Suprasellar pediatric craniopharyngioma resection via endonasal endoscopic approach

Childs Nerv Syst DOI 10.1007/s00381-013-2153-1

ORIGINAL PAPER

Suprasellar pediatric craniopharyngioma resection via endonasal endoscopic approach Zarina S. Ali & Shih-Shan Lang & Ameet R. Kamat & Nithin D. Adappa & James N. Palmer & Phillip B. Storm & John Y. K. Lee

Received: 2 April 2013 / Accepted: 7 May 2013 # Springer-Verlag Berlin Heidelberg 2013

Abstract Purpose Purely endoscopic endonasal approaches to surgical resection of pediatric suprasellar craniopharyngiomas are uncommonly performed. The aim of the study is to assess the feasibility and to describe the short-term outcomes of endonasal endoscopic approaches for the gross total resection of suprasellar craniopharyngiomas in the pediatric population. Methods A combined neurosurgical–otolaryngologic team performed gross total resection of craniopharyngiomas in seven pediatric patients (mean age 9.6 years) at The Children’s Hospital of Philadelphia over 2011–2012. Short-term outcomes were analyzed over a mean followup period of 6.3 months. Results All tumors involved the sellar and/or suprasellar space and contained some cystic component. The mean maximal tumor diameter was 31.5 mm (range 18.5– 62.0 mm). Using a binostril approach, gross total tumor resection was obtained in all patients (100 %). All patients with preoperative visual dysfunction demonstrated Electronic supplementary material The online version of this article (doi:10.1007/s00381-013-2153-1) contains supplementary material, which is available to authorized users. Z. S. Ali (*) : S. solid Solid > cystic Cystic Solid > cystic Cystic > solid Cystic > solid Cystic > solid

Childs Nerv Syst

Fig. 1 Ten-year-old male who presented with polyuria, visual changes, and fatigue (patient 4, Table 1). Sagittal T1 with contrast (a) and sagittal T2 (b) MRI demonstrate a 62×38×31 mm contrast enhancing sellar and suprasellar lesion extending superiorly into the third ventricle consistent with a craniopharyngioma. Sagittal T1 with contrast MRI (c) postoperatively shows gross total resection of the lesion

of the endonasal, endoscopic approach for sellar and suprasellar craniopharyngioma resection in the pediatric population. In particular, we support this approach for resection of tumors that are primarily cystic, but we also demonstrate that mixed solid and cystic tumors are candidates for tumor

resection using this strategy. Compared to previously reported case series, our patient population represents a more homogeneous group of pediatric patients with craniopharyngiomas encompassing the suprasellar, with or without sellar involvement and/or extension into the third ventricle, that were all treated by the same surgical procedure. Our short-term outcomes suggest that gross total tumor resection was achieved in all patients. Our surgical results are similar to or supersede outcomes achieved with traditional transcranial approaches or microscopic transsphenoidal approaches in the pediatric population [1, 12, 19, 20, 24]. Importantly, 100 % of our patients achieved improvement in visual dysfunction in the short-term period. This supports the notion put forth by Laws et al., who noted in 1977 that transsphenoidal surgery affords the quickest, most effective, and least dangerous mode of therapy for lesions producing visual impairment by compression of the optic nerves and chiasm [25]. Pituitary dysfunction and DI, on the other hand, represent some of the most prevalent deficits in patients with craniopharyngioma, both pre- and postoperatively, and contributes to increased hospital length of stay in the short-term. However, many believe that the endocrinologic deficits that develop after definitive craniopharyngioma treatment should be accepted, and, in the modern era of sophisticated medical treatment for endocrinopathies, not compromise definitive tumor control by gross total resection [9, 13–15]. CSF leak complicated the postoperative course of one of our seven patients (14 %) undergoing an expanded endonasal transtuberculum and transplanum approach. Indeed, the major barrier to successful transsphenoidal approaches is CSF leak with resultant meningitis. While our patient did not develop signs of meningitis, he required further therapy, including lumbar drainage, and associated sequelae of this therapy. We support the use of multilayered closure techniques, including routine fat and fascia lata graft as well as a vascularized nasoseptal flap [8, 16, 18, 23, 27]. Advancements in sellar closure techniques and surgical experience with these multilayered closures will undoubtedly decrease the rate of CSF leaks in the future. As described previously, the use of the binostril “twonostrils-four-hands” endoscopic approach affords significant advantages to pediatric craniopharyngioma resection [3, 7, 29]. Given the narrow working corridor of the nasal cavities in these patients, this binostril route allows for increased maneuverability of the endoscope together with surgical instruments. This, in turn, allows for improved visualization of the surgical field, making gross total resection feasible via this technique. Furthermore, these approaches allow for identification of small vessels at risk for injury and result in reduced intraoperative bleeding, which is a major concern in pediatric surgery [29]. This study is inherently limited by its retrospective design, small series of patients, and short-term follow-up.

Childs Nerv Syst

Fig. 2 Eleven-year-old male who presented with headache and diplopia from a bilateral cranial nerve 6 palsy (patient 5, Table 1). Sagittal T2 (a) and coronal T2 (b) MRI demonstrate a 21× 35×32 mm cystic suprasellar lesion extending superiorly into

the 3rd ventricle consistent with a craniopharyngioma. Intraoperative high-definition images (c) show the enlargement of the third ventricle and displacement of suprasellar structures by the lesion. Gross total resection was achieved (d)

Direct comparison of surgical approaches is beyond the scope of this study. Further studies are needed to characterize the long-term surgical and clinical outcome of pediatric patients treated with this surgical approach. However, the study achieves its stated goal in characterizing the feasibility of the endoscopic, endonasal approach for the treatment of suprasellar pediatric craniopharyngiomas. In conclusion, we recommend consideration of the endoscopic endonasal resection via a transtuberculum approach for the definitive treatment of craniopharyngiomas in

the pediatric population. In particular, we find that this approach can be performed safely using the “twonostrils-four-hands” technique with intraoperative neuronavigation. This approach should be strongly considered in patients with visual dysfunction, as it affords relatively immediate improvement of symptoms. Endocrinopathy remains a postoperative sequelae of this approach and CSF leak poses postoperative morbidity, but may be improved with future advances in skull base reconstruction techniques.

Table 3 Postoperative outcomes

Case number

Visual deficit

Anterior pituitary dysfunction

DI

CSF leak

1 2 3 4

None None None Improving

New panhypopituitarism New panhypopituitarism Preop partial → new panhypopituitarism Preop partial → new panhypopituitarism

Stable New New New

No No No No

5

Improving, delayed right Horner’s syndrome Improving Improving

New panhypopituitarism

New

Yes

Stable panhypopituitarism Stable panhypopituitarism

Stable New

No No

6 7

Childs Nerv Syst Conflict of interest The authors have no conflicts of interest to disclose.

References 1. Abe T, Ludecke DK (1999) Transnasal surgery for infradiaphragmatic craniopharyngiomas in pediatric patients. Neurosurgery 44:957–964, discussion 964–956 2. Adappa ND, Learned KO, Palmer JN, Newman JG, Lee JY (2012) Radiographic enhancement of the nasoseptal flap does not predict postoperative cerebrospinal fluid leaks in endoscopic skull base reconstruction. Laryngoscope 122:1226–1234 3. Briner HR, Simmen D, Jones N (2005) Endoscopic sinus surgery: advantages of the bimanual technique. Am J Rhinol 19:269–273 4. Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM (1998) The descriptive epidemiology of craniopharyngioma. J Neurosurg 89:547–551 5. Cappabianca P, Cavallo LM (2012) The evolving role of the transsphenoidal route in the management of craniopharyngiomas. World neurosurg 77:273–274 6. Carmel PW, Antunes JL, Chang CH (1982) Craniopharyngiomas in children. Neurosurgery 11:382–389 7. Castelnuovo P, Pistochini A, Locatelli D (2006) Different surgical approaches to the sellar region: focusing on the "two nostrils four hands technique". Rhinology 44:2–7 8. Cavallo LM, de Divitiis O, Aydin S, Messina A, Esposito F, Iaconetta G, Talat K, Cappabianca P, Tschabitscher M (2007) Extended endoscopic endonasal transsphenoidal approach to the suprasellar area: anatomic considerations–part 1. Neurosurgery 61:24–33, discussion 33–24 9. Chakrabarti I, Amar AP, Couldwell W, Weiss MH (2005) Long-term neurological, visual, and endocrine outcomes following transnasal resection of craniopharyngioma. J Neurosurg 102:650–657 10. Chan DG, Francis IC (2004) Transsphenoidal diplopia. Surv Ophthalmol 49:618–619, author reply 619 11. de Divitiis E, Cappabianca P, Gangemi M, Cavallo LM (2000) The role of the endoscopic transsphenoidal approach in pediatric neurosurgery. Child's Nerv Syst 16:692–696 12. Di Rocco C, Caldarelli M, Tamburrini G, Massimi L (2006) Surgical management of craniopharyngiomas—experience with a pediatric series. J Pediatr Endocrinol Metab 19(Suppl 1):355–366 13. Duff J, Meyer FB, Ilstrup DM, Laws ER Jr, Schleck CD, Scheithauer BW (2000) Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46:291–302, discussion 302–295 14. Elliott RE, Hsieh K, Hochm T, Belitskaya-Levy I, Wisoff J, Wisoff JH (2010) Efficacy and safety of radical resection of primary and recurrent craniopharyngiomas in 86 children. J Neurosurg Pediatr 5:30–48 15. Elliott RE, Sands SA, Strom RG, Wisoff JH (2010) Craniopharyngioma clinical status scale: a standardized metric of preoperative function and posttreatment outcome. Neurosurg Focus 28:E2 16. Esposito F, Dusick JR, Fatemi N, Kelly DF (2007) Graded repair of cranial base defects and cerebrospinal fluid leaks in transsphenoidal surgery. Neurosurgery 60:295–303, discussion 303–294

17. Frazier JL, Chaichana K, Jallo GI, Quinones-Hinojosa A (2008) Combined endoscopic and microscopic management of pediatric pituitary region tumors through one nostril: technical note with case illustrations. Child's Nerv Syst: ChNS: Off J Int Soc Pediatr Neurosurg 24:1469–1478 18. Hadad G, Bassagasteguy L, Carrau RL, Mataza JC, Kassam A, Snyderman CH, Mintz A (2006) A novel reconstructive technique after endoscopic expanded endonasal approaches: vascular pedicle nasoseptal flap. Laryngoscope 116:1882–1886 19. Im SH, Wang KC, Kim SK, Chung YN, Kim HS, Lee CH, Cho BK (2003) Transsphenoidal microsurgery for pediatric craniopharyngioma: special considerations regarding indications and method. Pediatr Neurosurg 39:97–103 20. Jane JA Jr, Prevedello DM, Alden TD, Laws ER Jr (2010) The transsphenoidal resection of pediatric craniopharyngiomas: a case series. J Neurosurg Pediatr 5:49–60 21. Jang YJ, Kim SC (2000) Pneumatization of the sphenoid sinus in children evaluated by magnetic resonance imaging. Am J Rhinol 14:181–185 22. Kassam AB, Gardner PA, Snyderman CH, Carrau RL, Mintz AH, Prevedello DM (2008) Expanded endonasal approach, a fully endoscopic transnasal approach for the resection of midline suprasellar craniopharyngiomas: a new classification based on the infundibulum. J Neurosurg 108:715–728 23. Kassam AB, Thomas A, Carrau RL, Snyderman CH, Vescan A, Prevedello D, Mintz A, Gardner P (2008) Endoscopic reconstruction of the cranial base using a pedicled nasoseptal flap. Neurosurgery 63: ONS44-52; discussion ONS52-43 24. Laws ER Jr (1994) Transsphenoidal removal of craniopharyngioma. Pediatr Neurosurg 21(Suppl 1):57–63 25. Laws ER Jr, Trautmann JC, Hollenhorst RW Jr (1977) Transsphenoidal decompression of the optic nerve and chiasm. Visual results in 62 patients. J Neurosurg 46:717–722 26. Learned KO, Adappa ND, Loevner LA, Palmer JN, Newman JG, Lee JY (2012) MR imaging evaluation of endoscopic cranial base reconstruction with pedicled nasoseptal flap following endoscopic endonasal skull base surgery. Eur J Radiol 27. Lee JM, Palmer JN (2011) Indications for the osteoplastic flap in the endoscopic era. Curr Opin Otolaryngol Head Neck Surg 19:11–15 28. Locatelli D, Castelnuovo P, Santi L, Cerniglia M, Maghnie M, Infuso L (2000) Endoscopic approaches to the cranial base: perspectives and realities. Child's Nerv Syst: ChNS: Off J Int Soc Pediatr Neurosurg 16:686–691 29. Locatelli D, Massimi L, Rigante M, Custodi V, Paludetti G, Castelnuovo P, Di Rocco C (2010) Endoscopic endonasal transsphenoidal surgery for sellar tumors in children. Int J Pediatr Otorhinolaryngol 74:1298–1302 30. Maira G, Anile C, Rossi GF, Colosimo C (1995) Surgical treatment of craniopharyngiomas: an evaluation of the transsphenoidal and pterional approaches. Neurosurgery 36:715–724 31. Rigante M, Massimi L, Parrilla C, Galli J, Caldarelli M, Di Rocco C, Paludetti G (2011) Endoscopic transsphenoidal approach versus microscopic approach in children. Int J Pediatr Otorhinolaryngol 75:1132–1136 32. Tan HK, Ong YK, Teo MS, Fook-Chong SM (2003) The development of sphenoid sinus in Asian children. Int J Pediatr Otorhinolaryngol 67:1295–1302 33. Teo C (2005) Application of endoscopy to the surgical management of craniopharyngiomas. Childs Nerv Syst 21:696–700