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Congenital glabellar hemangioma
CLINICAL PHOTOGRAPH Congenital glabellar hemangioma SIU-KWAN NG,
FRCS,
GORDON SOO,
FRCS,
VICTOR ABDULLAH,
CASE REPORT A 2-month-old boy was referred to our unit because of a congenital glabellar swelling. The swelling was firm, nonpulsatile, and noncompressible with small telangiectasia on the overlying skin. There was no clinical evidence of an intranasal mass or of cerebrospinal fluid (CSF) leakage. Computed tomography (CT) scanning and magnetic resonance imaging (MRI) were performed and excluded intracranial extension. The swelling was nonenhancing and isodense with brain tissue on MRI. The clinical and imaging findings were consistent with a diagnosis of a nasal glioma. The lesion was excised via an external approach. The final histopathologic diagnosis was capillary hemangioma (Fig 1). DISCUSSION Congenital nasal masses are rare. The glabellar region is one of the most common sites for congenital lesions. The important differential diagnoses include nasal dermoid, hemangioma, nasal glioma, and meningoencephalocele.1 Nasal dermoids are formed as a result of sequestration of epithelial elements during the fusion of the median nasal processes. They are usually adherent to the skin, and a concomitant dermal dimple or sinus may be present. Sometimes a strand of hair protrudes from the sinus opening.2 A hemangioma is regarded as a vascular harmatoma rather than a true neoplasm. Discoloration From the Division of Otorhinolaryngology, Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong. Reprint requests: C. A. van Hasselt, MMED, Division of Otorhinolaryngology, Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, N.T., Hong Kong. Otolaryngol Head Neck Surg 2003;129:161-2. Copyright © 2003 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. 0194-5998/2003/$30.00 ⫹ 0 doi:10.1016/S0194-5998(02)00037-2
FRCS,
and C. A. VAN HASSELT,
MMED,
Shatin, N.T., Hong Kong
of the overlying skin and telangiectasia are often present. Despite the initial unsightly appearance, almost all of these lesions regress spontaneously.2 Conservative management and parental reassurance generally suffice. Nasal gliomas and meningoencephaloceles share a common developmental origin. As a consequence of the faulty closure of the anterior neuropore, there is residual herniation of brain tissue. Meningoencephaloceles usually retain their communication with the CSF-filled subarachnoid space, whereas gliomas become detached from the intracranial cavity.2 As a result, nasal meningoencephaloceles are usually compressible, soft, and cystic. They may increase in size on gentle compression of the internal jugular veins (Furstenberg test). This increase in size is also notable when the child cries or strains. Nasal gliomas, in contrast, are firm in consistency and do not fluctuate in size as do meningoencephaloceles with CSF pressure changes. The overlying skin may be normal or faintly red or blue. Interestingly, areas of telangiectasia may be present.1 Fine needle aspiration or incisional biopsy of glabellar lesions is strongly contraindicated before imaging because either procedure may yield inconclusive results3 and may cause CSF leakage and meningitis if an intracranial connection is present. MRI and/or CT scanning should be performed to delineate the exact extent of the lesion and the presence of any intracranial communication. CT scanning demonstrates clearly any bony defects, whereas MRI offers excellent soft tissue contrast without the use of ionizing radiation. CT and MRI are complementary investigations in congenital glabellar lesions. MRI is particularly useful in distinguishing between brain parenchyma and inflamed nasal mucosa. With the exception of hemangiomas, these lesions require excision; if left, they may lead to cosmetic or infectious complications because of intracranial connections. Particularly in cases of meningoencephalocele, early excision is impera161
Otolaryngology– Head and Neck Surgery July 2003
162 NG et al
Fig 1. Congenital glabellar hemangioma.
tive to promote normal facial growth.1 Nasal dermoids may also become complicated with recurrent local infection. The surgical approach depends on the extent of the lesion. Masses with an intracranial extension should be approached via a craniotomy or a combined intracranial and extracranial approach. Most nasal lesions can be explored via an external approach. A vertical incision provides good access for midline lesions, whereas a lateral rhinotomy approach is recommended for a laterally placed or intranasal mass. Cervicofacial vascular lesions are now conveniently classified into hemangiomas and vascular malformations. A hemangioma typically presents during infancy and undergoes a stage of rapid growth that is followed by regression. A vascular malformation is present at birth, grows with the child, and does not regress. A facial hemangioma does not usually require surgical intervention. Surgical excision may be indicated when the size or position of a hemangioma causes visual axis obstruction or distortion of the cornea, which may result in amblyopia.4 Hemangiomas, which cause life-threatening complications such as high output congestive
heart failure and platelet-trapping coagulopathy (Kasabach-Merritt syndrome), also require early active treatment. Corticosteroids remain the main pharmacologic treatment with interferon-␣2a reserved for unresponsive case and patients with Kasabach-Merritt syndrome. Subglottic hemangiomas that cause airway obstruction also require urgent aggressive treatment.4 In the reported case, the external appearance and isodensity of the lesion with the brain on MRI favored the choice of early excision. This is an exception rather than the rule in the treatment of a hemangioma. REFERENCES
1. Haafiz AB, Sharma R, Faillace WJ. Congenital midline nasofrontal mass. Two case reports with a clinical review. Clin Pediatr (Phila) 1995;34:482-6. 2. Cinnamond MJ. Congenital anomalies of the nose. In: Adams DA, Cinnamond MJ, editors. Scott-Brown’s Otolaryngology: Vol 6. 6th ed. London: Butterworth-Heinemann; 1997. p. 15-18. 3. Levine MR, Kellis A, Lash R. Nasal glioma masquerading as a capillary hemangioma. Ophthal Plast Reconstr Surg 1993;9:132-4. 4. McGill TJI, Forsen JW Jr, Mulliken JB. Hemangiomas and vascular anomalies of the head and neck. In: Richardson MA, editor. Pediatric otolaryngology head and neck surgery, 3rd ed. St Louis: Mosby; 1998. p. 66-80.
FRCS,
GORDON SOO,
FRCS,
VICTOR ABDULLAH,
CASE REPORT A 2-month-old boy was referred to our unit because of a congenital glabellar swelling. The swelling was firm, nonpulsatile, and noncompressible with small telangiectasia on the overlying skin. There was no clinical evidence of an intranasal mass or of cerebrospinal fluid (CSF) leakage. Computed tomography (CT) scanning and magnetic resonance imaging (MRI) were performed and excluded intracranial extension. The swelling was nonenhancing and isodense with brain tissue on MRI. The clinical and imaging findings were consistent with a diagnosis of a nasal glioma. The lesion was excised via an external approach. The final histopathologic diagnosis was capillary hemangioma (Fig 1). DISCUSSION Congenital nasal masses are rare. The glabellar region is one of the most common sites for congenital lesions. The important differential diagnoses include nasal dermoid, hemangioma, nasal glioma, and meningoencephalocele.1 Nasal dermoids are formed as a result of sequestration of epithelial elements during the fusion of the median nasal processes. They are usually adherent to the skin, and a concomitant dermal dimple or sinus may be present. Sometimes a strand of hair protrudes from the sinus opening.2 A hemangioma is regarded as a vascular harmatoma rather than a true neoplasm. Discoloration From the Division of Otorhinolaryngology, Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong. Reprint requests: C. A. van Hasselt, MMED, Division of Otorhinolaryngology, Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, N.T., Hong Kong. Otolaryngol Head Neck Surg 2003;129:161-2. Copyright © 2003 by the American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. 0194-5998/2003/$30.00 ⫹ 0 doi:10.1016/S0194-5998(02)00037-2
FRCS,
and C. A. VAN HASSELT,
MMED,
Shatin, N.T., Hong Kong
of the overlying skin and telangiectasia are often present. Despite the initial unsightly appearance, almost all of these lesions regress spontaneously.2 Conservative management and parental reassurance generally suffice. Nasal gliomas and meningoencephaloceles share a common developmental origin. As a consequence of the faulty closure of the anterior neuropore, there is residual herniation of brain tissue. Meningoencephaloceles usually retain their communication with the CSF-filled subarachnoid space, whereas gliomas become detached from the intracranial cavity.2 As a result, nasal meningoencephaloceles are usually compressible, soft, and cystic. They may increase in size on gentle compression of the internal jugular veins (Furstenberg test). This increase in size is also notable when the child cries or strains. Nasal gliomas, in contrast, are firm in consistency and do not fluctuate in size as do meningoencephaloceles with CSF pressure changes. The overlying skin may be normal or faintly red or blue. Interestingly, areas of telangiectasia may be present.1 Fine needle aspiration or incisional biopsy of glabellar lesions is strongly contraindicated before imaging because either procedure may yield inconclusive results3 and may cause CSF leakage and meningitis if an intracranial connection is present. MRI and/or CT scanning should be performed to delineate the exact extent of the lesion and the presence of any intracranial communication. CT scanning demonstrates clearly any bony defects, whereas MRI offers excellent soft tissue contrast without the use of ionizing radiation. CT and MRI are complementary investigations in congenital glabellar lesions. MRI is particularly useful in distinguishing between brain parenchyma and inflamed nasal mucosa. With the exception of hemangiomas, these lesions require excision; if left, they may lead to cosmetic or infectious complications because of intracranial connections. Particularly in cases of meningoencephalocele, early excision is impera161
Otolaryngology– Head and Neck Surgery July 2003
162 NG et al
Fig 1. Congenital glabellar hemangioma.
tive to promote normal facial growth.1 Nasal dermoids may also become complicated with recurrent local infection. The surgical approach depends on the extent of the lesion. Masses with an intracranial extension should be approached via a craniotomy or a combined intracranial and extracranial approach. Most nasal lesions can be explored via an external approach. A vertical incision provides good access for midline lesions, whereas a lateral rhinotomy approach is recommended for a laterally placed or intranasal mass. Cervicofacial vascular lesions are now conveniently classified into hemangiomas and vascular malformations. A hemangioma typically presents during infancy and undergoes a stage of rapid growth that is followed by regression. A vascular malformation is present at birth, grows with the child, and does not regress. A facial hemangioma does not usually require surgical intervention. Surgical excision may be indicated when the size or position of a hemangioma causes visual axis obstruction or distortion of the cornea, which may result in amblyopia.4 Hemangiomas, which cause life-threatening complications such as high output congestive
heart failure and platelet-trapping coagulopathy (Kasabach-Merritt syndrome), also require early active treatment. Corticosteroids remain the main pharmacologic treatment with interferon-␣2a reserved for unresponsive case and patients with Kasabach-Merritt syndrome. Subglottic hemangiomas that cause airway obstruction also require urgent aggressive treatment.4 In the reported case, the external appearance and isodensity of the lesion with the brain on MRI favored the choice of early excision. This is an exception rather than the rule in the treatment of a hemangioma. REFERENCES
1. Haafiz AB, Sharma R, Faillace WJ. Congenital midline nasofrontal mass. Two case reports with a clinical review. Clin Pediatr (Phila) 1995;34:482-6. 2. Cinnamond MJ. Congenital anomalies of the nose. In: Adams DA, Cinnamond MJ, editors. Scott-Brown’s Otolaryngology: Vol 6. 6th ed. London: Butterworth-Heinemann; 1997. p. 15-18. 3. Levine MR, Kellis A, Lash R. Nasal glioma masquerading as a capillary hemangioma. Ophthal Plast Reconstr Surg 1993;9:132-4. 4. McGill TJI, Forsen JW Jr, Mulliken JB. Hemangiomas and vascular anomalies of the head and neck. In: Richardson MA, editor. Pediatric otolaryngology head and neck surgery, 3rd ed. St Louis: Mosby; 1998. p. 66-80.