60no: 4rivista: Journal of neurosurgical sciencescod rivista: J neurosurg sci

lavoro: titolo breve: THe enDoscoPic enDonasal aPProacH for cranioPHarYngioMasprimo autore: solaripagine: 454-62citazione: J neurosurg sci 2016;60:454-62

giomas tend to involve and adhere to a great number of vital neurovascular structures, at the level of the sella and surrounding areas of the skull base, including the optic nerves, internal carotid arteries (icas), the third ventricle — above all its floor —, the hypothalamus and pituitary gland, causing a variety of symptoms.

Typical presenting signs are represented by visual dysfunction, with symptoms of chiasmatic and/or ret-rochiasmatic compression, hypothalamic dysfunction, such as behavioral changes, alterations of eating pat-terns, apathy, or even stupor, and pituitary dysfunction, often figuring out as panhypopituitarism.2

craniopharyngiomas are disembryogenetic tumors — considered benign according to WHO classifi-

cation — that origin from squamous epithelial remnants of Rathke’s pouch; indeed, they can develop from any segment of its course, virtually from rhino-pharynx to the hypothalamus.1, 2 They account for 1.4-4.7% of all intracranial tumors (Central Brain Tumor Registry of the United States), mostly affecting childhood (mean age 5-14 years) and late adulthood (mean age 50-74 years).1, 3 These tumors appear cystic, solid or as com-bination of both, being intralesional calcifications often observed (around 60% to 80% of cases). Craniopharyn-

R E V I E WE N D O S C O P I C S K U L L B A S E S U R G E R Y

The endoscopic endonasal approach for the management of craniopharyngiomas

Domenico solari 1, roberta Morace 2, Luigi M. CAVALLO 1, francesca aMoroso 3, gilda cennaMo 3, Marialaura Del Basso De caro 4, Paolo caPPaBianca 1 *

1Division of Neurosurgery, Department of Neurosciences, Reproductive and Odontostomatological Sciences, Università degli Studi di Napoli Federico II, Naples, Italy; 2Department of Neurosurgery, IRCCS Neuromed, Pozzilli, Isernia, Italy; 3Eye Clinic, Department of Neurosciences, Reproductive and Odontostomatological Sciences, Università degli Studi di Napoli Federico II, Naples, Italy; 4Department of Advanced Biomedical Sciences, Università degli Studi di Napoli Federico II, Naples, Italy*Corresponding author: Paolo Cappabianca, Division of Neurosurgery, Università degli Studi di Napoli Federico II, Via Pansini 5, 80131 Naples, Italy. E-mail: [email protected]

a B s T r a c TCraniopharyngiomas are disembryogenetic, benign, tumors that origin from squamous epithelial remnants of Rathke’s pouch, developing from any segment of its course, virtually from rhino-pharynx to the hypothalamus. Historically, different microscopic transcranial routes, have been advocated as possible surgical options for the treatment of craniopharyngiomas. The endonasal technique offers a direct approach that permits access to the suprasellar, retrosellar and retroclival space, obviating brain retraction; it provides the advantage of appraoching cranioopharyngi-omas without optic nerve manipulation and/or retraction. We herein present the surgical nuances of the endoscopic endonasal approach for the treatment of craniopharyngiomas, highlighting hints, advantages and drawbacks, also in regards of the anatomy dealt with. The endoscopic en-donasal technique has been emerging as a viable approach/alternative for the treatment of this disease as the endoscope itself increased its safety and effectiveness. It allows the removal of both infra and supradiaphragmatic lesions — eventually involving the third ventricle chamber but not extending laterally off the ICA out of the visibility and maneuverability of the instruments — avoiding brain and optic nerve manipulation and retraction, with good visualization of the pituitary gland and stalk and the main neurovascular structures.(Cite this article as: solari D, Morace r, cavallo lM, amoroso f, cennamo g, Del Basso De caro M, et al. The endoscopic endonasal approach for the management of craniopharyngiomas. J Neurosurg Sci 2016;60:454-62)Key words: Craniopharyngioma - Endoscopy - Skull base – Surgery.

Journal of neurosurgical sciences 2016 December;60(4):454-62© 2016 eDiZioni MinerVa MeDicaOnline version at http://www.minervamedica.it

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2016 EDIZIONI MINERVA MEDICA

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THe enDoscoPic enDonasal aPProacH for cranioPHarYngioMas solari

Vol. 60 - No. 4 Journal of neurosurgical sciences 455

lavoro: titolo breve: THe enDoscoPic enDonasal aPProacH for cranioPHarYngioMasprimo autore: solaripagine: 454-62citazione: J neurosurg sci 2016;60:454-62

of incomplete resection and increased risk of mortality and morbidity.5, 20, 21, 24, 25

Recurrences have been reported also after apparently complete tumor resection and negative postoperative brain imaging; on the other hand this shed light on the possibility of considering non radical surgery combined with other therapeutic approaches, in order to achieve long-term control.26-37

Hence radiation therapy,34 stereotactic placement of a draining catheter,36 transsphenoidal insertion of the catheter into the cyst for the cyst content drain-age,36, 38 wide marsupialization of the cysts into CSF spaces (cysto-ventriculo-cisternostomy) with neuroen-doscopic technique 39 stereotactic aspiration with the in-stillation of bleomycin 40 or interferon alpha, intracystic radiotherapy 32, 41, 42 or chemotherapy for cystic lesions can be used as viable alternatives.

Historically, different microscopic transcranial routes, namely subfrontal, frontolateral and pterional routes have been advocated as possible surgical op-tions for the treatment of craniopharyngiomas. The use of transsphenoidal approach was introduced in the early 1960’s by Gerard Guiot,43 being this technique reserved only for lesions preferably with a cystic com-ponent, with a minimal supradiaphragmatic extension in patients with an enlarged sella, preferably with pan-hypopituitarism. These strict indications have lasted for over three decades and only later, thanks to the evolu-tion of surgical techniques and technology, resulting in improved effectiveness along with decreased morbid-ity, it has been possible to access the suprasellar area and remove extrasellar craniopharyngiomas via the endonasal corridor.44-47 This technique, described and termed by Weiss in 1987 as “extended transsphenoidal approach”,48 created the possibility to gain access to the suprasellar space from a ventral route.

The endoscope perfectly suited this scenario: the wide and panoramic view offered by the endoscope pushed the development of a variety of modifications of the endona-sal approaches expanding the targeted area to the whole median and paramedian skull base.16, 49-51 The endonasal technique offers a direct approach that permits access to the suprasellar, retrosellar and retroclival space, obviating brain retraction; since craniopharyngiomas grow along a vertical axis, it provides the advantage of accessing these lesions immediately after suprasellar dural opening with-out optic nerve manipulation and/or retraction.

From histological point of view, two major variants have been identified: the papillary occurring almost in adults and the adamantinomatous, most frequent in childhood, being these latter much more common than the other one (ratio: 9/1).4, 5 regarding macroscopic fea-tures, the adamantinomatous subtype shows irregular interface, adhesions to surrounding structures, and cys-tic contents have dark ‘motor-oil’ fluid, with cholesterol crystals inside; calcification may occur in the majority of cases. On the contrary, the papillary form shows no adherence to surrounding structures and/or calcifica-tions, while cystic contents are often clear. Furthermore it should be highlighted that the adamantinomatous sub-type demonstrates positivity for CK7, CK8, CK14 6-8 and beta-catenin.9-11 Recently, these membrane proteins have been claimed responsible for the aggressiveness of the adamantinomatous tumor histotype, as they actively coordinate the infiltration of the tumor into surrounding tissues.12, 13

Thus far, these unpredictable features and biologi-cal behavior, along with anatomical relationships that craniopharyngiomas establish, represent a key aspect to be considered when defining surgical management of these lesions. Surgical lesion removal maneuvers could eventually determine visual impairment, endocri-nological disturbances, namely diabetes insipidus and hypopituitarism, and/or hypothalamic disturbances, resulting in impairment of social and behavioral dis-turbances.

Along years, several authors advocated different clas-sifications according to the growth path and the surgical route used, as related to the optic chiasm, diaphragma sellae, third ventricle and infundibulum.14-19

regardless of techniques adopted, complete removal at first surgical attempt has been suggested as the most effective treatment.5, 14, 20, 21 Nevertheless, it may not al-ways be possible due to the tumor’s inner features and or location and anatomical relationships, or it can be surgeon’s peer choice.22 indeed, in pediatric patients it may be preferred a lower radical surgical resection — above all when hypothalamus is involved — in order to spare hypothalamic functions.23

However, craniopharyngiomas can recur even after radical resection and the surgical treatment of a re-current lesion results even more troublesome due to scar formation along with the loss of the gliotic reac-tion.5, 20, 21 These factors inevitably lead to higher rates

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solari THe enDoscoPic enDonasal aPProacH for cranioPHarYngioMas


setting will be adopted in case of left-handed surgeon.as per microsurgical paradigm, bimanual dissection

is performed under dynamic visual control between close-up and panoramic views.49, 53, 54

Standard craniopharyngioma surgery

Several lesions, namely infradiaphragmatic cranio-pharyngiomas, can be operated via a “standard” ap-proach, as described for pituitary adenomas 47, 55, 56 avoiding in most of the cases the middle turbinectomy or the needs to harvest a naso-septal flap. This proce-dure is reserved to those patients with enlargement of the sella, preferably cystic extra-arachnoidal infradia-phragmatic tumors,38, 44, 57-59 eventually with minimal supra 60 and retrosellar median 45 extension, and more suitable when hypopituitarysm has already developed.59

Extended craniopharyngioma surgery

On the other side, in cases of craniopharyngiomas with prevalent extension in the supradiaphragmatic space (intracranial compartment), the so-called extend-ed technique is adopted.

Bone is removed off the superior half of the sella and the planum sphenoidale is opened up to the level of the posterior ethmoidal arteries or according to the ante-rior margin of the tumor, defined by image-guidance. a complete removal of the tuberculum sellae, i.e. the suprasellar notch,61 including mocrs (medial opto-ca-rotid recess) is accomplished to access suprasellar area and gain adequate exposure for tumor resection.

Bleeding from the superior intercavernous sinus dur-ing the removal of tuberculum sellae 16, 50, 51, 62, 63 is ex-pected and can be managed with the use of hemostatic agents or, preferably, the sinus should be closed with the bipolar coagulation and transected: the two dural sinus leaves are further coagulated to obtain their retraction and though proceed with dural opening over the pla-num.

Tumor management responds to the same principles and goals of the microsurgical procedure: internal de-bulking of the solid part and/or cystic evacuation, fol-lowed by dissection of the tumor from the main sur-rounding neurovascular structures. Endoscopic direct visual control provides an extra value in terms of effec-tiveness 53 with the opportunity a close-up and a wider

Backing upon our experience with endoscopic en-donasal technique on more than 1500 cases we herein present the surgical nuances of this approach for the treatment of craniopharyngiomas, highlighting hints, advantages and drawbacks, also in regards of the anat-omy dealt with.

Surgical technique

The endoscopic endonasal approach for the removal of a craniopharyngioma is run using a rigid endoscope (0 degrees), 18 cm in length 4 mm in diameter (Karl storz® & Co, Tuttlingen, Germany), as the sole visualiz-ing tool along the whole procedure. Dedicated surgical instruments are required, and some additional tools are crucial to improve safety and effectiveness.

This is a 2-surgeon, 3-or 4-handed technique: requir-ing a duo usually an ENT or Head and neck surgeon and a neurosurgeon.49-51

The otolaryngologist performs the nasal steps of the approach and then drives the endoscope “dynamically”, while the neurosurgeon performs a bimanual dissection and tumor removal.

Each surgeon looks into a dedicated monitor, adjusted in front of him-her at personalized height and distance.

The patient, under general anesthesia, is placed su-pine or in slight Trendelenburg position, with the head extended about 10-15 degrees on the sagittal plane.

adequate surgical corridor 51 is achieved by displac-ing laterally a middle turbinate on one side and remov-ing the controlateral one — its mucopericondrium graft will serve for the skull base repair — in the nostril where the endoscope will be inserted and thereafter and further enlarged by mean of a tailored bilateral ethmoidectomy.

When performing refining of bone opening, care should be taken to not damage the sphenopalatine artery branches and the posterior ethmoidal arteries.

The harvesting of a vascularized nasal septal flap (Hadad-Bassagasteguy Flap) 52 could be performed at this point: nevertheless, we retain it is better to draw mucosa incision and then raise it off at the end of the surgical procedure.53

The final working set-up is reached: whether the sur-geon is right-handed, the endoscope is placed in the su-perior aspect of patient’s right nostril (12 o’clock) to allow suction insertion inferiorly (6 o’clock), while the main instruments will be in the left nostril; the opposite

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be transected, the pituitary gland be transposed 64 or a double corridor — above and below the gland 65 — be created.

Because craniopharyngiomas are often adherent to the chiasm and/or hypothalamus, or third ventricle floor, particularly in cases of recurrence,25 it is advisable “not to force” resection in order to achieve “maximum allowed” surgical outcome and above all maintain tis-sue integrity function (Figure 2).

after lesion removal an accurate reconstruction of the skull base defect is required; it should be not un-derestimated that this step could result troublesome be-cause endoscopic endonasal approach for craniopharyn-giomas requires wider osteo-dural opening, along with

panoramic view, while completing dissection maneu-vers. Extracapsular dissection 44 can be safely accom-plished approaching the tumor from its ventral aspect, thanks to direct visualization of the inferior aspect of the chiasm, the infundibulum, the third ventricle and/or the retro and parasellar spaces (Figures 1, 2).

The stalk-infundibulum complex with arterial branches of hypophyseal arteries stand at the center of the surgical field: it is pushed off, posteriorly by pre-infundibular lesions, representing a sort of capsule in case of lesions growing along stalk infundibular axis, or hinder access to the lesion whether these are retroin-fundibular. In cases the stalk is displaced to one side, a very narrow lateral corridor could be adopted, or it can

Figure 1.—Intraoperative pictures showing the removal via endoscopic endonasal approach of an intra-suprasellar, partially cystic craniopharyngi-oma. A) Upon dura opening the so-called “motor oil” fluid is drained; B) the solid part of the tumor is debulked and dissected off the optic chiasm; C) after tumor removal the endoscopic exploration of the subchiasamtic area allows the visualization of the pituitary stalk and the outer aspect of the floor of the third ventricle.*Motor oil fluid; ON: optic nerve; T: tumor; Ch: chiasm; PS: pituitary stalk.

Figure 2.—Intraoperative pictures showing the removal via endoscopic endonasal approach of an infundibulo-intravertricular craniopharyngioma. A) Dissection maneuvers are carried out in the infundibular area with bimanual technique; B) tumor is followed and removed off the third ventricle; C) close up view of the third ventricle floor and chamber after tumor removal.Ch: chiasm; T: tumor; Th: thalamus; FThV: floor of the third ventricle.

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guidance technologies and modern instrumentation.Recently, “pure” endoscopic endonasal procedures

targeted to different areas and lesions of the skull base have been defined:49, 51, 63, 71, 73, 78-81 this technique pro-vides a median and direct visualization of the suprasel-lar space with extra advantages as related to both the corridor and the endoscope itself. Above all, the wider, close-up view of the surgical field grants better ana-tomical understanding, and though safer dissection and removal maneuvers, without any brain retraction and optic apparatus manipulation. Focusing on this latter aspect, recently the use of Optical Coherence Tomog-raphy has been claimed for possibility of predicting the optic nerve status and thus far defines indications and outcomes.82-84

Hence, several authors adopted this technique for the surgical treatment of supradiaphragmatic lesions, in-cluding craniophayngiomas, defining specific lesions’ features amenable of this surgery, describing its pros and cons in terms of removal rate, surgical outcomes and quality of life.25, 53, 62, 79, 85-91

Along with the growing experience and the increas-ing number of cases treated via the endoscopic endo-nasal approach, the technique has been refined and im-proved according to the different craniopharyngiomas morphological features and location, and in regards of the anatomy dealt with.

Accordingly, we noted that in cases of intra-suprasel-lar infradiaphragmatic lesions - eventually compressing the medial walls of cavernous sinus or eroding the dor-sum sellae - a higher likelihood of gross-total removal could be reported.64, 65, 92

Conversely, when dealing with supradiaphragmatic lesions, differences in terms of removal should be at-tributed to the location and to the involvement of the neurovascular structures, above all the optic chiasm, the third ventricle and the stalk-infundibulum axis and eventual pial invasion, which definitely hinder a safe radical removal.58

Craniopharyngiomas growing anteriorly or within the stalk-infundibulum axis, extend along the same tra-jectory of the endonasal corridor, being though easily removed via this corridor as compared to those that pre-sented with a retroinfundibular growth path. We noticed that the possibility of accomplishing a safe dissection varies upon the involvement of pre and/or post chias-matic areas; lesions involving both spaces with large

large opening of the arachnoid cisterns and/or of the third ventricle and/or Liliequist membrane. It is man-datory though to perform the reconstruction according to Kelly’s Scale, acting as for Grade 3 leakage 66 in the attempt of obtain: 1) intradural sealing of the arachnoid; 2) resilient closure of the osteo-dural defect; 3) stability and integration of the materials.

Usually, a thin layer of fat and fibrin glue (Tisseel®, Baxter, Vienna, Austria) is positioned intradurally as first barrier to CSF filling the post-surgical cavity. Thereafter, the closure of the osteo-dural defect could be achieved mainly according to two different strate-gies: 1) the so-called “gasket seal” or “grandma’s cap“ technique, in which a single layer of the dural substitute is positioned in the extradural space 67, 68 with a tailored foil of resorbable semi-solid material overlapped in order to fix the first one in the extradural space; 2) in the “sandwich” technique three-layers foil of dural sub-stitute with the fat sutured to its inner aspect is placed both intradurally (the fat and two layers) and extradu-rally (the outer sheath of dural substitute). Vascularized Hadad pedicled flap 52, 69 and, eventaully, free mucho-perichondrium flap are then placed over the posterior wall of the sphenoid sinus. Fibrin glue and oxidized cel-lulose are used to ease adherence of muchosa flap over bony surface and hold the material in place.

Discussion

Craniopharyngiomas are considered very difficult le-sions to treat, due to an extremely variable growth pat-tern along with an unpredictable behavior: a univocal surgical management could not be advocated and this matter still represents one of the most debated in mod-ern neurosurgery.

Transcranial microsurgical approaches are currently adopted in clinical practice for the removal of such tu-mors, weighing in on the fact that they often involve the suprasellar and ventricular areas. Along the years, the trans-sphenoidal approach has been limited only for the intra-suprasellar infradiaphragmatic lesions.44, 45, 70 The ideation of endonasal approaches to the skull base has revolutioned this paradigm with the possibility of accessing median and paramedian lesions via a direct corridor; this kind of surgery brought inner advan-tages,46-48, 71-77 further amplified by the introduction of several innovative tools such as the endoscope, image

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mean of extended endoscopic endonasal approach, the same problems of re-do surgery have been observed.

It is of utmost importance to remember that however the effectiveness of the surgery could be limited if le-sions present an eccentric extension off the median and paramedian skull base — i.e. beyond ICA or abutting in the middle cranial fossa, however out of the safe range of visibility and maneuverability — or presenting en-casement or tight adherence to the neurovascular struc-tures. In these conditions, a two-stage surgical strategy could be adopted with the endonasal approach compli-mentary to the transcranial, with each route being ad-opted to overcome the limitations of the other.16, 53, 54

Finally, it has to be said that postoperative CSF leak represented the most dreaded complication, being this latter one of the main issues of this technique.66-69, 97 However, the recent development of different methods of skull base reconstruction, along with the use of new materials, and the vascularized flaps 52, 53, 68, 69, 98-100 have contributed to reduce considerably the risk of this event.

Conclusions

Craniopharyngioma surgery is still figured out as a major challenge in modern neurosurgery, due to the unique nature and unpredictable attitude of these tu-mors. The endoscopic endonasal technique has been emerging as a viable approach/alternative for the treat-ment of this disease as the endoscope itself increased its safety and effectiveness. It allows the removal of both infra and supradiaphragmatic lesions — eventually in-volving the third ventricle chamber but not extending laterally off the ICA out of the visibility and maneuver-ability of the instruments — avoiding brain and optic nerve manipulation and retraction, with good visualiza-tion of the pituitary gland and stalk and the main neuro-vascular structures.

This technique with its variations, whose indications respond to proper selection criteria, have to be taken into account among surgical strategies for the manage-ment of craniopharyngiomas.

References

1. Jane JA Jr, Laws ER. Craniopharyngioma. Pituitary 2006;9:323-6. 2. Karavitaki N. Management of craniopharyngiomas. J Endocrinol In-

vest 2014;37:219-28. 3. Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F,

parasellar extension are not easily dissected from ves-sels and nerves, so that a transcranial route should be considered alone or as a part of a staged surgery.

furthermore, the endoscopic endonasal route grants the possibility of managing effectively and safely tu-mors extending into the third ventricle: the direct and close-up view consents to identify the degree of tumor hypothalamic/third ventricle pial invasion 16, 17, 19, 89, 92-95 and accordingly dissect or not tumor off these struc-tures. Radical resection of these lesions — especially when the involvement extends posteriorly to the mam-millary bodies — should be not attempted to avoid tre-mendous injuries.

Owing that, particularly in pediatric patients in some cases we prefer to realize a subtotal surgical resection, in order to balance the need of an adequate psychic and motor development and reduce the risk of postoperative hypothalamic obesity.23, 89

In these regards it is worth underlining that adjunc-tive radiotherapy could be a viable option to achieve a long-term disease control, in cases of subtotal removal due to these increased risks.96

nevertheless, postoperative irradiation has to be peerly indicated: in cases of small residual tumors, adherent to neurovascular vital structures, eventually with calcified fragments, not growing, a watchful wait-ing strategy with close neuroradiological follow-up can be adopted; in case of rapidly growing residual tumors an early second surgical procedure can be preferred to achieve relief from syptoms.47, 53, 89

Besides, we identified several conditions/features that is worth to discuss whether a recurrent craniopha-ryngioma is approached via an endoscopic endonasal route:25

— whether patient underwent previous transcranial surgery, the endonasal route offers a naïve corridor: the most inferior and posterior portions of the tumor that were not properly managed through the corridor from above are easily accessed via a ventral approach; con-versely, its prechiasmatic aspect is difficult to remove, being burdened by the arachnoidal scars;

— when a previous standard transsphenoidal ap-proach has been done, the endoscopic endonasal tech-nique is favored in cases of intra-suprasellar prechias-matic lesions because of the possibility of dealing with intact arachnoidal plane;

— in patients that have been already operated on by

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Conflicts of interest.—The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript.Article first published online: June 7, 2016.

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