Craniopharyngioma endoscopic endonasal approach case series

Madsen et al., from the Hospital of the University of Pennsylvania, performed a retrospective review of pediatric patients undergoing resection of craniopharyngioma between 2001 and 2017. Volumetric analysis of tumor size and postoperative ischemic injury was performed. Charts were reviewed for a number of outcome measures.

A total of 43 patients with an average age of 8.2 years were identified. Open surgery was the initial intervention in 15 and EES in 28. EES was performed in patients 3-17 years of age. EES has been the only approach used since 2011. In the entire cohort, GTR was more common in the EES group (85.7% vs 53.3%, p = 0.03). Recurrence rate (40% vs 14.2%, p = 0.13) and need for adjuvant radiation (20.0% vs 10.7%, p = 0.71) were higher in the open surgical group, although not statistically significant. Pseudoaneurysm development was only observed in the open surgical group. Volumetric imaging analysis showed a trend toward larger preoperative tumor volumes in the open surgical group, so a matched cohort analysis was performed with the largest tumors from the EES group. This revealed no difference in residual tumor volume (p = 0.28), but the volume of postoperative ischemia was still significantly larger in the open group (p = 0.004). Postoperative weight gain was more common in the open surgical group, a statistically significant finding in the complete patient group that trended toward significance in the matched cohort groups. Body mass index at follow-up correlated with volume of ischemic injury in regression analysis of the complete patient cohort (p = 0.05).

EES was associated with similar, if not better, extent of resection and significantly less ischemic injury than open surgery. Pseudoaneurysms were only seen in the open surgical group. Weight gain was also less prevalent in the EES cohort and appears be correlated with extent of ischemic injury at time of surgery 1).


A retrospective cohort analysis of 43 consecutive EEA procedures in 40 patients operated from September 2006 to February 2012 for suprasellar craniopharyngiomas. In 21 patients (48.8%) the disease was recurrent. We have assessed the surgical results, visual, endocrinological, and functional outcomes and resection rates in this patient cohort.

At presentation, 31 (72.1%) patients had visual deficits, 15 patients (34.9%) complained of headaches, 25 patients (58.1%) had anterior pituitary insufficiency, and 14 (32.5%) had diabetes insipidus. Total resection was achieved in 44.2% surgeries, of which 77.3% were in primary lesions and 9.5% in recurrent lesions (P < .001). Vision improved in 92.6% patients and worsened in 2.3%. Complications other than vision were encountered in 25.6% including 9/43 Cerebrospinal fluid fistula, 2/43 meningitis. A total of 51.9% of patients with preoperative residual anterior pituitary function had new anterior pituitary deficiencies and 42.8% had new diabetes insipidus. There was no mortality. Six patients (14%) had recurrence of disease during the follow-up period (mean 56.8 mo), 5 of which required repeat surgery.

The EEA can be integrated in the overall management of both primary and recurrent craniopharyngiomas with good results; however, in this series recurrent surgery was associated with significantly lower rates of gross total resection 2).

From July 2010 to March 2016, 82 adult craniopharyngioma patients underwent an EEA at the authors' institution. Of these cases, intraoperative records and immediate postoperative MR images were available for 68 patients. The patients underwent systemized endocrinological evaluation. Eighteen of 68 patients who underwent EEA for recurrence or regrowth of residual lesions after previous surgical management were excluded in the analysis of the anatomical tumor classification. The authors retrospectively analyzed preoperative clinical features and previous anatomical classifications, focusing on the relationship of the pituitary stalk and tumor, to determine predictive factors for the clinical outcome, such as the extent of resection, visual function, endocrinological function, recurrence rate, and complications. RESULTS The mean tumor size was 2.5 cm (3.1 cm for primary tumors and 1.9 cm for recurrent lesions). Gross-total resection (GTR) was achieved in 62 (91.1%) patients (48 [96.0%] patients with primary tumors and 14 [77.8%] patients with recurrent tumors). The rate of GTR was higher in the primary group than in the group with recurrence (p = 0.038). The overall pre- and postoperative visual impairment scale (VIS) scores were 40.8 and 22.1, respectively (50.9 and 14.3 in the primary group and 30.7 and 29.9 in patients with recurrence, respectively). The improvement rate in VIS score was higher in the primary group than in the recurrent group (p = 0.001). Endocrinological function was improved in 4 patients (5.9%) and deteriorated in 32 of 68 patients (47.1%). Tumor invasion into the center of the pituitary stalk affected the postoperative outcomes most significantly. Cognitive dysfunction was observed in 22 patients before surgery and improved in 20 patients (90.9%) after surgery. Hydrocephalus was found in 7 patients and resolved after surgery in all cases. Cerebrospinal fluid fistula occurred in 2 (2.9%) of 68 patients and was repaired by revision surgery in both patients. Ten patients without Cerebrospinal fluid fistula also received antibiotics for the treatment of meningitis. The infection rate was higher in the recurrent group. Postoperative endocrinological evaluation showed no deficits in 12 patients and panhypopituitarism in 55 patients. The remaining patient had growth hormone deficiency. Forty-three patients had new-onset diabetes insipidus, and 1 patient had persistent diabetes insipidus after surgery. There were 2 (2.9%) cases of recurrence during the mean 30.7-month follow-up period; one patient underwent radiosurgery and the other underwent reoperation. CONCLUSIONS The EEA resulted in excellent surgical outcomes and acceptable morbidity rates, regardless of the anatomical location of the tumor. Invasion of the craniopharyngioma into the center of the pituitary stalk has strong predictive power for postoperative endocrinological outcome 3).

33 procedures performed in 31 patients. The average postoperative Anterior Skull Base Quality of Life questionnaire (ASBQ) was 3.35 and the 22 Item Sinonasal Outcome Test score (SNOT-22) was 19.6. Better quality of life (QOL) was associated with gross total resection (GTR) and postoperative radiation. Worse QOL was associated with persistent visual defects, hypopituitarism, tumor recurrence, increase in body mass index, and worsening Wen score. In a subset of 10 patients, both pre- and postoperative (> 9 months) QOL scores were obtained. Both ASBQ and SNOT-22 scores showed stability and a trend toward improvement, from 2.93 ± 0.51 to 2.96 ± 0.47 (ASBQ) and 23.7 ± 10.8 to 18.4 ± 11.6 (SNOT-22). Compared with 62 patients undergoing endoscopic pituitary macroadenoma resection, patients with craniopharyngiomas had worse postoperative QOL on the ASBQ (3.35 vs 3.80; p = 0.023) and SNOT-22 (19.6 vs 13.4; p = 0.12).

This report of validated site-specific QOL following endoscopic surgery for craniopharyngiomas shows an overall maintenance of postoperative compared with preoperative QOL. Better QOL could be seen in patients with GTR and radiation therapy, and worse QOL was found in patients with visual or endocrine deficits. Nevertheless, patients with craniopharyngiomas still had worse QOL than those undergoing similar surgery for pituitary macroadenomas, confirming the worse prognosis of craniopharyngiomas even when removed via a minimally invasive approach. These measures should serve as benchmarks for comparison with open transcranial approaches to similar tumors 4).


A retrospective study of 44 craniopharyngiomas. The goal of surgery was gross-total resection in all cases. All patients underwent pre- and post-operative comprehensive ophthalmological and endocrinological evaluation. lumbar drainage at the start of the operation was used in all cases with tumor larger than 3 cm maximum diameter. Binostril technique vascularized nasoseptal flap and multilayer closure of the dural defect were used. Wide sphenoidotomy, posterior ethmoidectomy, tuberculum selle, and planum removal were performed in all cases. Perioperative antibiotic prophylaxis was used for 72 h.

There were 44 patients of age ranging from 8 to 65 (mean: 42) years. Diameter of the tumor varied from 3.1 cm to 6.6 cm (average: 4.3 cm). Visual and pituitary dysfunctions were observed in 44 and 33, respectively, before surgery. Vision improvement, gross-total removal, cerebrospinal fluid (CSF) leak and recurrence were observed in 34, 26, four and six patients, respectively. Average follow-up was 19 months.

Endoscopic endonasal transsphenoidal approach for craniopharyngioma is safe and effective alternative to transcranial approach in selected patients. Although this technique is associated with effective tumor removal and improved visual outcome, Cerebrospinal fluid fistula, and endocrine dysfunctions remain a major challenge 5)

4 examples of endoscopic transsphenoidal resection of a craniopharyngioma. Figures and captions for relevant anatomy during tumor resection are depicted at the end of each video.

Case 1 (0:06): The patient is a 54-year-old male that was found to have a 2.6 x 2.0 x 3.6 cm cystic sellar mass with suprasellar extension upon workup of headaches, fatigue, gynecomastia, and decreased libido. His laboratory studies demonstrated central hypogonadism and central hypothyroidism.

Case 2 (2:28): The patient is a 29-year-old male that was found to have a 3.6 x 2.7 x 2.5 cm sellar mass with suprasellar extension upon workup of headaches, decreased libido, and visual field deficits. The mass has both a cystic and solid component. His pre-operative endocrine laboratory studies demonstrated adrenal insufficiency, hypogonadism, and hypothyroidism.

Case 3 (4:39): The patient is a 61-year-old female that was found to have a 1.7 x 1.4 x 1.1 cm sellar mass with suprasellar extension upon workup of headaches, fatigue, vertigo, and blurry vision. The mass has both a cystic and solid component. Her pre-operative endocrine laboratory studies were unremarkable.

Case 4 (5:58): The patient is a 32-year-old female that was found to have a 1.9 x 1.3 x 2.8 cm solid sellar mass with extension into the 3rd ventricle upon workup of headaches, horizontal diplopia, and bilateral abducens nerve palsies. Her pre-operative endocrine laboratory studies were unremarkable 6).


1)
Madsen PJ, Buch VP, Douglas JE, Parasher AK, Lerner DK, Alexander E, Workman AD, Palmer JN, Lang SS, Kennedy BC, Vossough A, Adappa ND, Storm PB. Endoscopic endonasal resection versus open surgery for pediatric craniopharyngioma: comparison of outcomes and complications. J Neurosurg Pediatr. 2019 Jun 7:1-10. doi: 10.3171/2019.4.PEDS18612. [Epub ahead of print] PubMed PMID: 31174192.
2)
Radovanovic I, Dehdashti AR, Turel MK, Almeida JP, Godoy BL, Doglietto F, Vescan AD, Zadeh G, Gentili F. Expanded Endonasal Endoscopic Surgery in Suprasellar Craniopharyngiomas: A Retrospective Analysis of 43 Surgeries Including Recurrent Cases. Oper Neurosurg (Hagerstown). 2019 Feb 15. pii: opy356. doi: 10.1093/ons/opy356. [Epub ahead of print] PubMed PMID: 30768130.
3)
Dho YS, Kim YH, Se YB, Han DH, Kim JH, Park CK, Wang KC, Kim DG. Endoscopic endonasal approach for craniopharyngioma: the importance of the relationship between pituitary stalk and tumor. J Neurosurg. 2018 Sep;129(3):611-619. doi: 10.3171/2017.4.JNS162143. Epub 2017 Sep 29. PMID: 28960155.
4)
Patel KS, Raza SM, McCoul ED, Patrona A, Greenfield JP, Souweidane MM, Anand VK, Schwartz TH. Long-term quality of life after endonasal endoscopic resection of adult craniopharyngiomas. J Neurosurg. 2015 Sep;123(3):571-80. doi: 10.3171/2014.12.JNS141591. Epub 2015 Apr 17. PubMed PMID: 25884258.
5)
Yadav YR, Nishtha Y, Vijay P, Shailendra R, Yatin K. Endoscopic endonasal trans-sphenoid management of craniopharyngiomas. Asian J Neurosurg. 2015 Jan-Mar;10(1):10-6. doi: 10.4103/1793-5482.151502. PubMed PMID: 25767569; PubMed Central PMCID: PMC4352621.
6)
Chu J, Oyesiku N. 3-D Endoscopic Transsphnoidal Pituitary Surgery: 4 Unique Cases of Craniopharyngioma. Neurosurgery. 2014 Aug 27. [Epub ahead of print] PubMed PMID: 25167380.
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