Endoscopic Endonasal Repair of a Persistent Craniopharyngeal Canal and Sphenoid Meningoencephalocele: Case Report and Review of Literature

Accepted Manuscript Endoscopic endonasal repair of a persistent craniopharyngeal canal and sphenoid meningoencephalocele: Case report and review of literature Dr Gopikrishnan Rajasekar, MCh, Fellow, Dr Prakash Nair, MCh, Dr Mathew Abraham, MCh, Dr Palak Jaiswal, MS, Dr A.N. Deepti, MD PII:

S1878-8750(18)32436-7

DOI:

https://doi.org/10.1016/j.wneu.2018.10.138

Reference:

WNEU 10582

To appear in:

World Neurosurgery

Received Date: 13 July 2018 Revised Date:

20 October 2018

Accepted Date: 22 October 2018

Please cite this article as: Rajasekar G, Nair P, Abraham M, Jaiswal P, Deepti AN, Endoscopic endonasal repair of a persistent craniopharyngeal canal and sphenoid meningoencephalocele: Case report and review of literature, World Neurosurgery (2018), doi: https://doi.org/10.1016/ j.wneu.2018.10.138. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

ACCEPTED MANUSCRIPT Title: Endoscopic endonasal repair of a persistent craniopharyngeal canal and sphenoid

Type of manuscript: Case Report Running title: Endoscopic repair of persistent craniopharyngeal canal

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meningoencephalocele: Case report and review of literature

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Keywords: Persistent craniopharyngeal canal, endonasal repair, endoscopic repair, sphenoidal meningoencephalocele

Authors:

Fellow in Skull Base Neurosurgery, Department of Neurosurgery,

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1.Dr. Gopikrishnan Rajasekar, MCh

Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India

2.Dr. Prakash Nair, MCh Assistant Professor, Department of Neurosurgery,

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E-mail: [email protected]

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Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India

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E-mail: [email protected]

3.Dr. Mathew Abraham, MCh

Professor and Head of the Department, Department of Neurosurgery,

Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India E-mail: [email protected]

4.Dr. Palak Jaiswal, MS Senior Resident,

ACCEPTED MANUSCRIPT Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India E-mail: [email protected]

5.Dr. Deepti A.N., MD

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Associate Professor, Department of Pathology

Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India

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E mail: [email protected]

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Correspondence Dr. Prakash Nair Assistant Professor, Department of Neurosurgery,

Sree Chitra Tirunal Institute for Medical Sciences and Technology,

Tel No: 8592833489 E-mail: [email protected]

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Trivandrum,India

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Funding: No funding was obtained from any source for the preparation of this manuscript

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Declarations of interest: none

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Title:

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Endoscopic endonasal repair of a persistent craniopharyngeal canal and sphenoid meningoencephalocele: Case report and review of literature Background:

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The persistent craniopharyngeal canal (PCC) is a well-corticated defect in the body of the sphenoid extending from the floor of the sella to the alae of the vomer1. The PCC occurs as the result of non-obliteration of the

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craniopharyngeal canal which, during embryological development, passes between the pre-sphenoid and post sphenoid cartilages. Others have attributed this canal to a nutrient artery within the sphenoid or to cerebral hetertopia1,2. The canal may be incidentally seen on imaging for an unrelated cause or may be found when a child presenting with a nasopharyngeal mass is evaluated. An association has been noted been disorders of

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adenohypophyseal migration and the cleft lip, cleft palate and other midline fusion anomalies3,4. While it is often an incidental radiological finding, in symptomatic patients, the PCC manifests itself as meningoencephalocoeles in the nasopharynx causing respiratory obstruction, with CSF rhinorrhoea or as

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recurrent episodes of meningitis.

The conventional modality of repair for the PCC is through transcranial or trans facial approach, usually

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designed to tackle the nasopharyngeal mass and the skull base defect simultaneously. The purely endonasal approach has also been described, however, the literature on this approach is still sparse. We describe a purely endoscopic endonasal approach for a PCC in a five-year-old child who had a large nasopharyngeal meningoencephalocele, CSF rhinorrhoea and an episode of bacterial meningitis.

Case report:

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A five-year-old female child born of nonconsanguineous parents presented with bacterial meningitis and active CSF rhinorrhoea from her left nostril. Her mother had no history of taking teratogenic medication before conception. The antenatal period was uneventful, and the child was born through an uncomplicated vaginal

years. No nasopharyngeal mass was noted during the surgery.

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delivery. She had cleft palate diagnosed at birth and underwent surgery for repair at the age of one and a half

MR imaging done to evaluate the nasopharyngeal mass, showed a large meningoencephalocoele in the left

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nasopharynx, the neck of the sac could be traced into the subarachnoid space through a canal in the sphenoid bone, starting just anterior to the sella and the pituitary, no cerebral contents were seen herniating through the

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defect (Fig 1). CT imaging and reconstruction (Fig 2) showed the cranial end of the defect starting at the tuberculum sellae and the chiasmatic sulcus, passing through the non-pneumatised body sphenoid, opening inferiorly through the ala of the vomer on the left side.

She had undergone a prior transcranial surgery for the CSF rhinorrhoea, during which the sellar opening was

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occluded, however, she continued to have CSF rhinorrhoea following the procedure. We then decided upon an endonasal approach for excising the PCC and the meningoencephalocele, and to repair the resulting skull base defect. Post-operative course was uneventful with resolution of the CSF rhinorrhea. At seven months follow-up,

Technique:

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the patient was symptom free and an MRI showed no residual nasopharyngeal mass (Fig 3).

The approach was performed using a 0 degree and a 30 degree, 4mm sinus endoscope (@Karl Storz, Tuttlingen, Germany) utilizing a bi-nostril, 2 surgeon technique. A lumbar drain was inserted just before the start of the procedure through which fluorescein dye was instilled in the subarachnoid space. A right-sided nasoseptal flap based on the posterior nasal branch of the right sphenopalatine artery was elevated and stored in the choana. A pulsatile meningoencephalocele was seen occupying the left nasal cavity. This sac was submucosally dissected and the neck of the sac was identified at the floor of the sphenoid sinus which was continuous with the PCC.

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The nasal end of the canal was cut, following which the PCC was dissected by circumferentially drilling away the bone around the PCC. The canal was seen entering the skull-base through the tuberculum. We removed a part of the tuberculum to identify the cranial opening of the canal and normal dural margins. The entire course

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of the PCC from the nasopharynx to the opening at the skull-base dura had been delineated following which it was excised. The resulting skull base defect was then repaired in a multilayered fashion with intradural fat inlay, onlay fascia lata gasket sealed in place with cartilage, and then the nasoseptal flap. The lumbar drain was

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removed after overnight CSF drainage.

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Histopathology:

The histopathological examination of the resected craniopharyngeal canal showed fibro-collagenous tissue with few glial islands (Figure 4A), highlighted by GFAP immunostain (Figure 4B). There was no epithelial lining, epithelial rests in the stroma as indicated negative staining for CAM 5.2 (Figure 4C). There was no

Discussion:

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glial islands in the stroma.

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adenohypophyseal tissue or tumor. The encephalocele sac was covered by respiratory mucosa and contained

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The PCC is a well-corticated defect in the sphenoid extending from the floor of the sella to the roof of the nasopharynx, with an incidence of 0.42% in population.1 The description of a foramen in the hypophyseal fossa, as well as its pharyngeal opening was described by Virchow1. The first description of the PCC in an infant was made by Landzert in 1868.1 Abele et al while reviewing the incidence of the CC divided them into three types incidental canals(type1), canals with ectopic adenohypophysis (type 2) and canals containing cephaloceles (type 3A), tumours (type 3B), or both (type 3C), including pituitary adenoma, craniopharyngioma, dermoid, teratoma, and glioma.3The present case under study can be categorised at a type 3A.

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The pituitary gland develops from the fusion of the adenohypophyseal anlage which starts as an invagination at the roof of the stomodeum and the neurohypophyseal anlage of the diencephalon. The adenohypophyseal anlage when migrating cranially elongates into a narrow stalk, which lies between the pre and post sphenoid cartilages.

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the stalk then separates from the foregut following the fusion of the pre and post sphenoid cartilage. Defective fusion of the sphenoid cartilages results in a canal within the sphenoid bone extending variably from the sella to the vomer. Disorders of adenohypophyseal migration have been reported not just in human but also in many

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primates and give rise to a spectrum of anomalies such as the pharyngeal hypophysis, pituitary gland ectopia, infrasellar craniopharyngiomas and nasopharyngeal teratomas.3,5 This anomaly often co-exists with other

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midline fusion defects like the cleft palate.4 The occurrence of PCC has been explained by certain alternate theories ascribing the phenomenon to non-obliteration of a vascular channel which passes through the same path, after the fusion of the cartilages.1,6 Another postulate is that heterotypic migration of neuronal tissue thought clefts in the developing sphenoid cartilage result in PCC.2 In our patient, the canal started just anterior

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to the pituitary fossa, coursing through the body of the sphenoid to open into the nasopharynx through the vomer. The canal was in direct communication with the subarachnoid space. She also had cleft palate at birth which was surgically corrected. We therefore are of the view that, the, simultaneous occurrence of cleft palate

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and PCC, as noted in our patient, as well as the presence of neuroglia within the canal, supports the theory that PCC results from incomplete fusion of the pre and post sphenoid cartilages, rather than cerebral heterotopia or a

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persistent vascular channel.

The presentation in these cases can vary from nasal masses causing respiratory obstruction to recurrent episodes of meningitis due to CSF leaks. In children undergoing procedures for nasal masses, unrecognized intracranial extension can result in CSF leaks, pituitary dysfunction and meningitis. Other rare presentations include seizures7, bilateral diminution of vision8 and PHACE syndrome3 (Table 1). The age at presentation in the literature ranges from less than one year9,10 to 20 years7 The present case is an illustration of how endoscopic endonasal techniques preclude morbid procedures involving facial incisions and maxillofacial osteotomies that are not just disfiguring, but also affect the

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development of the midface. The growth of the mid face continues till the age of 17-18 years. Osteotomies and resection of growing bone in this period is often associated with malocclusion11, impairment of dentition and airway obstruction.12 In addition to this, anosmia is also known to occur following surgery.12 The transcranial

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route to occlude the proximal end may prevent CSF leak, but identifying the defect and occluding the cranial end requires brain retraction and dissection around the optic chiasma and the pituitary.13 Even following such an intervention, the identification of the defect and obliteration of the fistula is far from satisfactory. By using the

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endonasal corridor we were able to identify the encephalocele within the nasopharynx, and then dissect the PCC tract leading up to the skull base. The resection of nasal encephalocele occluding the choana relieved the

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obstruction restored the nasal air passage.

We routinely use lumbar drain in cases of CSF rhinorrhea for fluorescein infusion. Flourescein helps in identifying the site of CSF leak and in ensuring the absence of leak once the repair is completed. We do not routinely keep the lumbar drain open postoperatively to facilitate healing of the repair except when the defect is

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expected to be large (eg.Anterior skull base meningiomas). Routine use of post-operative CSF drainage may not be beneficial for small CSF leaks.14

Repair of basal encephaloceles have significantly improved since Fenger’s description of a repair through a

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superior maxillotomy.13 Transpalatal approaches either through an intact palate or through a cleft palate have been described for repair of basal meningoencephaloceles, especially in neonates in whom endoscopic

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procedures are not suitable.13,15 The cleft palate offers a unique surgical corridor for approaching the skull base in such cases. Formica et al15 have described the repair of an enephalocele in an 8-month old patient through a cleft palate (Table 1).Presently, the endoscopic endonasal approach is the preferred technique for repair of anterior meningoencephaloceles.16 There are only few reported cases in the literature of an endonasal repair of a persistent craniopharyngeal canal. Chen17 has reported a case of supra and infrasellar craniopharyngioma with PCC in an eight-year old child , operated through a frontotemporal craniotomy and transnasal endoscopy. Biswas et al have reported a case of

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recurrent pneumococcal meningitis with a persistent craniopharyngeal canal in a five-year old child, repaired through an endoscopic approach. Mohindra et al18 (Table 1) have described an endonasal repair for a PCC where they transected the canal from the encephalocoele and occluded the base of the sphenoid.18 Unlike their

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described technique, we resected the entire tract up to the skull base and reconstructed the skull base with bone and a nasoseptal flap. The fate of such a tract that remains is uncertain, with reports of craniopharyngiomas arising from within the canal as also with the view that future pneumatization of the sphenoid might lead to this

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fibro collagenous canal lying within a pneumatised sphenoid, with no bone to support it, predisposing it to

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another CSF leak.

Conclusion:

The extended endonasal approach can be utilized to treat PCC with nasopharyngeal encephaloceles in young children. The approach is suitable to address both the conditions at the same time. The EEA avoids potentially

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morbid trans facial approaches and at the same time it can help in earlier recovery following surgery.

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References Arey LB. The craniopharyngeal canal reviewed and reinterpreted. Anat Rec. 106(1):1-16. doi:10.1002/ar.1091060102

2.

Kau T, Gysin C, Dohmen-Scheufler H, et al. Transsphenoidal extension of heterotopic glioneuronal tissue: pathoanatomic considerations in symptomatic neonates. Childs Nerv Syst. 2011;27(5):771–778. doi:10.1007/s00381-010-1326-4

3.

Abele TA, Salzman KL, Harnsberger HR, Glastonbury CM. Craniopharyngeal Canal and Its Spectrum of Pathology. Am J Neuroradiol. 2014;35(4):772–777. doi:10.3174/ajnr.A3745

4.

Holanda MM de A, Rocha AB, Santos RHP, Furtado PGC. Basal sphenoethmoidal encephalocele in association with midline cleft lip and palate: case report. Radiol Bras. 2011;44(6):399-400. doi:10.1590/S0100-39842011000600013

5.

Leela K, Kanagasuntheram R. Craniopharyngeal Canal and Pharyngeal Hypophysis in the Siamang Gibbon (Symphalangus syndactylus). Folia Primatol (Basel). 1971;14(1-2):118-122.

6.

Alonso RC, de la Peña MJ, Caicoya AG, Rodriguez MR, Moreno EA, de Vega Fernandez VM. Spontaneous Skull Base Meningoencephaloceles and Cerebrospinal Fluid Fistulas. RadioGraphics. 2013;33(2):553-570. doi:10.1148/rg.332125028

7.

Marsot-Dupuch K, Smoker WRK, Grauer W. A Rare Expression of Neural Crest Disorders: An Intrasphenoidal Development of the Anterior Pituitary Gland. Am J Neuroradiol. 2004;25(2):285–288.

8.

Akyel NG, Alımlı AG, Demirkan TH, Sivri M. Persistent craniopharyngeal canal, bilateral microphthalmia with colobomatous cysts, ectopic adenohypophysis with Rathke cleft cyst, and ectopic neurohypophysis: case report and review of the literature. Childs Nerv Syst. February 2018. doi:10.1007/s00381-018-3747-4

9.

Currarino G, Maravilla KR, Salyer KE. Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. Am J Neuroradiol. 1985;6(1):39–43.

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SC

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1.

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10. Cohen R, Nelson MD, Segall HD. Epignathic teratoma associated with craniopharyngeal canal. Am J Neuroradiol. 1989;10(3):652–652. 11. Fritsch MJ, Singh RV, Morcos JJ. The transmaxillary approach (level V). Oper Tech Neurosurg. 2000;3(1):25-43. doi:10.1016/S1092-440X(00)80005-5 12. Kassam A, Carrau R, Kanaan H. Fully endoscopic expanded endonasal approach treating skull base lesions in pediatric patients. J Neurosurg. 2007;106:12. 13. Hoff SR, Edwards MSB, Bailey CM, Koltai PJ. The Transpalatal Approach to Repair of Congenital Basal Skull Base Cephaloceles. J Neurol Surg Part B Skull Base. 2014;75(2):96-103. doi:10.1055/s-00331358374 14. Casiano RR, Jassir D. Endoscopic Cerebrospinal Fluid Rhinorrhea Repair: Is a Lumbar Drain Necessary? Otolaryngol-Head Neck Surg. 1999;121(6):745-750. doi:10.1053/hn.1999.v121.a98754

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15. Formica F, Iannelli A, Paludetti G, Di Rocco C. Transsphenoidal meningoencephalocele. Childs Nerv Syst. 2002;18(6):295–298. doi:10.1007/s00381-002-0578-z 16. Nyquist GG, Anand VK, Mehra S, Kacker A, Schwartz TH. Endoscopic endonasal repair of anterior skull base non-traumatic cerebrospinal fluid leaks, meningoceles, and encephaloceles. J Neurosurg. 2010;113(5):961-966. doi:10.3171/2009.10.JNS08986

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17. Chen CJ. Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology. 2001;43(9):760–762. doi:10.1007/s002340100581

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18. Mohindra S, Gupta K, Mohindra S. A novel minimally invasive endoscopic repair in a case of spontaneous CSF rhinorrhea with persistent craniopharyngeal canal. Neurol India. 2015;63(3):434. doi:10.4103/00283886.158243 19. Hughes ML, Carty AT, White FE. Persistent hypophyseal (craniopharyngeal) canal. Br J Radiol. 1999;72(854):204-206. doi:10.1259/bjr.72.854.10365075

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20. Kizilkilic O, Yalcin O, Yildirim T, Sener L, Parmaksiz G, Erdogan B. Hypothalamic Hamartoma Associated with a Craniopharyngeal Canal. Am J Neuroradiol. 2005;26(1):65–67. 21. Pinilla-Arias D, Hinojosa J, Esparza J, Muñoz A. Recurrent meningitis and persistence of craniopharyngeal canal: case report. Neurocirugía. 2009;20(1). doi:10.4321/S1130-14732009000100007

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22. Ma J, Huang Q, Li X, et al. Endoscopic transnasal repair of cerebrospinal fluid leaks with and without an encephalocele in pediatric patients: from infants to children. Childs Nerv Syst. 2015;31(9):1493–1498. doi:10.1007/s00381-015-2746-y

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23. Biswas S, Millward CP, Riordan A, Sinha A, Avula S. Craniopharyngeal duct: a cause of recurrent meningitis. BJRcase Rep. 2015;1(3):20150022. doi:10.1259/bjrcr.20150022

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Legend: Figure 1

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MRI T2 weighted sagittal sections show a tubular defect with well defined borders arising from just in front of the sella measuring 5mm in cross section and 14mm in length(A) and a large meningoencephalocele in the left nasopharynx(B)

Figure 2

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CT shows the PCC starting as a well corticated defect at the tuberculum sellae (B, C), the canal then passes through the non-pneumatised sphenoid, opening inferiorly through the ala of the vomer(A) on the left side and a soft tissue density consistent with a meningoencephalocoele is seen in the left nasopharynx (D). CT reconstruction shows the cranial end of the canal just in front of the sella (E) and the caudal end in the ala of the vomer (F)

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MRI study seven months after the procedure. T2 weighted sagittal image shows no evidence of meningomyelocele in the nasal cavity (A). Midsagittal CT scan shows the residual cranial end of the canal(B)

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Figure 4

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The histopathological examination of the resected craniopharyngeal canal. Fibro-collagenous tissue with few glial islands(A), highlighted by GFAP immunostain (B).There was no epithelial lining, epithelial rests in the stroma as indicated negative staining for CAM 5.2(C).

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Table 1: Reported cases of Persistent Craniopharyngeal canal in literature and their management Cases

Age

1985

2

1.Infant 2.Infant

Cohen et al10

1989

1

1. 5-6mm

Endocrine Dysfunction

Presentation

1.Asymptomatic 2.Asymptomatic

1.None 2.None

Infant

Asymptomatic

None

10 mm

1.Airway obstruction 2.Delay in puberty

+

1 mm

Decreased visual acuity

NA

Content

1.None 2.None

Associated Conditions

1999

2

1.21 yrs 2.18 yrs

Chen17

2001

1

8 yrs

Formica et al15

2002

1

8 mons

MarsotDupuch et al7

2004

1

20 yrs

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Hughes et al19

Stridor

8 mm

Seizures

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Currarino et al9

Diameter

NA

None

1.Fetal Dilantin Syndrome (hypertelorism, small fingernails and fingertips, and abnormally formed distal phalanges) 2. Midline facial cleft, bilateral cleft lip and a wide palatal bony defect, hypertelorism, slight hypoplasia of the left orbit and forehead, clubfeet Epignathic teratoma in hard palate

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Year

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Author

-

-

Craniopharyngioma

-

Transcranial followed by endoscopy

Meningoencephalocystocele -Hypothalamus -Optic apparatus - Pituitary structures -Third ventricle

Hypertelorism, cleft palate/lip Partial agenesis of the corpus callosum

Open trans palatal approach

Adenohypophysis

-

-

Packed sphenoid sinus with bone

7 yrs

10mm

Precocious puberty

+

Pituitary gland

PinillaArias et al21

2009

1

4 ½ yrs

13 mm

Pneumococcal meningitis

NA

Meningocele

-

Median4 yrs

Type 10.8 mm Type 23.9 mm Type 39mm

Trauma, PHACE syndrome, Blake remnant, NP mass, Coloboma, Headache, Hydrocephalus, Meningitis,

Dermoid (3), Teratoma (1), Glioma (2), Adenoma (2), Craniopharyngioma (1)

Cleft lip/palate, PHACE Syndrome, Blake remnant, Duplicated pituitary gland, Coloboma, Holoprosencephaly, Cortical

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1

Type1-0/9 Type 2-2/7 Type3-4/13

1.No recurrence at 21 years follow up 2. No recurrence at 2 years -

-

2005

29

-

1.Hypertelorism

Hypothalamic hamartoma, No craniofacial abnormality

2014

Outcome/ Followup* -

-

1.Meningocele 2.None

Kizilkilic et al20

Abele et al3

Management

-

Asymptomatic at 2 years followup -

-

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Table 1: Reported cases of Persistent Craniopharyngeal canal in literature and their management Weakness

6 yrs

Biswas et al23

2015

1

5 yrs

Mohindra et al18

2015

1

17 yrs

NA

NA

Meningoencephalocele

6mm

Recurrent Pneumococcal Meningitis

None

-

18mm

CSF Rhinorrhea

NA

-

Akyel et al8

2018

1

2 yrs

3.1 -7.1 mm

Bilateral diminution of vision

NA

Present case

2017

1

5 yrs

5 mm

CSF Rhinorrhea , meningitis

None

-

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1

-

-

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2015

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Ma et al22

dysplasia, Schizencephaly, Absent right ICA

Ectopic Adenohypophysis

Meningoencephalocele

Bilateral microphthalmia, Colobomatous cyst, Patent Ductus arteriosus, Tetralogy of Fallot

Cleft palate

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* post operative outcomes as reported in the articles

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•

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NA=not available; PHACE=posterior fossa malformations, hemangiomas, arterial abnormalities, cardiac defects, eye abnormalities; yrs=years;

Pedunculated nasoseptal flap with fascia lata Endoscopic trans nasal repair Endoscopic trans nasal repair

No recurrence

No recurrence at 10 months follow up -

-

Endoscopic trans nasal repair

No recurrence at 7 months follow up

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Table 1: Reported cases of Persistent Craniopharyngeal canal in literature and their management

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Abbreviations CAM 5.2 – Cytokeratin

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CSF – Cerebrospinal Fluid CT – Computerised Tomography

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EEA – Extended Endonasal Approach GFAP - Glial fibrillary acidic protein

NA – Not Available PCC -Persistent Craniopharyngeal Canal

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MR – Magnetic Resonance

Eye abnormalities

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Yrs - Years

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PHACE- Posterior fossa malformations, Hemangiomas, Arterial abnormalities, Cardiac defects,