Outcomes following endoscopic, expanded endonasal resection of suprasellar craniopharyngiomas: a case series

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Object

Craniopharyngiomas are challenging tumors that most frequently occur in the sellar or suprasellar regions. Microscopic transsphenoidal resections with various extensions and variations have been performed with good results. The addition of the endoscope as well as the further expansion of the standard and extended transsphenoidal approaches has not been well evaluated for the treatment of this pathological entity.

Methods

The authors performed a retrospective review of all patients who underwent a purely endoscopic, expanded endonasal approach (EEA) for the resection of craniopharyngiomas at their institution between June 1999 and February 2006. Endocrine and ophthalmological outcomes, extent of resection, and complications were evaluated.

Results

Sixteen patients underwent endoscopic EEA for the resection of craniopharyngiomas. Five patients (31%) presented with recurrent disease. Complete resection was planned in 11 of the 16 patients. Three elderly patients with vision loss underwent planned debulking, 1 patient with vision loss and a moderate-sized tumor had express wishes for debulking, and 1 patient had a separate, third ventricular nodule that was not resected. Of those in whom complete resection was planned, 91% underwent near-total (2/11) or gross-total (8/11) resection. No patient who underwent gross-total resection suffered a recurrence. The mean follow-up period was 34 months. Of the 14 patients who presented with vision loss, 93% had improvement or complete recovery and 1 patient's condition remained stable. No patient experienced visual worsening. Eighteen percent of patients (without preexisting hypopituitarism) developed panhypopituitarism and 8% developed permanent diabetes insipidus. There were no cases of new obesity. The postoperative cere-brospinal fluid leak rate was 58%. All leaks were resolved, and there were no cases of bacterial meningitis. There was 1 vascular injury (posterior cerebral artery perforator branch) resulting in the only new neurological deficit. No patient died.

Conclusions

Endoscopic EEA for the resection of craniopharyngiomas provides acceptable results and holds the potential to improve outcomes.

Abbreviations used in this paper: CSF = cerebrospinal fluid; DI = diabetes insipidus; EEA = expanded endonasal approach; GH = growth hormone; GKS = Gamma Knife surgery; ICP = intracranial pressure; PCA = posterior cerebral artery; TSH = thyroid-stimulating hormone; VP = ventriculoperitoneal.

Abstract

Object

Craniopharyngiomas are challenging tumors that most frequently occur in the sellar or suprasellar regions. Microscopic transsphenoidal resections with various extensions and variations have been performed with good results. The addition of the endoscope as well as the further expansion of the standard and extended transsphenoidal approaches has not been well evaluated for the treatment of this pathological entity.

Methods

The authors performed a retrospective review of all patients who underwent a purely endoscopic, expanded endonasal approach (EEA) for the resection of craniopharyngiomas at their institution between June 1999 and February 2006. Endocrine and ophthalmological outcomes, extent of resection, and complications were evaluated.

Results

Sixteen patients underwent endoscopic EEA for the resection of craniopharyngiomas. Five patients (31%) presented with recurrent disease. Complete resection was planned in 11 of the 16 patients. Three elderly patients with vision loss underwent planned debulking, 1 patient with vision loss and a moderate-sized tumor had express wishes for debulking, and 1 patient had a separate, third ventricular nodule that was not resected. Of those in whom complete resection was planned, 91% underwent near-total (2/11) or gross-total (8/11) resection. No patient who underwent gross-total resection suffered a recurrence. The mean follow-up period was 34 months. Of the 14 patients who presented with vision loss, 93% had improvement or complete recovery and 1 patient's condition remained stable. No patient experienced visual worsening. Eighteen percent of patients (without preexisting hypopituitarism) developed panhypopituitarism and 8% developed permanent diabetes insipidus. There were no cases of new obesity. The postoperative cere-brospinal fluid leak rate was 58%. All leaks were resolved, and there were no cases of bacterial meningitis. There was 1 vascular injury (posterior cerebral artery perforator branch) resulting in the only new neurological deficit. No patient died.

Conclusions

Endoscopic EEA for the resection of craniopharyngiomas provides acceptable results and holds the potential to improve outcomes.

The microscopic, transsphenoidal approach for the resection of certain craniopharyngiomas is well established and generally accepted,6,13–15,17,20–24,29,30 and there are several studies comparing this approach with a transcranial approach.21,24,30 Although the transsphenoidal approach is traditionally limited to predominantly sellar lesions with secondary suprasellar extension,23 the development and application of the “extended approach” have provided greater access to suprasellar lesions.17,20,25 Despite this modification, outcomes from the transsphenoidal approach for the resection of suprasellar tumors with adherence to critical surrounding structures have led to more frequent recurrences and poorer overall outcomes compared with purely sellar tumors.17 Some of the limitations of this approach, as previously described, may be secondary to the use of the microscope,2 distance from the surgical target, the small portal of entry to the surgical field (that is, the nostril), and the need for a speculum. Attempting to remedy these issues, in 1979 Halves and Bushe10 recognized the facilitatory role of the endoscope when accessing supra-sellar lesions. To date, however, the endoscope has played a limited role in most studies of craniopharyngioma, and there are few studies7 in which the outcomes following endoscopic, endonasal craniopharyngioma resection have been evaluated.

We present a series of 16 cases in which we undertook a purely endoscopic EEA for the resection of craniopharyngiomas. Short-term ophthalmological and endocrine outcomes along with extent of resection were examined. This report is meant to be an early evaluation of the technique's value in the management of these formidable lesions. By comparing these results with those previously published for other approaches, we hope to better judge both approaches in terms of their respective advantages and disadvantages.

Although authors of many past studies have assessed craniopharyngioma resection via a transsphenoidal route,2,17,23,24,29 few7 have used a purely endoscopic technique and most are biased toward the resection of tumors with minimal supra- or parasellar extension. Our series consists of tumors located above and/or with extension superior and lateral to the sella, superior and posterior to the chiasm, and even extending into the third ventricle.

Methods

After obtaining institutional review board approval, we reviewed the medical records and imaging studies of all patients with the histopathological diagnosis of craniopharyngioma treated via an EEA between June 1999 and February 2006. No patient was excluded or lost to follow-up, resulting in a series of 16 patients (Table 1). Patients' ages ranged from 36 to 80 years (mean age 55 years). There was a male/ female ratio of 5:3. Five patients (31%) presented to us with recurrent disease. One patient had undergone prior transsphenoidal resection, 4 had prior craniotomies (1 of whom also underwent prior stereotactic cyst drainage with intracystic brachytherapy), and 1 had undergone radiosurgery. In addition, 1 patient underwent a previous transsphenoidal procedure for a pituitary adenoma (histopathology verified). Other complicating conditions included obesity, chronic obstructive pulmonary disease, and pregnancy. Most patients (14 [87.5%] of 16) presented with progressive visual deficit as the primary indication for surgery (Table 2). We report ophthalmological and endocrine outcomes, as well as extent of resection and complications. The follow-up period for this study ranged from 2 to 82 months (mean follow-up 34 months).

TABLE 1

Characteristics in patients who underwent an endoscopic EEA for craniopharyngioma

Case No.Age (yrs), SexDate of Op s(mo/day/yr)CommentTumor Type*
142, M06/08/99noneI
236, F04/12/02presented 14 wks pregnantI
346, F10/21/02previous stereotactic cyst drainage, intracystic brachytherapy & eyebrow craniotomyIIIb
443, M11/05/02noneIIIa & b
550, M05/08/03recurrent tumor (previous bicoronal craniotomy)II
658, M07/16/03obese/severe COPDIIIb
769, M08/20/03transsphenoidal adenoma resection 7/17/02II
834, M08/25/03recurrent tumor (previous bicoronal craniotomy)I
956, F01/26/04noneIIIb
1051, F06/21/04noneIIIa & b
1155, F07/02/04recurrent tumorII
1252, F09/01/04presented w/ increased thirstII
1379, M12/06/04noneIIIa
1480, M01/10/05prior craniotomy SRS; ataxia w/ recurrence (pontine compression)IIIb
1548, M08/08/05panhypopituitarism, gland completely calcifiedII
1679, M02/20/06noneIIIa & b

* Type I denotes preinfundibular; Type II, transinfundibular; and Type III, retroinfundibular (a, rostral extension [interpeduncular cistern]; b, caudal extension [prepontine cistern]). Abbreviations: COPD = chronic obstructive pulmonary disease; SRS = stereotactic radiosurgery.

TABLE 2

Ophthalmological results*

Case No.Preop Deficit/Presenting SymptomPostop Result
1chiasmal compressionimproved
2progressive chiasmal syndromeresolved
3progressive, severe visual lossunchanged/stabilized
4mild–mod chiasmal compressionresolved
5lt > rt optic neuropathyimproved
66-mo history decreased visual acuityresolved
(mild–mod afferent visual defect)
7recurrent chiasmal compressiondelayed postop deficit, improved w/ VP shunt compared w/ preop
8chiasmal compressionimproved
9lt > rt superior temporal quadrantanopiaimproved
10progressive vision lossresolved
11profound vision lossimproved
12vision lossresolved
13vision lossimproved
14no vision loss/brainstem compressionno change
15no vision loss/headacheno change
16vision lossresolved

* mod = moderate.

Operative Technique

We detail the operative technique elsewhere.19 As such, only a brief overview will be provided here. The operations were all performed by 2 surgeons (A.B.K. or P.A.G. and C.H.S. or R.L.C.), a neurosurgeon, and an otolaryngologist. A binarial, purely endoscopic, image-guided approach was used. The patient was placed supine, the head fixed in pins, the neck in a neutral or slightly extended position (10–15°, depending on the anterior/superior extent of the tumor), and the head turned 5–10° toward the surgeon to allow comfortable hand position and improve access. First, oxymetazoline-soaked pledgets were placed into the nares for several minutes, prior to preparation. Then, the image-guided system was calibrated with the patient. We preferred to use MR imaging, but we used CT angiography in cases in which the patient was unable to undergo MR imaging or the bone anatomy or involved vasculature was critical for resection. Next, the abdomen (for fat graft harvest) and midface were prepared with Betadine-soaked sponges. The patient was then draped, leaving only the nose and prepared abdomen exposed. Imaging guidance was once more confirmed, using external landmarks.

Entering the right nostril, the middle turbinate was either lateralized or removed under endoscopic guidance and the anterior wall of the sphenoid was entered. The contralateral nostril was then entered and the sphenoid similarly approached, with lateralization of the middle turbinate. Next, the critical step of partial removal of the posterior internasal septum (~ 1 cm) was performed. This permitted introduction of instrumentation from the contralateral naris without obscuring the endoscopic view. After the posterior wall of the sphenoid sinus was exposed, the image guidance was once more introduced to confirm bone landmarks as well as tumor location. This step is important, as the normal sellar anatomy is often greatly distorted by tumor. The bone over the tumor's dural covering was next drilled and completely removed using angled rongeurs. Through this process, the surrounding anatomy was defined, with special attention given to the location of the cavernous sinuses, carotid arteries, and optic nerves. The bone over the superior inter-cavernous sinus was removed next (if necessary) using a combination of drill and rongeur. At this point, the critical portion of the sellar bone removal, over the medial opticocarotid recess, was carried out as described elsewhere.19 This variable indentation on the sphenoid sinus side of the lateral tuberculum represents the medial confluence of the optic nerve, carotid artery, and anterior skull base, providing a “keyhole” of sorts for this access. When necessary, the superior intercavernous sinus was carefully and completely coagulated using bipolar cautery, prior to its division. This additional rostral exposure often provided the starting point for tumor surgery and/or dural entry, especially when approaching a supra- or retrochiasmatic lesion. In suprasellar lesions, the dura was opened in a cruciate manner, and the tumor was located if not immediately apparent. An extracapsular dissection allowed the surgeon to define tumor as well as key surrounding structures (for example, the optic chiasm and perforating vessels, pituitary gland and stalk, and major arteries). The tumor capsule was then entered, and debulking was performed. Finally, the extracapsular dissection was completed, and the tumor was removed from any adherent normal structures by using sharp dissection. After a final inspection, the dural closure was begun. This reconstruction step has undergone considerable evolution during our experience and continues to be evaluated for improvement. By the end of the series, a lumbar drain was used only in cases in which the third ventricle was entered or in communication as a result of the resection.

Throughout this series, some type of onlay graft, followed by fat graft has been used. At the end of the series, this has progressed to an inlay of DuraGen (Integra Lifesciences Corp.), followed by an acellular dermal onlay graft. The sphenoid sinus was ultimately packed with fat, which was covered with Surgicel (Ethicon) and followed by nasal stents and either pledgets or a Foley catheter balloon as a buttress. Since then, we have begun using a vascularized nasal septal mucosal flap as a final layer over the onlay graft with promising results.9

Results

Ophthalmological Results

All 16 patients underwent a detailed neuroophthalmological examination before and after surgery (Table 2). The conditions of 2 patients without preoperative visual deficits were unchanged postoperatively. The remaining 14 patients had progressive visual deficits preoperatively. Six (43%) of these patients had complete resolution of their visual defect (Fig. 1), and 7 (50%) had improvement but not complete resolution (Fig. 2). One patient's deficit remained stable. It was difficult to determine changes in this patient's vision related to the endoscopic procedure because her vision was extremely poor preoperatively, due in part to unrelated retinal disease. No patient had worsening of vision from optic apparatus damage following surgery. One patient had visual worsening 4 days after surgery secondary to hydrocephalus. His vision improved following the placement of a VP shunt.

Fig. 1.
Fig. 1.

Humphrey visual field tests. A: Preoperative study demonstrating a dense temporal defect. B: Postoperative visual field testing showing resolution of visual deficit.

Fig. 2.
Fig. 2.

Humphrey visual field tests. A: Preoperative study showing dense bilateral defects, greatest bitemporally. B: Postoperative study performed in the same patient demonstrating significant improvement compared with preoperative testing.

Endocrine Results

All 16 patients underwent endocrine evaluation both pre-and postoperatively with complete serum pituitary hormone panels interpreted by an endocrinologist. Five patients suffered from panhypopituitarism (anterior gland) preoperatively (1 had DI), 1 had DI with other deficits, and 1 had isolated DI (Table 3). Only 1 (8%) of 12 patients who underwent long-term follow-up suffered permanent DI. Four others developed temporary DI in the postoperative period that resolved at last follow-up without further treatment. Of the 11 patients without preoperative panhypopituitarism, 2 developed new-onset panhypopituitarism following surgery (18%). One patient had a new, single axis deficit that required TSH replacement. One patient's hormone dysfunction (GH and TSH) resolved following tumor resection. The 7 remaining patients had no change in the anterior gland.

TABLE 3

Endocrine results*

Anterior Pituitary FunctionPosterior Pituitary Function (DI)
Case No.Preop DeficitPostop Deficit (long term)Preop DIPostop/Transient DIPermanent Postop DI
1nonepanhypopitism (except ACTH)noyesyes
2nonepanhypopitismnoyesno
3panhypopituitarismno changenonono
4nonenonenoyesno
5nonenoneyesNANA
6panhypopituitarismpanhypopituitarismnonono
7hypothyroidism & hypogonadismno changenonono
8panhypopituitarismno changeyesNANA
9GH & thyroid axis deficitsnormalnonono
10IGF-I deficit & TSH elevationcontinued hypothyroidism, Hashimoto thyroiditis & papillary carcinoma diagnosednoyesno
11hypothyroidism, borderline adrenal insufficiencyhypothyroid & adrenal insufficiencynonono
12hypothyroidism, adrenal in-sufficiencyno changeyesNANA
13hypogonadism, borderline adrenal in-sufficiency1-mo FU (hypothyroidism at discharge), lost to FUnoyesNA; 1-mo FU (DI at discharge)
14panhypopituitarismno changenonono
15panhypopituitarismno changenoyesno
16thyroid & testosterone deficienciesno changenonono

* ACTH = adrenocorticotropic hormone; FU = follow-up; IGF-I = insulin-like growth factor–I; NA = not applicable.

† The DI was present following a prior craniotomy.

When evaluated by tumor classification, preoperative anterior gland panhypopituitarism or significant dysfunction (Case 12) seemed to be more common in primary (nonrecurrent) Type II tumors (2 of 3, compared with 0 of 2 Type I and 2 of 6 Type III). All patients with Type I tumors suffered from panhypopituitarism postoperatively (1 of these patients had already been suffering from panhypopituitarism). Not surprisingly, the only patient with preoperative DI had a Type II tumor. In addition, permanent DI was more common, although not universal, in postoperative (whether treated with EEA or prior surgery) Type II tumors (3 of 5 [1 new after EEA] vs 1 of 3 [new] Type I and 0 of 8 Type III).

Extent of Resection and Recurrence

Extent of resection was determined using pre- and postoperative volumetric analysis of MR images (Table 4). Fat saturation was used during postoperative contrast-enhanced imaging to remove any confusion between enhancing tumor and fat graft. The comparison was performed by an independent neuroradiologist (S.G.).

TABLE 4

Extent of resection

Vol (cm3)
Case No.PreopPostop% Resection (solid & cystic portion)FU (mos)
11.60.0100.082
23.70.0100.059
39.70.0100.052
45.83.244.3*; cyst progression48
52.50.196.0; regrowth*45
68.30.0100.043
712.03.075.0; regrowth*36
86.60.0100.021
97.20.3 (residual rim enhancement w/in stalk)95.837
104.30.0100.033
116.82.069.8; regrowth15
124.30.4 (residual enhancement w/in stalk)90.7; regrowth29.5
1351.518.364.5*2
1414.10.397.9*10
154.30.0100.018
167.50.0100.010

* Complete resection not planned.

† Small separate enhancing nodule in the lateral wall of the third ventricle not resected.

Evaluation of the series without consideration of goal of surgery has shown that 12 (75%) of the 16 patients underwent at least near-total resection. Two of these had a small amount of residual enhancement within the stalk (Fig. 3) and 2 had < 5% residual tumor, for a gross-total resection rate of 50%. However, complete resection was planned for only 11 of the 16 patients. Of these 11 patients, 8 (73%) underwent gross-total resection (Fig. 4) and 2 other patients (Cases 9 and 12) had only residual enhancement within the stalk (Fig. 3), resulting in 91% (10/11) with gross-total or near-complete (> 95%) resection. The 2 patients with residual stalk enhancement underwent intentional “stalk-sparing” surgery during which tumor was dissected from the stalk, but the stalk was not sacrificed, thus preserving pituitary function. One of these patients suffered regrowth, and the other's condition has remained stable (treated with radiosurgery postoperatively). One patient underwent an unintentional, subtotal (70%) resection. This case was aborted due to arterial (PCA branch) injury. All patients with primary (nonrecurrent) tumors underwent gross- or near-total resection. Six (75%) of 8 patients with primary tumors underwent gross-total resection. The other 2 patients with primary tumors underwent “stalk-sparing” surgery.

Fig. 3.
Fig. 3.

Preoperative (left) and postoperative (right) coronal MR images showing suprasellar, Type II (infundibular) craniopharyngioma treated using a stalk-sparing resection. The enhancement seen under the chiasm has not progressed during follow-up (see text on recurrence).

Fig. 4.
Fig. 4.

Contrast-enhanced T1-weighted MR images. Preoperative sagittal (A) and coronal (B) images showing a complex craniopharyngioma with suprasellar extension. Postoperative sagittal (C) and coronal (D) images showing gross-total resection.

Five patients underwent intentional, incomplete resections/debulking. Three of these patients were > 65 years old with large tumors causing visual compromise (Fig. 5). Two were recurrent tumors. One patient had a large, supra-sellar solid portion as well as a large cyst that extended well into the third ventricle (similar to a Type IV, purely intra-ventricular lesion). He was a highly educated patient and expressed distinct wishes for a subtotal resection with preservation of pituitary function. The cyst was treated separately via stereotactic 32P injection. All patients had relief of compressive symptoms, and residual solid tumor was treated with radiosurgery. One patient underwent complete resection of a suprasellar craniopharyngioma with the exception of a small, separate, enhancing nodule in the lateral wall of the third ventricle (Fig. 6). This area was treated using radiosurgery, but the nodule recurred considerably and was treated using repeated EEA. The decision to merely debulk these tumors rather than attempt complete resection was complex and depended on patient age and overall condition, patient wishes, potential impact of aggressive resection given tumor location (for example, hypothalamic or vascular involvement), and presenting symptoms among other factors.

Fig. 5.
Fig. 5.

Contrast-enhanced T1-weighted MR images. Preoperative sagittal (A) and coronal (B) images showing a giant, complex craniopharyngioma, with extension into the third and lateral ventricles in an elderly man with vision loss. Postoperative sagittal (C) and coronal (D) images showing debulking of this giant tumor. Debulking was chosen due to the patient's advanced age and concern over potential hypothalamic and thalamic injury with aggressive resection.

Fig. 6.
Fig. 6.

Coronal contrast-enhanced T1-weighted MR images. Left: Preoperative image showing a suprasellar craniopharyngioma with a separate nodule (arrow) in the lateral wall of the third ventricle. Resection of the nodule was not planned as the floor of the third ventricle appeared intact on preoperative imaging. In retrospect and with further experience, perhaps this nodule would have been accessible. Right: Postoperative image obtained in the same patient showing resection of the suprasellar tumor, with the third ventricular nodular residual lesion (arrow).

In this series we treated tumor remnants, whether left intentionally or not, using radiosurgery. No patient suffered recurrence in the setting of gross-total resection. Four patients with known residual tumor experienced regrowth. One of these patients, mentioned earlier, with only residual rim enhancement within the stalk, experienced regrowth of the remnant. This patient (Case 12) had a particularly aggressive tumor and underwent repeated endoscopic EEA twice, successfully treating visual symptoms, followed by stereotactic radiosurgery. The patient's tumor has responded to radiosurgery and was noted to be decreasing at last follow-up. Three other patients had regrowth of a known remnant. All 3 were recurrent prior to EEA. The patients in Cases 5 and 11 experienced regrowth and were treated endonasally, and the patient in Case 7 underwent a second resection via a craniotomy due to growth lateral to the middle cerebral artery. The advantage to the endonasal approach lies in treating tumors that are medial to neurovascular structures, thus allowing resection without traversing these structures. This advantage was negated in this situation, making an endonasal approach impractical. This patient eventually died (3 years after EEA) secondary to complications associated with stereotactic cyst treatment.

Postoperative Complications

The most common complication in this series was postoperative CSF leak. There were 11 CSF leaks (58%) during 19 total operations (including 3 reoperations for recurrence/regrowth; 69% not including reoperations) (Table 5). Lumbar drain diversion in the immediate postoperative period is used in all cases requiring extensive arachnoid dissection or ventricular transgression. One leak resolved with lumbar drainage alone, and 10 leaks were managed with reexploration and repair. Long-term CSF diversion was required in 6 (40%) of 15 patients who had lumbar puncture–proven chronic elevation in ICP. Another patient developed delayed visual decline postoperatively because of documented hydrocephalus. This condition resolved to the immediate postoperative, improved state after shunt treatment.

TABLE 5

Surgical complications*

Case No.ComplicationTreatmentResult
1noneNANA
2CSF leakreexplorationresolution
3CSF leakreexploration/VP shuntresolution
4delayed CSF leak w/ HCPreexploration/VP shuntresolution
5noneNANA
6CSF leak w/ chemical meningitis, recurrencereexploration, awaiting reopresolution
7postop HCP/cyst chiasm compression(delayed vision deficit)VP shuntimprovement
8noneNANA
9PCA/P1 injury (avulsion) w/ resultant partial lt thalamic CVA, DVT, delayed CSF leak w/ HCPtherapy & brief rehab hospital stay, anticoagulants, reexploration twice/VP shuntrecovered except trace dysarthria & facial droop, no sequelae, resolution
10CSF leakLD onlyresolution
11CSF leakreexploration/VP shuntresolution
12CSF leak, recurrencereexploration/VP shunt, resection twice (1/05 & 5/05) w/o postop CSF leak, GKSresolution
13CSF leak, DVTreexploration/VP shunt, IVC filter, anticoagulationresolved, then died of SDH while taking anticoagulants
14noneNANA
15CSF leakreexploration/LD placedresolved
16CSF leakreexplorationresolved

* CVA = cerebrovascular accident; DVT = deep venous thrombosis; HCP = hydrocephalus; IVC = inferior vena cava; LD = lumbar drain; rehab = rehabilitation; SDH = subdural hematoma.

The overall shunt placement rate was 7 (44%) of 16, and despite CSF leaks there were no cases of bacterial meningitis. Shunt treatment was not used as a primary modality for CSF leak repair. Rather, it was used as an adjuvant in those patients with recurrent leakage and documented (lumbar puncture) increase in ICP.

Several tumors were closely associated with or attached to posterior and anterior circulation arteries. During one such case, a PCA (P1) perforating vessel was injured. Copious irrigation with the aid of a ventriculostomy catheter inserted into the naris revealed the site of arterial avulsion, and a successful repair/closure was achieved using hemostatic agent (Syvek marine polymer [off-label use]) with gentle compression. The patient did suffer a small thalamic infarction from this arterial injury with resultant aphasia, but fortunately has recovered well with minimal residual aphasia. The overall vascular injury rate for all surgeries8 was 5% (1 of 19). The rate of permanent neurological deficit is the same (same patient).

No patient died during or as a result of surgery or during the 30-day postoperative period. As mentioned earlier, 1 patient died following a stereotactic cyst treatment 3 years after EEA. One patient died of complications of anticoagulation therapy (acute subdural hematoma, distant from the site of surgery) 2 months following surgery. Another patient died of an unrelated medical condition ~ 2 years after surgery.

Discussion

Although small, this series demonstrates the potential of the purely endoscopic EEA as another aid in the treatment of suprasellar craniopharyngiomas. The addition and sole use of the endoscope in the expanded, microscopic approaches described by others17,20,25 allows improved visualization, which, in turn, allows the further expansion of the previously described approaches.

Craniopharyngiomas are notoriously difficult to cure, but complete resection has been shown to provide a survival benefit.1,3–5,8,16,27,33,34 Complete resection rates reported in the literature range from 6 to 0%,3,5,6,12,13,24,26,27,31,32,34–36 with the exception of the study by Yaşargil et al.,36 in which the authors reported a 90% gross-total resection rate (at the cost of a 16.7% mortality rate). Our small series of suprasellar tumors has a 73% gross-total resection rate for tumors intended for complete resection (50% for the overall series). The series' size limits direct comparisons; however, thus far, the rates of resection compared with historical case series do seem to be equivalent or better. Even more difficult to quantify is the patient's quality of life following tumor resection. Although the aim in most cases is complete resection and cure, there is a greater potential for some decrement in the patient's function, whether neurological, ophthalmological, or endocrinological, following radical surgery. In our study, all patients were functional postoperatively. Indeed, only 1 patient suffered a decrement in function (the patient with a PCA branch infarct). This resulted in a significant aphasia that recovered over the course of a year. This patient is living independently at home. In this series, the extent of resection was evaluated using an emphasis on an intent-to-treat analysis; only those tumors in which complete resection was the goal based on clinical factors were used in the analysis. Han et al.11 have previously reported on surgical cohorts using this type of intention-to-treat analysis.

No patient who underwent a gross-total resection experienced recurrence. The regrowth rate for the series is 25% (9% for those intended for complete resection). It is worth noting that all patients in our series who suffered recurrence or regrowth were patients with Type II (transinfundibular) tumors (Table 4), which grow and presumably originate from within the infundibulum. These tumors are perhaps the most difficult to cure surgically (3 of the 4 in this study had been previously resected). In all of these cases, an attempt was made to preserve the stalk, with resultant preservation of existing endocrine function.13 Although this was successful, in the future, it may be wiser to merely sacrifice the stalk in those cases in which there is no other remnant. Although certain to provide worse endocrine outcomes, in the long term, this practice may benefit the patient by reducing recurrence rates.28

As an initial evaluation of the endoscopic EEA technique, this series shows it to be effective in chiasmal decompression. Although published studies have reported rates of postoperative visual decline ranging from 2 to 15%,5,6,26,32,34,35 there were no such occurrences in this series. Furthermore, the likelihood of improvement in or complete recovery of vision was 92%.

In terms of endocrinopathy, panhypopituitarism and DI are particularly common following the resection of craniopharyngiomas; rates in the literature have ranged from 24 to 66% and 43 to 79%,6,31,34–36 respectively. In our series, 18% of patients developed panhypopituitarism and 8% developed permanent DI.

Perhaps most surprising is the high rate of panhypopituitarism following resection of Type I tumors. This may be due to the fact that both Type I tumors without preoperative dysfunction were resected early (first) in the series. Therefore, it is difficult to know if this finding is a result of tumor location or a reflection of our learning curve. In contrast, Type III tumors had a very low rate of dysfunction (1 [20%] of 5).

The aggressiveness of resection obviously has a significant effect on the aforementioned rates. This small series, however, appears to support the use of purely endoscopic techniques to provide a high rate of endocrine function preservation, while concurrently achieving respectable resections. This is especially true for Types I (preinfundibu-lar) and III (retroinfundibular) tumors, where the stalk can be separated and potentially preserved. In this series, this is illustrated by Type III tumors, where there was a very low rate of postoperative pituitary dysfunction. Although there are only a few patients in whom complete resection with preservation of current hormonal status can be achieved (2 in this series, both with Type III tumors), we believe that an attempt at preservation of pituitary gland and stalk is reasonable and at times successful. Given the tumor type and locations, some degree of postoperative pituitary dysfunction is expected and preservation of function must be weighed against the recurrence risk. Once again, this is emphasized by Type II tumors, in which the tumor has often invaded the pituitary stalk. This problem illustrates the difficulty in dealing with such a pathological entity.

Our experience with EEA resection of craniopharyngiomas has demonstrated the need for new techniques that prevent postoperative CSF leaks. Leakage rates were higher in this series than in our experience with other sellar and suprasellar lesions, and there are several reasons for this. One is the extensive, intentional violation of the anterior skull base dura that is necessary for lesion access. This larger opening is more difficult to effectively seal, and its proximity to critical neurovascular structures (carotid arteries, optic nerves, and so on) increases the challenge of obtaining a watertight repair.

Of particular interest has been our realization that despite similar size exposures for other anterior skull base and parasellar pathological entities such as pituitary adenomas and meningiomas, craniopharyngiomas have a disproportionately higher incidence of CSF leakage following surgery.7,18 This increased rate may be related to a higher incidence of either transient or permanent hydrocephalus associated with craniopharyngiomas.5,12,36 This is also reflected in the high rate of permanent CSF diversion demonstrated in this series. Given the early and often ineffective reconstruction techniques used in this series, any increased ICP, whether transient or permanent, was detrimental. However, the most important differentiating factor for increased CSF leakage is likely that resection of craniopharyngiomas, more than other parasellar lesions, involves the violation of arachnoid cisterns and offers entry into the third ventricle. This violation inevitably creates a greater obstacle to defect closure. Further modification of closure techniques will hopefully provide reduction in the CSF leakage rate, as has been the case after changes instituted later in this series. Through constant modification, the CSF leakage rates decreased considerably over the course of this study (Fig. 7). From 1998 to 2004, the leakage rate was 69%. The following year it was reduced to 20% (including the reoperations for tumor regrowth in this group, not listed in Table 5), once suturing and balloon buttresses were used. The recent addition of a vascularized, nasal septal flap9 to our reconstructions appears to hold tremendous promise.

Fig. 7.
Fig. 7.

Bar graph illustrating CSF leakage rates early in the series compared with the last year of the series. The time periods are misleading, as only 1 case of CSF leakage occurred prior to 2003, making this comparison largely one of 2003–2004 and 2005.

It is important to note that all CSF fistulas were eventually sealed without associated morbidity. This result was associated with a high permanent CSF diversion rate in this series. There were no cases of bacterial meningitis and no complications associated with reoperation or shunt placement, although the potential for serious complication clearly exists. With careful management and awareness of this issue, CSF leakage is not believed to be an insurmountable problem and does not negate the efficacy of this approach for craniopharyngioma.

As for vascular injury, perhaps the most feared complication of endoscopic manipulation of neurovascular structures, our rate of injury was low (5%). Just as important was the ability to successfully manage an arterial injury during a purely endoscopic endonasal approach.

Conclusions

The incorporation of the transplanum modification into traditional transsphenoidal approaches, facilitated by the use of the endoscope, has provided access to many tumors with suprasellar or even retrochiasmatic extension, previously thought to be unresectable via a transsphenoidal route. Craniopharyngioma is one such tumor. In our opinion, the use of the endoscope as the sole means of visualization in the EEA provides surgeons improved visualization of a region inherently fraught with potential disaster and helps to expand the indications for transsphenoidal resection of sellar region tumors in general and craniopha-ryngiomas in particular.

Comparison of our results with those previously described is best left to others. Hopefully, however, these data will help guide the future development of endoscopic techniques. This series illustrates the ability to access complex suprasellar, parasellar, and retrosellar craniopharyngiomas via an EEA, regardless of the degree of sellar expansion. Difficulties with preventing postoperative CSF leakage are highlighted by this series. Together with standard approaches and other technological advances such as ste-reotactic cyst therapies and radiosurgery, the endoscopic EEA may prove a valuable weapon in the armamentarium against this truly challenging tumor. Expansion of the series, both in terms of number of patients and period of follow-up is critical for complete and accurate evaluation of the approach and technique.

References

  • 1

    Chakrabarti IAmar APCouldwell WWeiss MH: Long-term neurological, visual, and endocrine outcomes following transnasal resection of craniopharyngioma. J Neurosurg 102:6506572005

  • 2

    Couldwell WTWeiss MHRabb CLiu JKApfelbaum RIFukushima T: Variations on the standard transsphenoidal approach to the sellar region, with emphasis on the extended approaches and parasellar approaches: surgical experience in 105 cases. Neurosurgery 55:5395472004

  • 3

    De Vile CJGrant DBKendall BENeville BGStanhope RWatkins KE: Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted?. J Neurosurg 85:73811996

  • 4

    Dhellemmes PVinchon M: Radical resection for craniopharyngiomas in children: surgical technique and clinical results. J Pediatr Endocrinol Metab 19:1 Suppl3293352006

  • 5

    Duff JMMeyer FBIlstrup DMLaws ERSchleck CDScheithauer BW: Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46:2913022000

  • 6

    Fahlbusch RHonegger JPaulus WHuk WBuchfelder M: Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90:2372501999

  • 7

    Frank GPasquini EDoglietto FMazzatenta DSciarretta VFarneti G: The endoscopic extended transsphenoidal approach for craniopharyngiomas. Neurosurgery 59:1 SupplONS75ONS832006

  • 8

    Gupta DKOjha BKSarkar CMahapatra AKSharma BSMehta VS: Recurrence in pediatric craniopharyngiomas: analysis of clinical and histological features. Childs Nerv Syst 22:50552006

  • 9

    Hadad GBassagasteguy LCarrau RLMataza JCKassam ASnyderman CH: A novel reconstructive technique following endoscopic expanded endonasal approaches: vascular pedicle nasoseptal flap. Laryngoscope 116:188118852006

  • 10

    Halves EBushe KA: Transsphenoidal operation on craniopharyngiomas with extrasellar extensions. The advantage of the operating endoscope. Acta Neurochir Suppl (Wien) 28:3621979

  • 11

    Han PPPonce FASpetzler RF: Intention-to-treat analysis of Spetzler-Martin grades IV and V arteriovenous malformations: natural history and treatment paradigm. J Neurosurg 98:372003

  • 12

    Hoffman HJDeSilva MHumphreys RPDrake JMSmith MLBlaser SI: Aggressive management of craniopharyngiomas in children. J Neurosurg 76:47521992

  • 13

    Honegger JBuchfelder MFahlbusch R: Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg 90:2512571999

  • 14

    Honegger JBuchfelder MFahlbusch RDaubler BDorr HG: Transsphenoidal microsurgery for craniopharyngioma. Surg Neurol 37:1891961992

  • 15

    Im SHWang KCKim SKChung YNKim HSLee CH: Transsphenoidal microsurgery for pediatric craniopharyngioma: special considerations regarding indications and method. Pediatr Neurosurg 39:971032003

  • 16

    Isaac MAHahn SSKim JABogart JAChung CT: Management of craniopharyngioma. Cancer J 7:5165202001

  • 17

    Kaptain GJVincent DASheehan JPLaws ER Jr: Transsphenoidal approaches for the extracapsular resection of midline suprasellar and anterior cranial base lesions. Neurosurgery 49:941002001

  • 18

    Kassam ABCarrau RLSnyderman CHGardner PMintz A: Evolution of reconstructive techniques following endoscopic expanded endonasal approaches. Neurosurg Focus 19:1E82005

  • 19

    Kassam ABGardner PASnyderman CHCarrau RLMintz AHPrevedello DM: Expanded endonasal approach, a fully endoscopic transnasal approach for the resection of midline suprasellar craniopharyngiomas: a new classification based on the infundibulum. J Neurosurg 108:7157282008

  • 20

    Kouri JGChen MYWatson JCOldfield EH: Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases. J Neurosurg 92:102810352000

  • 21

    Laws ER Jr: Comment on: Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:7241995

  • 22

    Laws ER Jr: Transsphenoidal microsurgery in the management of craniopharyngioma. J Neurosurg 52:6616661980

  • 23

    Laws ER Jr: Transsphenoidal removal of craniopharyngioma. Pediatr Neurosurg 21:57631994

  • 24

    Maira GAnile CRossi GFColosimo C: Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:7157241995

  • 25

    Mason RBNieman LKDoppman JLOldfield EH: Selective excision of adenomas originating in or extending into the pituitary stalk with preservation of function. J Neurosurg 87:3433511997

  • 26

    Matson DDCrigler JF Jr: Radical treatment of craniopharyngioma. Ann Surg 152:6997041960

  • 27

    McMurry FGHardy RWDohn DFSadar EGardner J: Long term results in the management of craniopharyngiomas. Neurosurgery 1:2382411977

  • 28

    Minamida YMikami THashi KHoukin K: Surgical management of the recurrence and regrowth of craniopharyngiomas. J Neurosurg 103:2242322005

  • 29

    Norris JSPavaresh MAfshar F: Primary transsphenoidal micro-surgery in the treatment of craniopharyngiomas. Br J Neurosurg 12:3053121998

  • 30

    Patterson RH: Comment on: Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:7241995

  • 31

    Shirane RSu CKusaka YJokura HYoshimoto T: Surgical outcomes in 31 patients with craniopharyngiomas extending outside the suprasellar cistern: an evaluation of the frontobasal approach. J Neurosurg 96:7047122002

  • 32

    Symon L: An approach to radical excision of craniopharyngioma by the temporal route. Pediatr Neurosurg 21:1 Suppl64681994

  • 33

    Tena-Suck MLSalinas-Lara CArce-Arellano RIRembao-Bo-jórquez DMorales-Espinosa DSotelo J: Clinico-pathological and immunohistochemical characteristics associated to recurrence/regrowth of craniopharyngiomas. Clin Neurol Neurosurg 108:6616692006

  • 34

    Van Effenterre RBoch AL: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3112002

  • 35

    Weiner HLWisoff JHRosenberg MEKupersmith MJCohen HZagzag D: Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35:100110111994

  • 36

    Yaşargil MGCircic MKis MSiegenthaler GTeddy PJRoth P: Total removal of craniopharyngiomas: approaches and long-term results in 144 patients. J Neurosurg 73:3111990

Sources of support: none reported.

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Article Information

Address correspondence to: Amin Kassam, M.D., Department of Neurological Surgery, Suite B-400, Presbyterian University Hospital, 200 Lothrop Street, Pittsburgh, Pennsylvania 15213. email: kassamab@upmc.edu.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Humphrey visual field tests. A: Preoperative study demonstrating a dense temporal defect. B: Postoperative visual field testing showing resolution of visual deficit.

  • View in gallery

    Humphrey visual field tests. A: Preoperative study showing dense bilateral defects, greatest bitemporally. B: Postoperative study performed in the same patient demonstrating significant improvement compared with preoperative testing.

  • View in gallery

    Preoperative (left) and postoperative (right) coronal MR images showing suprasellar, Type II (infundibular) craniopharyngioma treated using a stalk-sparing resection. The enhancement seen under the chiasm has not progressed during follow-up (see text on recurrence).

  • View in gallery

    Contrast-enhanced T1-weighted MR images. Preoperative sagittal (A) and coronal (B) images showing a complex craniopharyngioma with suprasellar extension. Postoperative sagittal (C) and coronal (D) images showing gross-total resection.

  • View in gallery

    Contrast-enhanced T1-weighted MR images. Preoperative sagittal (A) and coronal (B) images showing a giant, complex craniopharyngioma, with extension into the third and lateral ventricles in an elderly man with vision loss. Postoperative sagittal (C) and coronal (D) images showing debulking of this giant tumor. Debulking was chosen due to the patient's advanced age and concern over potential hypothalamic and thalamic injury with aggressive resection.

  • View in gallery

    Coronal contrast-enhanced T1-weighted MR images. Left: Preoperative image showing a suprasellar craniopharyngioma with a separate nodule (arrow) in the lateral wall of the third ventricle. Resection of the nodule was not planned as the floor of the third ventricle appeared intact on preoperative imaging. In retrospect and with further experience, perhaps this nodule would have been accessible. Right: Postoperative image obtained in the same patient showing resection of the suprasellar tumor, with the third ventricular nodular residual lesion (arrow).

  • View in gallery

    Bar graph illustrating CSF leakage rates early in the series compared with the last year of the series. The time periods are misleading, as only 1 case of CSF leakage occurred prior to 2003, making this comparison largely one of 2003–2004 and 2005.

References

1

Chakrabarti IAmar APCouldwell WWeiss MH: Long-term neurological, visual, and endocrine outcomes following transnasal resection of craniopharyngioma. J Neurosurg 102:6506572005

2

Couldwell WTWeiss MHRabb CLiu JKApfelbaum RIFukushima T: Variations on the standard transsphenoidal approach to the sellar region, with emphasis on the extended approaches and parasellar approaches: surgical experience in 105 cases. Neurosurgery 55:5395472004

3

De Vile CJGrant DBKendall BENeville BGStanhope RWatkins KE: Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted?. J Neurosurg 85:73811996

4

Dhellemmes PVinchon M: Radical resection for craniopharyngiomas in children: surgical technique and clinical results. J Pediatr Endocrinol Metab 19:1 Suppl3293352006

5

Duff JMMeyer FBIlstrup DMLaws ERSchleck CDScheithauer BW: Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46:2913022000

6

Fahlbusch RHonegger JPaulus WHuk WBuchfelder M: Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90:2372501999

7

Frank GPasquini EDoglietto FMazzatenta DSciarretta VFarneti G: The endoscopic extended transsphenoidal approach for craniopharyngiomas. Neurosurgery 59:1 SupplONS75ONS832006

8

Gupta DKOjha BKSarkar CMahapatra AKSharma BSMehta VS: Recurrence in pediatric craniopharyngiomas: analysis of clinical and histological features. Childs Nerv Syst 22:50552006

9

Hadad GBassagasteguy LCarrau RLMataza JCKassam ASnyderman CH: A novel reconstructive technique following endoscopic expanded endonasal approaches: vascular pedicle nasoseptal flap. Laryngoscope 116:188118852006

10

Halves EBushe KA: Transsphenoidal operation on craniopharyngiomas with extrasellar extensions. The advantage of the operating endoscope. Acta Neurochir Suppl (Wien) 28:3621979

11

Han PPPonce FASpetzler RF: Intention-to-treat analysis of Spetzler-Martin grades IV and V arteriovenous malformations: natural history and treatment paradigm. J Neurosurg 98:372003

12

Hoffman HJDeSilva MHumphreys RPDrake JMSmith MLBlaser SI: Aggressive management of craniopharyngiomas in children. J Neurosurg 76:47521992

13

Honegger JBuchfelder MFahlbusch R: Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg 90:2512571999

14

Honegger JBuchfelder MFahlbusch RDaubler BDorr HG: Transsphenoidal microsurgery for craniopharyngioma. Surg Neurol 37:1891961992

15

Im SHWang KCKim SKChung YNKim HSLee CH: Transsphenoidal microsurgery for pediatric craniopharyngioma: special considerations regarding indications and method. Pediatr Neurosurg 39:971032003

16

Isaac MAHahn SSKim JABogart JAChung CT: Management of craniopharyngioma. Cancer J 7:5165202001

17

Kaptain GJVincent DASheehan JPLaws ER Jr: Transsphenoidal approaches for the extracapsular resection of midline suprasellar and anterior cranial base lesions. Neurosurgery 49:941002001

18

Kassam ABCarrau RLSnyderman CHGardner PMintz A: Evolution of reconstructive techniques following endoscopic expanded endonasal approaches. Neurosurg Focus 19:1E82005

19

Kassam ABGardner PASnyderman CHCarrau RLMintz AHPrevedello DM: Expanded endonasal approach, a fully endoscopic transnasal approach for the resection of midline suprasellar craniopharyngiomas: a new classification based on the infundibulum. J Neurosurg 108:7157282008

20

Kouri JGChen MYWatson JCOldfield EH: Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases. J Neurosurg 92:102810352000

21

Laws ER Jr: Comment on: Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:7241995

22

Laws ER Jr: Transsphenoidal microsurgery in the management of craniopharyngioma. J Neurosurg 52:6616661980

23

Laws ER Jr: Transsphenoidal removal of craniopharyngioma. Pediatr Neurosurg 21:57631994

24

Maira GAnile CRossi GFColosimo C: Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:7157241995

25

Mason RBNieman LKDoppman JLOldfield EH: Selective excision of adenomas originating in or extending into the pituitary stalk with preservation of function. J Neurosurg 87:3433511997

26

Matson DDCrigler JF Jr: Radical treatment of craniopharyngioma. Ann Surg 152:6997041960

27

McMurry FGHardy RWDohn DFSadar EGardner J: Long term results in the management of craniopharyngiomas. Neurosurgery 1:2382411977

28

Minamida YMikami THashi KHoukin K: Surgical management of the recurrence and regrowth of craniopharyngiomas. J Neurosurg 103:2242322005

29

Norris JSPavaresh MAfshar F: Primary transsphenoidal micro-surgery in the treatment of craniopharyngiomas. Br J Neurosurg 12:3053121998

30

Patterson RH: Comment on: Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:7241995

31

Shirane RSu CKusaka YJokura HYoshimoto T: Surgical outcomes in 31 patients with craniopharyngiomas extending outside the suprasellar cistern: an evaluation of the frontobasal approach. J Neurosurg 96:7047122002

32

Symon L: An approach to radical excision of craniopharyngioma by the temporal route. Pediatr Neurosurg 21:1 Suppl64681994

33

Tena-Suck MLSalinas-Lara CArce-Arellano RIRembao-Bo-jórquez DMorales-Espinosa DSotelo J: Clinico-pathological and immunohistochemical characteristics associated to recurrence/regrowth of craniopharyngiomas. Clin Neurol Neurosurg 108:6616692006

34

Van Effenterre RBoch AL: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3112002

35

Weiner HLWisoff JHRosenberg MEKupersmith MJCohen HZagzag D: Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35:100110111994

36

Yaşargil MGCircic MKis MSiegenthaler GTeddy PJRoth P: Total removal of craniopharyngiomas: approaches and long-term results in 144 patients. J Neurosurg 73:3111990

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