
==== Front
Plast Reconstr Surg Glob Open
Plast Reconstr Surg Glob Open
GOX
Plastic and Reconstructive Surgery Global Open
2169-7574
Lippincott Williams & Wilkins Hagerstown, MD

GOX-D-24-00486
00032
10.1097/GOX.0000000000006145
3
Craniofacial/Pediatric
Case Report
Ectopic Nasal Septum Neuroglial Tissue: A Case Review
Sayegh Odai MBBS, FACS *
Jumei’an Ala’ MD *
Ababneh Hamza MBBS, JBGS, JBPRS *
Azaizeh Alhareth MBBS †
Alnaser Mutaz MBBS, FACS, JBGS, JBPRS *
Aljader Mutaz MD *
Ellati Riyad MD, JBGS *
Al Bdour Mohammad MD *
From the * Royal Jordanian Rehabilitation Center, Department of Plastic and Reconstructive Surgery, Amman, Jordan
† Department of Pathology, Royal Medical Services, Amman, Jordan.
Hamza Ababneh, MBBS, JBGS, JBPRS, Department of Plastic and Reconstructive Surgery, King Hussein Medical Center, King Abdullah II St 230, Amman, Jordan 11822, E-mail: Hamza.ababneh@gmail.com
9 2024
10 9 2024
12 9 e61454 5 2024
24 7 2024
Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

Summary:

Ectopic neuroglial tissue, or heterotopic brain tissue, presents a rare congenital condition characterized by the presence of benign neuroglial cells outside the central nervous system. Diagnosing neuroglial heterotopia solely based on clinical examination and radiological tests can be challenging, necessitating confirmation through histopathologic examination. These lesions may mimic other conditions such as dermoid cysts, pilar cysts, or lipomas. Here, we report a case of a 19-month-old boy with a mass lesion on the dorsum of the nose since birth. The lesion was initially suspected to be a congenital dermoid cyst, and the patient underwent surgical excision under general anesthesia. However, histopathological examination revealed it as an isolated nasal septum neuroglial heterotopia.

OPEN-ACCESSTRUE
COUNTRYJORDAN
SDCT
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pmcNeuroglial heterotopia is a rare congenital anomaly that occurs outside the cranial cavity without any link to the subarachnoid space.1 Heterotopia denotes the presence of regular neurons in an atypical location.2 These anomalies more commonly affect the nasal cavity.3

Ectopic brain tissue, a rare developmental anomaly, typically exerts minimal impact on neurological development. Occasionally, it may occur with other congenital deformities such as cardiac anomalies or cleft palate.4 Prognostically, ectopic brain tissue usually carries a favorable prognosis, as it lacks the capacity to infiltrate adjacent tissues or metastasize.4 The literature reports instances of ectopic neuroglial tissue in the nasal cavity, and less frequently in areas such as the tongue, pharynx, respiratory system, and sacrococcygeal region.2

Computed tomography (CT) scans and magnetic resonance imaging play vital roles in preoperative planning. Past case reports have primarily showcased CT images of neuroglial heterotopia, depicting a low-attenuation mass with focal cystic formations in some areas.3

Here, we present the case of a 19-month-old boy with a mass lesion on the dorsum of the nose, present since birth. A surgical excision under general anesthesia was performed, initially suspected to be a congenital dermoid cyst. However, the final histopathology report confirmed it as an isolated nasal septum neuroglial heterotopia.

CASE REPORT

A 19-month-old boy, previously healthy with no other congenital anomalies, who was seen at our plastic and reconstructive surgery clinic, presented with a slowly growing mass on the dorsum of the nose, which had been present since birth. [See figure, Supplemental Digital Content 1, which shows preoperative image of the mass (2 months old). http://links.lww.com/PRSGO/D486.] [See figure, Supplemental Digital Content 2, which shows preoperative image of the mass (1 year old). http://links.lww.com/PRSGO/D487.] The patient was born full-term via normal vaginal delivery, with no history of admission to the neonatal intensive care unit. On examination, the mass appeared cystic, exhibiting free mobility under the skin, with no tenderness or skin changes observed.

Further investigations included a routine laboratory workup and a head CT scan without contrast. The CT scan revealed a well-defined oval-shaped soft-tissue density mass lesion measuring approximately 1.5 × 2.3 cm, located within the subcutaneous fat of the left nasal side, with no extension into the left orbit. Additionally, the mass appeared to extend into the anterior aspect of the left nostril cavity, where it merged with the thickened cartilaginous part of the nasal septum.

The treatment plan was discussed with the parents, and the patient was planned for surgery under general anesthesia. Methylene blue dye was injected into the mass during dissection to detect any rupture or deep connections (Fig. 1).

Fig. 1. Intraoperative image for the mass during dissection.

After complete mass dissection, the mass base was dissected off the depressed and deformed nasal bone. An obliterated band extending to the nasal septum was noted, with no methylene blue extravasation postexcision. Closure involved deep dermal sutures with Monocryl 5/0 and skin sutures with Vicryl 7/0, followed by steristrip dressing. The child was discharged the next day with oral cephalexin and paracetamol. At the 2-week postoperative follow-up, the patient’s recovery was smooth, and the parents were content with the fine scar on the dorsum of the nose.

The mass measured approximately 15 × 15 × 10 mm and appeared rounded and irregular on the outer surface, with a homogenous white cut surface upon gross examination. Microscopic analysis of the sections revealed a lesion comprising neuroglial cells and astrocytes embedded in fibrous and vascular connective tissue (Fig. 2).

Fig. 2. High-power view showing neuroglial tissue embedded in a fibrous and vascular connective tissue (hematoxylin and eosin).

Ancillary studies were conducted, including glial fibrillary acidic protein (GFAP) immunohistochemical staining , which highlighted the neuroglial tissue, indicating the absence of malignancy (Fig. 3). One-year follow-up showed no scar and no recurrence of any mass (Fig. 4).

Fig. 3. GFAP immunohistochemical stain highlighting the neuroglial tissue.

Fig. 4. Postoperative 12-month follow-up.

DISCUSSION

Heterotopia, as described by Willis,5 serves as a comprehensive term, encompassing diverse manifestations of ectopic and misplaced tissues, irrespective of their mode of inheritance. Within the brain, heterotopic brain tissue denotes displaced neuroglial tissue that lacks connection with the central nervous system.5 We present a case involving a 19-month-old boy exhibiting a mass lesion on the dorsum of the nose, evident since birth. Subsequent confirmation identified it as an isolated nasal septum neuroglial heterotopia.

Head and neck subcutaneous masses encompass a broad spectrum of potential diagnoses, necessitating a comprehensive differential assessment.6 Given this complexity, an interdisciplinary team approach is essential to optimize patient satisfaction, enhance quality of life, ensure proper care, and minimize the risk of complications.4 Special consideration should be given to lesions present since birth, as in our case, highlighting the importance of thorough preoperative investigations and consultations when necessary. This collaborative approach ensures comprehensive evaluation, accurate diagnosis, and tailored management strategies for each patient.

The pathogenesis of heterotopic neuroglial tissue remains unclear, with various proposed mechanisms. CT and magnetic resonance imaging scans are essential complementary studies in preoperative planning. They help determine the extent and location of the mass while excluding any intracranial and central nervous system connections.7,8 In our case, the diagnosis was established using a head CT scan without contrast. This imaging modality revealed a well-defined oval-shaped soft-tissue density mass lesion.

Treatment for neuroglial heterotopia usually entails surgical excision, intending to improve both aesthetic appearance and function.4,7 Early intervention is crucial, particularly before the child solidifies a photographic memory and forms social connections during preschool and school years, thereby enhancing the overall quality of life.4 In our case, the patient underwent surgical resection, involving complete resection of the mass. The child was discharged the day after undergoing surgery.

Recurrence due to incomplete excision has been documented,9 a recent systematic review showed that recurrence occurred in 14 of 72 patients within first-year follow-up.7 Although focal areas of immature cells have been noted,10 these masses do not exhibit invasive patterns and are not considered true neoplasms. Our patient underwent a 2-week follow-up during which his recovery was uneventful. The parents expressed satisfaction with the appearance of a fine scar on the dorsum of the nose. Seventeen months after the operation, the patient showed complete healing without any visible scarring or distortion in nasal appearance.

In conclusion, ectopic benign brain tissue represents rare lesions, often posing diagnostic challenges. They should be included in the differential diagnosis of neonatal head and neck masses.

DISCLOSURE

The authors have no financial interest to declare in relation to the content of this article.

PATIENT CONSENT

Parents or guardians provided written consent for the use of the patient’s image.

ETHICAL APPROVAL

The institutional review board at Royal Medical Services approved the study.

HELSINKI DECLARATION

The study was performed in accordance with the principles of the Declaration of Helsinki, 1975.

Supplementary Material

Published online 10 September 2024.

Disclosure statements are at the end of this article, following the correspondence information.

Related Digital Media are available in the full-text version of the article on www.PRSGlobalOpen.com.
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