- Email: [email protected]
Nasal glial heterotopia or congenital hemangioma? A case report
G Model
JORMAS-34; No. of Pages 4 J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case Report
Nasal glial heterotopia or congenital hemangioma? A case report R. Lartizien a,*, C. Durand b, S. Blaise c, B. Morand a a
Grenoble University Hospital, Department of Maxillo-facial Surgery, Avenue Maquis du Gre´sivaudan, 38700 La Tronche, France Grenoble University Hospital, Department of Pediatric Radiology, Avenue Maquis du Gre´sivaudan, 38700 La Tronche, France c Grenoble University Hospital, Department of Vascular Medicine, Avenue Maquis du Gre´sivaudan, 38700 La Tronche, France b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 11 October 2016 Accepted 29 March 2017
Nasal glial heterotopia (NGH) is a rare benign tumor of the median line. We describe the case of a child presenting a lateral nasal mass. The characteristics of the prenatal ultrasound and the postnatal clinical examination argued in favor of a congenital hemangioma (CH). The MRI performed at 6 weeks of life suggested glial heterotopia. This diagnosis was confirmed by the pathological analysis. Congenital hemangiomas and nasal glial heterotopies have similar clinical presentations. Prenatal ultrasound diagnosis between NGH and CH is difficult. Fetal MRI is not yet highly specific for these two lesions, but it can eliminate an intracerebral connection in cases of NGH. Postnatal exams are more specific. Flow on the Doppler exam is rapid for CH and slow for NGH. On MRI, these two lesions appear as a hypersignal on T2-weighted sequences, but less intense for NGH than for CH. Distinguishing between NGH and CH can be difficult. This does not have a direct incidence on treatment because it is surgical in both cases.
C 2017 Elsevier Masson SAS. All rights reserved.
Keywords: Nasal glial heterotopia Hemangioma Prenatal diagnosis MRI Ultrasound
1. Introduction Nasal glial heterotopia (NGH) is a rare congenital benign tumor. Differential diagnosis of tumors of the median line include tumors with a mesodermal (congenital and infantile hemangiomas, lymphangiomas, angiofibromas, lipomas, etc.), ectodermic (dermoid cysts), and neurogenic origin (encephaloceles, nasal glial heterotopias), and teratomas. The main differential diagnosis of NGH is congenital hemangioma (CH) because their clinical presentations are similar [1–4]. Prenatal ultrasound diagnosis of these two entities is difficult and we discuss the value of fetal and postnatal magnetic resonance imaging (MRI).
2. Clinical case A 27-year-old primipara gravida patient with no particular medical history presented at 23 weeks gestation a sessile mass that had developed on the left anterolateral side of the middle part of the nose of the fetus. It presented a hypoechogenic echostructure with visualization of slow vascular flow. This observation suggested a vascular lesion (Figs. A.1 and 2).
* Corresponding author. E-mail address: [email protected] (R. Lartizien).
The stability of the lesion was confirmed by two ultrasounds at 27 and 32 weeks gestation. A fetal MRI was not requested. The child was born at term (38 GW), by vaginal delivery, with no complications. She presented paramedian swelling of the nasal dorsum that measured 20 mm 20 mm. The mass was firm, noncompressible, bluish, with fine telangiectases on one side. There was no variation in volume when crying (Fig. B.1 and 2). Breathing was not disturbed and the flexible nasoendoscopy ruled out endonasal invasion. An echo-Doppler performed at 8 days of life confirmed the vascularized aspect of the lesion. The clinical examination suggested a congenital hemangioma whose nonregressive aspect argued in favor of a subtype of noninvoluting congenital hemangioma (NICH). Another tumor diagnosis could not be ruled out. An ultrasound as well as an MRI performed at the age of 6 weeks showed a tissue lesion. The MRI was not typical of a hemangioma (absence of hypersignal in T2-weighted images) and ruled out a meningoencephalocele. The diagnosis of nasal glial heterotopia was suggested (Fig. C.1 and 2). Given the unsightly aspect and particularly the risk for amblyopia because of visual field restriction, the tumor was excised at the age of 2.5 months (Figs. D and E). A vertical incision on the left of the lesion was proceed, carrying the skin under the lesion. It was difficult to find a dissection plane between the skin and the lesion. In depth, a cleavage plane was easier between the tumor and the nasal periosteum and the triangular cartilage’s perichondrium. A small mucosal infraction
http://dx.doi.org/10.1016/j.jormas.2017.03.005 C 2017 Elsevier Masson SAS. All rights reserved. 2468-7855/
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
G Model
JORMAS-34; No. of Pages 4 2
R. Lartizien et al. / J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
Figs. A.1 and A.2. Prenatal ultrasound appearance of the nasal mass at 22 week’s gestation in coronal (1) and parasagittal (2) sections.
Figs. B.1 and B.2. Appearance of infant shortly after birth.
Figs. C.1 and C.2. Axial (a) and sagittal (b) T2-W MRI showing a solid mass arising from the left side of the nose with no intracranial extension.
was closed with one point of absorbable suture. The lesion was resected and sent to the pathological analysis. Additional resection of the edge of the skin was performed. The closure was realized in two layers. The final pathological analysis concluded in glial heterotopia with a complete excision in depth but limit at the skin margins.
With eighteen months of follow-up, there were no signs of relapse. 3. Discussion NGH is often labeled CH [2,3]. This can be explained by the clinical variability of the two lesions, their rarity, and the presence
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
G Model
JORMAS-34; No. of Pages 4 R. Lartizien et al. / J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
Fig. D. Surgical resection.
Fig. E. 1 month postoperative view.
of telangiectasias for both of them. MRI can distinguish these two entities more reliably than ultrasound. The clinical presentation of NGH varies. This is a tissue formation, most often polypoid. It is nonpulsatile and does not increase when crying (negative Furstenberg signs), which differentiates it from encephalocele [5]. NGHs have an extranasal presentation in 60% of cases, intranasal in 30% of cases, and 10% are mixed. Telangiectasias are sometimes present and give a pinkish color to the lesion. CHs are vascular tumors. In contrast to infantile hemangiomas, which appear after birth, the CH develops in intrauterine life. The studies conducted by Enjolras [6] classified CHs into two categories: rapidly involuting congenital hemangioma (RICH) and NICH. RICH has a variable clinical aspect. It is a prominent tumor varying from purple to bluish. There can be central necrosis at birth as well as many telangiectasias. This lesion begins to regress in the first weeks of life, over the 1st year, and leaves no more than a scarred area. The NICH is rarer. There is a single round or flat lesion. Very often there is a pale bluish ring in the periphery. The pink or purple color at the surface is related to the presence of telangiectasias. In our patient, the lesion was round and well limited. It was bluish with fine telangiectasias. These characteristics resembled those of a vascular tumor, which participated in the confusion between the two etiologies. The NICH subtype was suggested given the nonregression of the tumor during the first few weeks of life. Clinical monitoring of this type of lesion is important during the first few weeks of life. Indeed, the etiologic diagnosis is not easy
3
and a malignant tumor should never be ruled out. The RICH will regress, the NICH and the NGH will remain stable, whereas a malignant tumor will show rapid growth. The initial diagnosis should be reviewed should any atypical presentation arise. Prenatal monitoring is an important step in the mother’s and fetus’s health. Currently, an ultrasound every trimester is performed. It is possible to detect craniofacial malformations in the second trimester. Most authors recommend a fetal MRI if this type of anomaly is diagnosed [7,8]. The differential diagnosis between NGH and CH is difficult in prenatal ultrasonography. NGH is readily hypoechogenic and clearly limited on the prenatal ultrasound. Doppler analysis can show slow arterial flow or an absence of flow [7–9]. CHs, and particularly RICHs, vary in echogenicity, which is most frequently heterogenous [10,11] but sometimes homogenous [12]. Doppler analysis often shows rapid arterial flow but an absence of flow in certain cases [12]. No prenatal diagnosis of NICH has been reported in the literature. Fetal MRI is not yet specific for NGH but can rule out an intracerebral connection. CHs seem to present a more specific hyperintense aspect on T2weighted sequences (with, however, a hypersignal less pronounced than postnatally) [10]. Only the MRI characteristics of RICH have been described in the literature: a heterogenous lesion with multiple vascular images in the T2-weighted sequence. The intensity of the vascular signals varies. However, this usually hyper-T2 aspect is not always found [10,12]. In our patient, the ultrasound suggested a vascular structure. A fetal MRI would probably have made it possible to specify the diagnosis and perhaps invalidate this hypothesis. The presence of a median line mass should motivate an echoDoppler and an MRI in the early weeks of life. MRI is mandatory to rule out intracerebral extension in the context of encephalocele or the persistence of a fibrous tract in NGH. MRI should be preferred to CT because it does not expose the patient to irradiation and provides clearer results. The lack of ossification in the anterior cranial fossa in newborns can simulate intracerebral communication [11,13]. In cases of CH, the echo-Doppler can identify a mixed tissue and vascular lesion whose echogenicity may be variable depending on the phase of progression of the CH. The lesion is clearly delimited and has high vascular density, comparable to what is observed in arteriovenous malformations. The peak in arterial velocity is high (high flow velocity in the end-diastolic phase) and the peak in venous velocity is generally higher when found in arteriovenous malformations, giving a low-resistance spectrum. Arteriovenous shunts do not exist. The echographic description of NICHs is rarely found, although the aspect is found in CHs. In certain cases, the presence of calcifications has been described [14]. In NGH, the lesion also resembles tissue and is vascularized, but the flow on Doppler is slow (low arterial flow velocity during the end-diastolic phase) [1,3]. The presentation of RICHs and NICHs on MRI is nearly identical: a tumor mass that is relatively homogenous in T1-weighted sequences, enhanced after injection of gadolinium. In T2 the lesion appears as a hypersignal. Vessels are observed as a tubular shadow with no signal in T1 and T2 [6]. For NGHs, the MRI can rule out intracerebral extension. The lesion can also be linked to the subarachnoid space by a fibrous tract that is visible on MRI. This connection is responsible for a risk of an osteomeningeal breach and meningitis. NGH is iso- or hypointense compared to gray matter in T1weighted images. It is enhanced in its center after gadolinium injection. In T2-weighted sequences, it appears slightly hyperintense, less than for CHs [13].
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
G Model
JORMAS-34; No. of Pages 4 R. Lartizien et al. / J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
4
NGH is classically iso-intense in T1 images compared to the signal of the gray matter, heterogenous in T2 images (with a signal that differs little from the cerebral parenchyma signal) and is slightly enhanced after gadolinium injection. NGH and CHs are benign lesions. Their treatment is therefore not urgent. Respiratory obstruction may be an indication for early surgery [5]. A RICH subtype can rapidly be ruled out based on clinical progression. Distinguishing NGH and NICH can be difficult, but this has no direct incidence on treatment because treatment is surgical in both cases and beta-blockers are not effective on NICHs. It is the risk of amblyopia caused by loss of the visual field that motivated early surgery in our patient. The final diagnosis was made by the pathological analysis. The excision was complete (with doubts about skin margins). Due to the slow progression of NGH and the esthetic concerns in the region, simple clinical monitoring is necessary in cases of incomplete excision. Disclosure of interest The authors declare that they have no competing interest. References
[2] Beegun I, Dua R, Connor S, Bentley R. Prenatal diagnosis and management of a craniofacial glioma detected at 20 weeks’ gestation. Case report and review of the literature. Int J Oral and Maxillofac Surg 2012;41:200–2. [3] Hoeger PH, Schaefer H, Ussmueller J, Helmke K. Nasal glioma presenting as capillary hemangioma. Eur J Pediatr 2001;160:84–7. [4] Oddone M, Granata C, Dalmonte P, Biscaldi E, Rossi U, Toma P. Nasal glioma in an infant. Pediatr Radiol 2002;32:104–5. [5] Yapo PC, Diabate AS, Konan E, Dede NS, Kouadio E, Gui LN, et al. Gliome nasopalatin avec obstruction nasale gauche conge´nitale: a` propos d’un cas. J Radiol 2010;91:1325–8. [6] Enjolras O. He´mangiomes conge´nitaux. Ann Dermatol Venereol 2003;130: 367–71. [7] De Biasio P, Sarso E, PREFUMO F, Odella C, Rossi A, Venturini PL. Prenatal diagnosis of a nasal glioma in the mid trimester. Ultrasound Obstet Gynecol 2006;27:571–3. [8] Grzegorezyk V, Brasseur-Daudruy M, Labadie G, Cellier C, Verspyck E. Prenatal diagnosis of nasal glioma. Pediatr Radiol 2010;40:1706–9. [9] Chmait RH, Pretorius DH, Hull AD. Nasal glioma. Ultrasound Obste Gynecol 2002. [10] Elia D, Garel C, Enjolras O, Vermouneix L, Soupre V, Oury JF, et al. Prenatal imaging findings in rapidly involuting congenital hemangioma of the skull. Ultrasound Obstet Gynecol 2008;31:572–5. [11] Uehara S, Hasegawa T, Okuyama H, Kawahara H, Kubota A, Osuga K, et al. Prenatally detected giant congenital hemangioma of the fetal neck. J Ped Surg Case Rep 2013;1:29–31. [12] Brix M, Soupre V, Enjolras O, Vazquez MP. Diagnostic ante´natal des RICH. Rev Stomatol Chir Maxillofac 2007;108:109–14. [13] Hedlung G. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol 2006;36:647–62. [14] Masand P. Radiographic findings associated with vascular anomalies. Semin Plast Surg 2014;28:69–78.
[1] Dasgupta NR, Bentz ML. Nasal gliomas: identification and differentiation from hemangiomas. J Craniofac Surg 2003;14(5):736–8.
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
JORMAS-34; No. of Pages 4 J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case Report
Nasal glial heterotopia or congenital hemangioma? A case report R. Lartizien a,*, C. Durand b, S. Blaise c, B. Morand a a
Grenoble University Hospital, Department of Maxillo-facial Surgery, Avenue Maquis du Gre´sivaudan, 38700 La Tronche, France Grenoble University Hospital, Department of Pediatric Radiology, Avenue Maquis du Gre´sivaudan, 38700 La Tronche, France c Grenoble University Hospital, Department of Vascular Medicine, Avenue Maquis du Gre´sivaudan, 38700 La Tronche, France b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 11 October 2016 Accepted 29 March 2017
Nasal glial heterotopia (NGH) is a rare benign tumor of the median line. We describe the case of a child presenting a lateral nasal mass. The characteristics of the prenatal ultrasound and the postnatal clinical examination argued in favor of a congenital hemangioma (CH). The MRI performed at 6 weeks of life suggested glial heterotopia. This diagnosis was confirmed by the pathological analysis. Congenital hemangiomas and nasal glial heterotopies have similar clinical presentations. Prenatal ultrasound diagnosis between NGH and CH is difficult. Fetal MRI is not yet highly specific for these two lesions, but it can eliminate an intracerebral connection in cases of NGH. Postnatal exams are more specific. Flow on the Doppler exam is rapid for CH and slow for NGH. On MRI, these two lesions appear as a hypersignal on T2-weighted sequences, but less intense for NGH than for CH. Distinguishing between NGH and CH can be difficult. This does not have a direct incidence on treatment because it is surgical in both cases.
C 2017 Elsevier Masson SAS. All rights reserved.
Keywords: Nasal glial heterotopia Hemangioma Prenatal diagnosis MRI Ultrasound
1. Introduction Nasal glial heterotopia (NGH) is a rare congenital benign tumor. Differential diagnosis of tumors of the median line include tumors with a mesodermal (congenital and infantile hemangiomas, lymphangiomas, angiofibromas, lipomas, etc.), ectodermic (dermoid cysts), and neurogenic origin (encephaloceles, nasal glial heterotopias), and teratomas. The main differential diagnosis of NGH is congenital hemangioma (CH) because their clinical presentations are similar [1–4]. Prenatal ultrasound diagnosis of these two entities is difficult and we discuss the value of fetal and postnatal magnetic resonance imaging (MRI).
2. Clinical case A 27-year-old primipara gravida patient with no particular medical history presented at 23 weeks gestation a sessile mass that had developed on the left anterolateral side of the middle part of the nose of the fetus. It presented a hypoechogenic echostructure with visualization of slow vascular flow. This observation suggested a vascular lesion (Figs. A.1 and 2).
* Corresponding author. E-mail address: [email protected] (R. Lartizien).
The stability of the lesion was confirmed by two ultrasounds at 27 and 32 weeks gestation. A fetal MRI was not requested. The child was born at term (38 GW), by vaginal delivery, with no complications. She presented paramedian swelling of the nasal dorsum that measured 20 mm 20 mm. The mass was firm, noncompressible, bluish, with fine telangiectases on one side. There was no variation in volume when crying (Fig. B.1 and 2). Breathing was not disturbed and the flexible nasoendoscopy ruled out endonasal invasion. An echo-Doppler performed at 8 days of life confirmed the vascularized aspect of the lesion. The clinical examination suggested a congenital hemangioma whose nonregressive aspect argued in favor of a subtype of noninvoluting congenital hemangioma (NICH). Another tumor diagnosis could not be ruled out. An ultrasound as well as an MRI performed at the age of 6 weeks showed a tissue lesion. The MRI was not typical of a hemangioma (absence of hypersignal in T2-weighted images) and ruled out a meningoencephalocele. The diagnosis of nasal glial heterotopia was suggested (Fig. C.1 and 2). Given the unsightly aspect and particularly the risk for amblyopia because of visual field restriction, the tumor was excised at the age of 2.5 months (Figs. D and E). A vertical incision on the left of the lesion was proceed, carrying the skin under the lesion. It was difficult to find a dissection plane between the skin and the lesion. In depth, a cleavage plane was easier between the tumor and the nasal periosteum and the triangular cartilage’s perichondrium. A small mucosal infraction
http://dx.doi.org/10.1016/j.jormas.2017.03.005 C 2017 Elsevier Masson SAS. All rights reserved. 2468-7855/
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
G Model
JORMAS-34; No. of Pages 4 2
R. Lartizien et al. / J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
Figs. A.1 and A.2. Prenatal ultrasound appearance of the nasal mass at 22 week’s gestation in coronal (1) and parasagittal (2) sections.
Figs. B.1 and B.2. Appearance of infant shortly after birth.
Figs. C.1 and C.2. Axial (a) and sagittal (b) T2-W MRI showing a solid mass arising from the left side of the nose with no intracranial extension.
was closed with one point of absorbable suture. The lesion was resected and sent to the pathological analysis. Additional resection of the edge of the skin was performed. The closure was realized in two layers. The final pathological analysis concluded in glial heterotopia with a complete excision in depth but limit at the skin margins.
With eighteen months of follow-up, there were no signs of relapse. 3. Discussion NGH is often labeled CH [2,3]. This can be explained by the clinical variability of the two lesions, their rarity, and the presence
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
G Model
JORMAS-34; No. of Pages 4 R. Lartizien et al. / J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
Fig. D. Surgical resection.
Fig. E. 1 month postoperative view.
of telangiectasias for both of them. MRI can distinguish these two entities more reliably than ultrasound. The clinical presentation of NGH varies. This is a tissue formation, most often polypoid. It is nonpulsatile and does not increase when crying (negative Furstenberg signs), which differentiates it from encephalocele [5]. NGHs have an extranasal presentation in 60% of cases, intranasal in 30% of cases, and 10% are mixed. Telangiectasias are sometimes present and give a pinkish color to the lesion. CHs are vascular tumors. In contrast to infantile hemangiomas, which appear after birth, the CH develops in intrauterine life. The studies conducted by Enjolras [6] classified CHs into two categories: rapidly involuting congenital hemangioma (RICH) and NICH. RICH has a variable clinical aspect. It is a prominent tumor varying from purple to bluish. There can be central necrosis at birth as well as many telangiectasias. This lesion begins to regress in the first weeks of life, over the 1st year, and leaves no more than a scarred area. The NICH is rarer. There is a single round or flat lesion. Very often there is a pale bluish ring in the periphery. The pink or purple color at the surface is related to the presence of telangiectasias. In our patient, the lesion was round and well limited. It was bluish with fine telangiectasias. These characteristics resembled those of a vascular tumor, which participated in the confusion between the two etiologies. The NICH subtype was suggested given the nonregression of the tumor during the first few weeks of life. Clinical monitoring of this type of lesion is important during the first few weeks of life. Indeed, the etiologic diagnosis is not easy
3
and a malignant tumor should never be ruled out. The RICH will regress, the NICH and the NGH will remain stable, whereas a malignant tumor will show rapid growth. The initial diagnosis should be reviewed should any atypical presentation arise. Prenatal monitoring is an important step in the mother’s and fetus’s health. Currently, an ultrasound every trimester is performed. It is possible to detect craniofacial malformations in the second trimester. Most authors recommend a fetal MRI if this type of anomaly is diagnosed [7,8]. The differential diagnosis between NGH and CH is difficult in prenatal ultrasonography. NGH is readily hypoechogenic and clearly limited on the prenatal ultrasound. Doppler analysis can show slow arterial flow or an absence of flow [7–9]. CHs, and particularly RICHs, vary in echogenicity, which is most frequently heterogenous [10,11] but sometimes homogenous [12]. Doppler analysis often shows rapid arterial flow but an absence of flow in certain cases [12]. No prenatal diagnosis of NICH has been reported in the literature. Fetal MRI is not yet specific for NGH but can rule out an intracerebral connection. CHs seem to present a more specific hyperintense aspect on T2weighted sequences (with, however, a hypersignal less pronounced than postnatally) [10]. Only the MRI characteristics of RICH have been described in the literature: a heterogenous lesion with multiple vascular images in the T2-weighted sequence. The intensity of the vascular signals varies. However, this usually hyper-T2 aspect is not always found [10,12]. In our patient, the ultrasound suggested a vascular structure. A fetal MRI would probably have made it possible to specify the diagnosis and perhaps invalidate this hypothesis. The presence of a median line mass should motivate an echoDoppler and an MRI in the early weeks of life. MRI is mandatory to rule out intracerebral extension in the context of encephalocele or the persistence of a fibrous tract in NGH. MRI should be preferred to CT because it does not expose the patient to irradiation and provides clearer results. The lack of ossification in the anterior cranial fossa in newborns can simulate intracerebral communication [11,13]. In cases of CH, the echo-Doppler can identify a mixed tissue and vascular lesion whose echogenicity may be variable depending on the phase of progression of the CH. The lesion is clearly delimited and has high vascular density, comparable to what is observed in arteriovenous malformations. The peak in arterial velocity is high (high flow velocity in the end-diastolic phase) and the peak in venous velocity is generally higher when found in arteriovenous malformations, giving a low-resistance spectrum. Arteriovenous shunts do not exist. The echographic description of NICHs is rarely found, although the aspect is found in CHs. In certain cases, the presence of calcifications has been described [14]. In NGH, the lesion also resembles tissue and is vascularized, but the flow on Doppler is slow (low arterial flow velocity during the end-diastolic phase) [1,3]. The presentation of RICHs and NICHs on MRI is nearly identical: a tumor mass that is relatively homogenous in T1-weighted sequences, enhanced after injection of gadolinium. In T2 the lesion appears as a hypersignal. Vessels are observed as a tubular shadow with no signal in T1 and T2 [6]. For NGHs, the MRI can rule out intracerebral extension. The lesion can also be linked to the subarachnoid space by a fibrous tract that is visible on MRI. This connection is responsible for a risk of an osteomeningeal breach and meningitis. NGH is iso- or hypointense compared to gray matter in T1weighted images. It is enhanced in its center after gadolinium injection. In T2-weighted sequences, it appears slightly hyperintense, less than for CHs [13].
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005
G Model
JORMAS-34; No. of Pages 4 R. Lartizien et al. / J Stomatol Oral Maxillofac Surg xxx (2017) xxx–xxx
4
NGH is classically iso-intense in T1 images compared to the signal of the gray matter, heterogenous in T2 images (with a signal that differs little from the cerebral parenchyma signal) and is slightly enhanced after gadolinium injection. NGH and CHs are benign lesions. Their treatment is therefore not urgent. Respiratory obstruction may be an indication for early surgery [5]. A RICH subtype can rapidly be ruled out based on clinical progression. Distinguishing NGH and NICH can be difficult, but this has no direct incidence on treatment because treatment is surgical in both cases and beta-blockers are not effective on NICHs. It is the risk of amblyopia caused by loss of the visual field that motivated early surgery in our patient. The final diagnosis was made by the pathological analysis. The excision was complete (with doubts about skin margins). Due to the slow progression of NGH and the esthetic concerns in the region, simple clinical monitoring is necessary in cases of incomplete excision. Disclosure of interest The authors declare that they have no competing interest. References
[2] Beegun I, Dua R, Connor S, Bentley R. Prenatal diagnosis and management of a craniofacial glioma detected at 20 weeks’ gestation. Case report and review of the literature. Int J Oral and Maxillofac Surg 2012;41:200–2. [3] Hoeger PH, Schaefer H, Ussmueller J, Helmke K. Nasal glioma presenting as capillary hemangioma. Eur J Pediatr 2001;160:84–7. [4] Oddone M, Granata C, Dalmonte P, Biscaldi E, Rossi U, Toma P. Nasal glioma in an infant. Pediatr Radiol 2002;32:104–5. [5] Yapo PC, Diabate AS, Konan E, Dede NS, Kouadio E, Gui LN, et al. Gliome nasopalatin avec obstruction nasale gauche conge´nitale: a` propos d’un cas. J Radiol 2010;91:1325–8. [6] Enjolras O. He´mangiomes conge´nitaux. Ann Dermatol Venereol 2003;130: 367–71. [7] De Biasio P, Sarso E, PREFUMO F, Odella C, Rossi A, Venturini PL. Prenatal diagnosis of a nasal glioma in the mid trimester. Ultrasound Obstet Gynecol 2006;27:571–3. [8] Grzegorezyk V, Brasseur-Daudruy M, Labadie G, Cellier C, Verspyck E. Prenatal diagnosis of nasal glioma. Pediatr Radiol 2010;40:1706–9. [9] Chmait RH, Pretorius DH, Hull AD. Nasal glioma. Ultrasound Obste Gynecol 2002. [10] Elia D, Garel C, Enjolras O, Vermouneix L, Soupre V, Oury JF, et al. Prenatal imaging findings in rapidly involuting congenital hemangioma of the skull. Ultrasound Obstet Gynecol 2008;31:572–5. [11] Uehara S, Hasegawa T, Okuyama H, Kawahara H, Kubota A, Osuga K, et al. Prenatally detected giant congenital hemangioma of the fetal neck. J Ped Surg Case Rep 2013;1:29–31. [12] Brix M, Soupre V, Enjolras O, Vazquez MP. Diagnostic ante´natal des RICH. Rev Stomatol Chir Maxillofac 2007;108:109–14. [13] Hedlung G. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol 2006;36:647–62. [14] Masand P. Radiographic findings associated with vascular anomalies. Semin Plast Surg 2014;28:69–78.
[1] Dasgupta NR, Bentz ML. Nasal gliomas: identification and differentiation from hemangiomas. J Craniofac Surg 2003;14(5):736–8.
Please cite this article in press as: Lartizien R, et al. Nasal glial heterotopia or congenital hemangioma? A case report. J Stomatol Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.jormas.2017.03.005