Beard hemangioma associated with cleft lip and palate: An unusual case and literature review

Nicolás E Solano; Ejusmar K Rivera; Salomon Ramos; Maholy Sánchez

doi:10.4103/jclpca.jclpca_27_18

CASE REPORT

Year : 2019 | Volume : 6 | Issue : 1 | Page : 59-61

Beard hemangioma associated with cleft lip and palate: An unusual case and literature review

Nicolás E Solano¹, Ejusmar K Rivera¹, Salomon Ramos¹, Maholy Sánchez²
¹ Dentistry Department, Oral and Maxillofacial Surgery Unit, University Hospital of Maracaibo; Oral Surgery Post-graduate Program, School of Dentistry, University of Zulia, Maracaibo, Venezuela
² Dentistry Department, Oral and Maxillofacial Surgery Unit, University Hospital of Maracaibo, Maracaibo, Venezuela

Date of Web Publication

4-Feb-2019

Correspondence Address:
Dr. Ejusmar K Rivera
Circunvalacion 1, Parque Las Colinas, Maracaibo, Edo Zulia
Venezuela

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jclpca.jclpca_27_18

Abstract

Infantile hemangiomas (IHs) are the most common benign vascular tumor of infancy. Sixty percent of lesions affect the head and neck. There are special considerations in IHs, like beard-distribution hemangiomas, in which preauricular areas, chin, anterior neck, and lower lip are involved and they have been associated with the presence of airway hemangiomas. In contrast, labial and palatal clefts are the most common congenital malformations of the craniofacial region. The etiology is multifactorial and includes both genetic and environmental factors. It is associated with more than 500 syndromes with varying levels of penetrance and expressiveness. IHs and cleft lip and palate (CLP) are relatively common conditions found in newborns. Even though they are common enough, there is no evidence reporting a patient with both entities simultaneously. The purpose of this study is to present an unusual case of beard hemangioma in a 3-month-old female baby associated with CLP.

Keywords: Beard hemangioma, cleft lip, cleft palate

How to cite this article:
Solano NE, Rivera EK, Ramos S, Sánchez M. Beard hemangioma associated with cleft lip and palate: An unusual case and literature review. J Cleft Lip Palate Craniofac Anomal 2019;6:59-61

How to cite this URL:
Solano NE, Rivera EK, Ramos S, Sánchez M. Beard hemangioma associated with cleft lip and palate: An unusual case and literature review. J Cleft Lip Palate Craniofac Anomal [serial online] 2019 [cited 2019 Aug 26];6:59-61. Available from: http://www.jclpca.org/text.asp?2019/6/1/59/251467

Introduction

Infantile hemangiomas (IHs) are the most common benign vascular tumor of infancy.^[1] There are special considerations in IHs, like the beard-distribution hemangiomas, in which preauricular areas, chin, neck, and lower lip are involved.^[2] In contrast, cleft lip and palate (CLP) is the most common congenital malformation of the craniofacial region.^[3] Although estimates vary, approximately 5% of infants develop a hemangioma during the 1^st year of life.^[1] In contrast, CLP is present in about 1 in 700 of newborns.^[3] Even though they are common enough, there is no evidence reporting a patient with both entities simultaneously.

Case Report

A 3-month-old female baby was presented by the mother to the Craniofacial Anomalies and Cleft Unit of University Hospital of Maracaibo, Venezuela, in June 2018, with a complaint of CLP and multiple lesions in the orofacial region since birth. The baby was the parent's second child without a history of consanguineous marriage. The patient presented with right complete unilateral cleft lip and Type III cleft palate. Likewise, red plaque-like lesions with moderate elevation were observed in the right temple, right ear lobe and preauricular regions, lower lip, chin, neck, and chest [Figure 1] and [Figure 2]. These clinical findings and the clinical history lead to the diagnosis of IH in a beard distribution associated with CLP, and thus, magnetic resonance imaging, ultrasound, or biopsy was not performed. An evaluation performed by the otolaryngologist rule out the presence of lesions in the airway. Genetic evaluation did not discern an underlying syndrome, and the vascular anomalies team evaluated the patient and found no additional findings of PHACE syndrome (posterior fossa anomalies, haemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, sternal clefting and supraumbilical raph).

Figure 1: Clinical images of the patient on admission. Frontal view

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Figure 2: Clinical images of the patient on admission. Lateral view

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This study was approved by the University Hospital of Maracaibo International Review Board, and all participants signed informed consent agreement.

Discussion

IHs are proliferative vascular tumors consisting of endothelial cells which can occur anywhere in the body. In most cases, IHs are not present at birth, but then develop in the 1^st month of life. The IH first enters a proliferation phase and rapidly grows for 3–5 months. This is followed by a prolonged involution phase with spontaneous regression over the course of years.^[4]

IHs are classified into three distinct morphologic patterns that include solitary, segmental/regional/diffuse, or multifocal lesions. These patterns are significant as they can be a clue to the possibility of other underlying syndromes. IHs that cover a diffuse area have a higher risk of complications, such as ulceration, and can be associated with syndromes, such as PHACE and Lower body hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies (LUMBAR).^[1],[2]

IHs are the most common head and neck tumor in children, but a segmental IH in a beard distribution is both rare and life-threatening, due to the possible airway involvement.^[4] Beard hemangiomas involve the V3 distribution of the trigeminal nerve and extend from the preauricular region to the lower lip. Although patients may also have bilateral hemangiomas of the parotid gland, the most important clinical association is with lesions that affect the airway, typically the subglottis.^[5] It was demonstrated that airway involvement in the oral cavity, oral pharynx, hypopharynx, glottis, or subglottis was seen in 29% of the children with beard-distribution hemangioma.^[6],[7] Airway IHs are usually associated with segmental hemangiomas in a bearded distribution, which may include all or some of the following: the preauricular skin, mandible, lower lip, chin, or anterior neck, although they can be found without skin lesions. It is important for an otolaryngologist to proactively assess lesions in this distribution before signs of stridor occur.^[2],[4]

The treatment of IHs is always individualized to the specific lesion as well as to the patient. Observation is frequently selected in the majority of IHs especially if the hemangioma is at the beginning of the proliferation phase. Early intervention should be pursued in some hemangiomas, depending on growth rate, extent, location, and complications.^[1]

Corticosteroids have been considered the standard medical treatment through the years. However, a nonselective β-blocker, propranolol, has become a first-line treatment for many hemangiomas, it has been suggested that this medication may gain its efficacy through mechanisms of vasoconstriction or apoptosis, and it is usually given for the entire proliferative phase, for a year or longer. A thorough initial workup along with the involvement of a pediatric cardiologist is strongly recommended. Other pharmacologic therapies are available for resistant or complicated lesions, but such cases are rare.^[1],[4],[8]

In our case, after a discussion with the cardiovascular surgery service, we decided not to apply any treatment on the hemangioma, given that there is a lack of functional alterations and the tendency of these lesions to involute. Applying propranolol could be considered in case the lesions present a more aggressive behavior or if they do not decrease in size in the expected period.

For the management of the CLP, we started the protocol used in our service, wich consists of an initial orthopedic phase, with the use of nasoalveolar molding and orthosis splints. Subsequently, once the patient is at least 3 months of age, a primary surgical phase will be initiated, where a primary cheilorhinoplasty using a modified Millard Technique will be performed, along with two-plane local flaps to cover the nasoalveolar cleft and a vomer flap to cover the nasal floor of the primary palate. Later on, when the patient is at least 9 months old, a secondary surgical phase will be performed, consisting of a primary palatoplasty and velopharyngeal reconstruction.

Given that the hemangiomas are not involved in the clefts, the postoperative healing should not be compromised by these lesions.

A beard distribution IH with concurrent CLP has not been reported. In 1998, Mulliken reported a newborn with a congenital ulceration of the upper lip, who was diagnosed with a cleft lip and developed an obvious hemangioma after 1 month of age. Similarly, Berenguer et al. described a case of a rapidly involuting congenital hemangioma of the upper lip, which produced a morphologic sequel that resembled a microform cleft lip.^[9],[10]

To the authors' knowledge, there is no evidence in the literature that reports both pathologies simultaneously, nor any syndromes that may associate these entities, as presented in our case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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3.	Díaz Casado GH, Díaz Grávalos GJ. Orofacial closure defects: Cleft lip and palate. A literature review. Semergen 2013;39:267-71.
4.	Adams DM, Ricci KW. Infantile hemangiomas in the head and neck region. Otolaryngol Clin North Am 2018;51:77-87.
5.	Fowell C, Monaghan A, Nishikawa H. Infantile haemangiomas of the head and neck: Current concepts in management. Br J Oral Maxillofac Surg 2016;54:488-95.
6.	McCormick AA, Tarchichi T, Azbell C, Grunwaldt L, Jabbour N. Subglottic hemangioma: Understanding the association with facial segmental hemangioma in a beard distribution. Int J Pediatr Otorhinolaryngol 2018;113:34-7.
7.	Piram M, Hadj-Rabia S, Boccara O, Couloigner V, Hamel-Teillac D, Bodemer C, et al. Beard infantile hemangioma and subglottic involvement: Are median pattern and telangiectatic aspect the clue? J Eur Acad Dermatol Venereol 2016;30:2056-9.
8.	Baselga Torres E, Bernabéu Wittel J, van Esso Arbolave DL, Febrer Bosch MI, Carrasco Sanz Á, de Lucas Laguna R, et al. Spanish consensus on infantile haemangioma. An Pediatr (Barc) 2016;85:256-65.
9.	Neri I, Piccolo V, Russo T, Bianchi A, Patrizi A. Congenital primarily ulcerated hemangioma mimicking cleft lip. J Pediatr 2013;162:882.e1.
10.	Berenguer B, González M, Marín, Tomás Palacios E. Rapidly involuting congenital hemangioma (RICH) that simulates a minimal cleft lip. Cir Pediatr 2010;23:130-3.