Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients

Clinical article

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Object

The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging and controversial management dilemmas in neurosurgery. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for both pediatric and adult craniopharyngiomas. The object of the present study was to present the results of EES and analyze outcome in both the pediatric and the adult age groups.

Methods

The authors retrospectively reviewed the records of patients with craniopharyngioma who had undergone EES in the period from June 1999 to April 2011.

Results

Sixty-four patients, 47 adults and 17 children, were eligible for this study. Forty-seven patients had presented with primary craniopharyngiomas and 17 with recurrent tumors. The mean age in the adult group was 51 years (range 28–82 years); in the pediatric group, 9 years (range 4–18 years). Overall, the gross-total resection rate was 37.5% (24 patients); near-total resection (> 95% of tumor removed) was 34.4% (22 patients); subtotal resection (≥ 80% of tumor removed) 21.9% (14 patients); and partial resection (< 80% of tumor removed) 6.2% (4 patients). In 9 patients, EES had been combined with radiation therapy (with radiosurgery in 6 cases) as the initial treatment. Among the 40 patients (62.5%) who had presented with pituitary insufficiency, pituitary function remained unchanged in 19 (47.5%), improved or normalized in 8 (20%), and worsened in 13 (32.5%). In the 24 patients who had presented with normal pituitary function, new pituitary deficit occurred in 14 (58.3%). Nineteen patients (29.7%) suffered from diabetes insipidus at presentation, and the condition developed in 21 patients (46.7%) after treatment. Forty-four patients (68.8%) had presented with impaired vision. In 38 (86.4%) of them, vision improved or even normalized after surgery; in 5, it remained unchanged; and in 1, it temporarily worsened. One patient without preoperative visual problems showed temporary visual deterioration after treatment. Permanent visual deterioration occurred in no one after surgery. The mean follow-up was 38 months (range 1–135 months). Tumor recurrence after EES was discovered in 22 patients (34.4%) and was treated with repeat surgery (6 patients), radiosurgery (1 patient), combined repeat surgery and radiation therapy (8 patients), interferon (1 patient), or observation (6 patients). Surgical complications included 15 cases (23.4%) with CSF leakage that was treated with surgical reexploration (13 patients) and/or lumbar drain placement (9 patients). This leak rate was decreased to 10.6% in recent years after the introduction of the vascularized nasoseptal flap. Five cases (7.8%) of meningitis were found and treated with antibiotics without further complications. Postoperative hydrocephalus occurred in 7 patients (12.7%) and was treated with ventriculoperitoneal shunt placement. Five patients experienced transient cranial nerve palsies. There was no operative mortality.

Conclusions

With the goal of gross-total or maximum possible safe resection, EES can be used for the treatment of every craniopharyngioma, regardless of its location, size, and extension (excluding purely intraventricular tumors), and can provide acceptable results comparable to those for traditional craniotomies. Endoscopic endonasal surgery is not limited to adults and actually shows higher resection rates in the pediatric population.

Abbreviations used in this paper:BMI = body mass index; CN = cranial nerve; DI = diabetes insipidus; EES = endoscopic endonasal surgery; GH = growth hormone; GTR = gross-total resection; HPA = hypothalamic-pituitary-adrenal; HPG = hypothalamic-pituitarygonadal; HPT = hypothalamic-pituitary-thyroid; TSS = transsphenoidal surgery; SIADH = syndrome of inappropriate antidiuretic hormone secretion.

Article Information

Address correspondence to: Paul A. Gardner, M.D., Department of Neurosurgery, UPMC Presbyterian, 200 Lothrop St., Ste. B400, Pittsburgh, PA 15213. email: gardpa@upmc.edu.

Please include this information when citing this paper: published online August 2, 2013; DOI: 10.3171/2013.6.JNS122259.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Left: Preoperative sagittal postcontrast T1-weighted MR image showing a craniopharyngioma with solid (lower) and cystic (upper) compartments. The cystic compartment extends up to the floor of the third ventricle, giving the impression of invading the ventricle (arrow). Right: Sagittal postcontrast T1-weighted MR image obtained after GTR. During surgery, the tumor capsule was dissected from the floor of the third ventricle, which was intact and not penetrated (arrow). The pituitary gland and stalk were also preserved, as demonstrated on MRI. The contrast-enhancing linear structure at the posterior aspect of the sphenoid sinus represents the vascularized nasoseptal flap that was used for reconstruction (arrowheads).

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    Coronal and sagittal postcontrast T1-weighted MR images obtained in an adult with a suprasellar craniopharyngioma. Upper: Preoperative images demonstrating a solid suprasellar craniopharyngioma with extension to the interpeduncular cistern and displacement of the optic chiasm. The pituitary stalk cannot be identified in these images. Lower: Postoperative images obtained after GTR via EES. The optic chiasm was totally decompressed. The pituitary stalk was preserved, as shown in both images. The pituitary gland was intact. Note the vascularized nasoseptal flap at the posterior aspect of the sphenoid sinus on the sagittal image.

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    Coronal and sagittal postcontrast T1-weighted MR images obtained in a pediatric patient with invasive craniopharyngioma. A: Preoperative images showing a sellar/suprasellar tumor that erodes the anterior skull base and extends into the sphenoid sinus, invades the left cavernous sinus (arrow), erodes the clivus and extends into the prepontine cistern deforming the brainstem. B: Postoperative images demonstrating extensive, safe resection of the tumor. The pituitary stalk was identified during surgery and preserved, as seen in both images. The preexisting hypopituitarism improved postoperatively. The intracavernous tumor was deemed not safely resectable (arrow). Extensive erosion of the skull base was repaired with a vascularized nasoseptal flap (arrowheads). Residual tumor showed regrowth, and the patient underwent reoperation (EES) and Gamma Knife surgery 2.5 years after the initial EES. C: Most recent images obtained after reoperation and radiosurgery, showing resolution of the left intracavernous tumor (arrow). However, an area suspicious for new recurrence is visible on the right cavernous sinus. Four years after the initial diagnosis, the patient was under close observation.

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    Coronal and sagittal postcontrast T1-weighted MR images of a pediatric suprasellar craniopharyngioma. Upper: Preoperative images demonstrating a suprasellar multicystic craniopharyngioma with invasion of the third ventricle and interpeduncular cistern, extension back to the level of the pineal region, and coexisting hydrocephalus. Lower: Images obtained after EES. Hydrocephalus was resolved with the placement of an external ventricular drain before surgery and did not necessitate a permanent shunt. No remnants of the tumor appear in the third ventricle. However, the tumor adhered to the hypothalamus, and thus further dissection was not attempted. Although not shown in these images, a minimal residual tumor capsule remained attached to the hypothalamus, and the case was considered a near-total resection.

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    Images of a suprasellar craniopharyngioma with intraventricular extension treated with GTR via EES. A and B: Preoperative coronal and sagittal postcontrast T1-weighted MR images demonstrating a mixed suprasellar tumor with a lower solid and an upper cystic component, invading the floor of the third ventricle. C: Intraoperative view with a 0° endoscope after bony resection of the planum, tuberculum, and anterior wall of the sella. The pituitary gland (pg) is covered by the sellar dura. The suprasellar dura has been incised in a “V” fashion, and the underlying arachnoid has been exposed. D: After opening the suprasellar arachnoid, the optic chiasm (oc) is identified and the solid compartment of the tumor (tu) is visualized between the optic chiasm and the pituitary gland (pg). The arachnoid above the optic chiasm covers the A2 branches of the 2 anterior cerebral arteries and is left intact. E: The tumor (tu) is dissected and removed from the suprasellar cistern. The optic nerves (on) are lying under the dura, which has been left intact to protect the nerves. F: A closer view with the 0° endoscope provides direct visualization of the third ventricle (3rd v), whose floor was eroded by the tumor. Once the tumor is removed, the pituitary stalk (ps) can be identified with deviation to the left. The optic chiasm (oc) and pituitary gland (pg) remain intact. G and H: Postoperative coronal and sagittal T1-weighted MR images obtained after contrast administration, confirming GTR of the craniopharyngioma. The pituitary stalk does not appear in the midline sagittal image (H) because of its deviation, but it does appear deviated to the left (G, upper arrow) on the coronal image, just above the pituitary gland (G, lower arrow). The linear contrast-enhancing area on the sagittal MR image (H, arrow) represents the vascularized nasoseptal flap that was used for skull base reconstruction, and the enhancing spot behind the flap is the intact pituitary gland.

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