| | External rhinoplasty excision of a nasal tip glioma in a 6-month-old infant: Case report and review of the literatureReceived 5 December 2008; received in revised form 3 March 2009; accepted 8 March 2009. published online 20 April 2009. Summary In this report, we describe an infant with a nasal tip glioma treated by excision via external rhinoplasty. A 3-month-old full-term, otherwise healthy infant presented with a slowly enlarging midline mass of the nasal tip. Clinically the mass appeared cystic without trans-illumination, enlargement with crying, or signs of infection. On successive CT scans at 3 and 5 months old the mass appeared as an enlarging focal prominence to the left of midline with central hypodensity without evidence for intracranial extension. Preoperatively the differential diagnosis included nasal dermoid, glioma, and hemangioma. The mass was removed at 6 months of age via an open rhinoplasty approach with conchal cartilage grafting to reconstruct the left upper lateral cartilage. The final pathological diagnosis was nasal glioma (glial heterotopia) with fibrosis. Pre-operative, intra-operative, and post-operative photos were taken. The patient has been followed up for 6 months without evidence of recurrence or other complications. A review of the literature on nasal glioma and external nasal surgery in infancy is presented. 1. Case review  A 3-month-old full-term, healthy female was referred for a slowly enlarging midline mass of the nasal dorsum and tip. Birth, medical, surgical, social, family history were unremarkable. Review of systems was negative for noisy or disordered breathing awake or asleep, distress, rhinorrhea, nasal obstruction, proptosis or signs of chronic or recurrent infection of the mass. On exam, there was a 4 cm cystic-appearing mass on the nasal dorsum and tip (Fig. 1). There was no fluctuance, erythema, evidence of a pit or sinus tract. The mass did not trans-illuminate or enlarge with crying. Furstenburg's sign was negative. The nares were patent bilaterally and without rhinorrhea. The remaining head and neck exam findings were normal. Serial CT scans (Fig. 2) showed an enlarging, relatively homogenous, spherical, non-enhancing mass with central hypodensity without extension to the anterior cranial fossa. The differential diagnosis was nasal dermoid, hemangioma, and glioma. Due to progressive growth and mass effect on nasal tip structures, early surgical intervention was recommended. An open external rhinoplasty approach was used at 6-months of age (Fig. 3, Fig. 4). Grossly, the mass was tan-pink, rubbery, and homogenous on cut section. It was located between the lower lateral cartilages, displacing them laterally and replaced part of the caudal end of the left upper lateral cartilage (ULC). There was no intracranial extension. After extirpation, the ULC defect was reconstructed with ipsilateral conchal cartilage (Fig. 4c). There were no complications. Histologic findings (Fig. 5) were consistent with nasal glioma (glial heterotopia) with fibrosis. There were no post-operative complications. The family was pleased with the results and happily submitted post-operative family photographs several weeks after surgery (Fig. 6). On 6-month post-operative follow-up, the family remained pleased. 2. Discussion Fewer 250 nasal gliomas have been reported in the literature and the largest series included 10 cases over 32 years [1], [2]. Their location varies: 60% are extranasal, 20% are intranasal and 10% are mixed [1], [2]. Imaging via contrast-enhanced CT or MRI is necessary to rule-out intracranial extension [1]. Incisional biopsy is contraindicated due to risk of CSF leak [1]. MRI may be more sensitive for intracranial extension due to incomplete ossification of the anterior skull base in infants [3], [4]. Nasal gliomas are often mistaken for capillary hemangiomas which also present as bluish or reddish masses with telangectatic surfaces [4]. Some authors have advocated the use of Doppler ultrasonography to help identify subcutaneous hemangioma which will demonstrate greater systolic peak flow velocity than gliomas [5], [6]. Most authors favor an external rhinoplasty approach for the location in this paper although other approaches have been reported including lateral and midline rhinotomy [1]. Endoscopic removal of intranasal tumors has been described [1], [2]. Some authors prefer the term, “nasal glial heterotopia” as nasal gliomas (unlike true gliomas) are not neoplasms [7], [8]. Histologically, there are glial cells in a fibrous collagen background [1], [8]. There can be malignant, astrocytoma-like characteristics although there is no malignant potential [7]. Theories of pathogenesis have attempted to explain nasal gliomas and encephaloceles as a spectrum stemming from normal extension of intracranial contents through the foramen cecum into the prenasal space that fails to involute completely during embryogenesis [1], [8]. The largest nasal glioma case series was reported by Rahbar et al. who described 9 cases in infants over a 32 year period [1]. Five patients underwent intranasal or trans-palatal excision for intranasal masses. In four patients with extranasal gliomas, the authors performed two external rhinoplasty procedures, one lateral rhinotomy, and one midline rhinotomy. No post-operative complications were reported. Oddone et al. also reported good results after open excision of an external nasal glioma in a 17-month-old although they do not describe what approach was used [6]. Although most authors advocate early excision of nasal gliomas, the long term effects of open nasal surgery in infants are not well understood [9], [10]. For nasal obstruction, septorhinoplasty was traditionally delayed until school-ages due to easier post-operative care, improved dissection planes, and deceleration of septal cartilage growth [11]. Recently, authors have advocated earlier intervention and reported good subjective results using both open and closed techniques [9]. There are no data comparing the relative morbidity of septorhinoplasty in infancy with delay of excision midline external nasal tumors. This may present a challenge in counseling families who are reluctant to proceed with surgery. Concern for abnormal septal and midface growth is attenuated by anthropometric data showing little growth impairment after external septoplasty [12], [13]. Most aspects of midface and nasal growth have not been shown to be atypical except for some shortening of the nasal dorsum [14]. Nevertheless, aspects of septorhinoplasty may more technically challenging in infants and children such as identification of medial crura [15]. Clearly, more longitudinal studies of outcomes of septorhinoplasty surgery are needed to better understand the morbidity of performing this surgery in young infants. In the meantime, it seems reasonable to advocate open septorhinoplasty as a cosmetically acceptable option for open resection of external nasal gliomas. Acknowledgements We thank our patient and her parents for contributing family photos. This case report was deemed exempt from need to review by the IRB at Indiana University School of Medicine. This work was previously presented as poster # 121 at the Combined Otolaryngologic Spring Meeting, American Society of Pediatric Otolaryngology section in Orlando, Florida in May 2008. References [1]. [1]Rahbar R, Resto V, Robson C, et al. Nasal glioma and encephalocele: diagnosis and management. Laryngoscope. 2003;113:2069–2077.
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[2]. [2]Rouev P, Dimov P, Shomov G. A case of nasal glioma in a new-born infant. International Journal of Pediatric Otorhinolaryngology. 2001;58:91–94. Abstract | Full Text |
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[4]. [4]Hedlund G. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatric Radiology. 2006;36:647–662. MEDLINE |
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[5]. [5]Dasqupta N, Bentz M. Nasal gliomas: identification and differentiation from hemangiomas. Journal of Craniofacial Surgery. 2003;14(5):736–738. MEDLINE |
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[6]. [6]Oddone M, Granata C, Dalmonte P, Biscaldi E, Rossi U, Toma P. Nasal glioma in an infant. Pediatric Radiology. 2002;32:104–105. MEDLINE |
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[7]. [7]Amin A, Monabati A, Kumar P, Hashemi S. Nasal glioma (neuroglial heterotopia) mimicking an astrocytoma: case report. Ear Nose and Throat Journal. 2005;84(10):657–658. MEDLINE [8]. [8]Patterson K, Kapur S, Chandra R. Nasal gliomas and related brain heterotopias: a pathologists perspective. Pediatric Pathology. 1986;5:353–362. MEDLINE [9]. [9]Emami A, Brodsky L, Pizzuto M. Neonatal septoplasty: a case report and review of the literature. International Journal of Pediatric Otorhinolaryngology. 1996;35(3):271–275. Abstract |
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[10]. [10]Koltai P, Hoehn J, Bailey C. The external rhinoplasty approach for rhinologic surgery in children. Archives of Otolaryngology Head and Neck Surgery. 1992;118(4):401–405. [11]. [11]Crysdale W. Septoplasty in children—yes, but do the right thing. Archives of Otolaryngology Head and Neck Surgery. 1999;125:701. [12]. [12]El-Hakim H, Crysdale W, Abdollel M, Farkas L. A study of anthropometric measures before and after external septoplasty in children: a preliminary study. Archives of Otolaryngology Head and Neck Surgery. 2001;127:1362–1366. [13]. [13]Walker P, Crysdale W, Farkas L. External septorhinoplasty in children: outcome and effect on growth of septal excision and reimplantation. Archives of Otolaryngology Head and Neck Surgery. 1993;119(9):984–989. [14]. [14]Bejar I, Farkas L, Messner A, Crysdale W. Nasal growth after septoplasty in children. Archives of Otolaryngology Head and Neck Surgery. 1996;122(8):816–821. [15]. [15]Crysdale W, Tatham B. External septorhinoplasty in children. Laryngoscope. 1985;95:12–16. Indiana University School of Medicine, Department of Otolaryngology, Head and Neck Surgery, Indianapolis, IN, United States  Corresponding author at: Indiana University School of Medicine, Department of Otolaryngology, Head and Neck Surgery, 699 West Drive, Riley Research Wing 044, Indianapolis, IN 46202, United States. Tel.: +1 317 274 4915; fax: +1 317 274 4949.
PII: S1871-4048(09)00020-3 doi:10.1016/j.pedex.2009.03.002 © 2009 Elsevier Ireland Ltd. All rights reserved. | |
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