| Abstract|| |
Rationale: Vascular malformation (VM) associated with jaws may cause jaw size discrepancy. Multiple phleboliths in VM are relatively rare. This case report aims to present one such case. Patient Concerns: A 33-year-old female patient sought surgical correction of her abnormally sized jaw and on examination, she was identified with VM. Diagnosis: Subsequent imaging tests revealed the presence of several phleboliths. Treatment: The patient was treated for an abnormal-sized mandible as well as partial removal of the superficial part of VM. Outcomes: The patient had satisfactory esthetics and there was less bleeding than anticipated. Take-away Lessons: VMs could cause jaw size discrepancy and the extent of the malformation could cause blood flow abnormalities leading to multiple phlebolith formation. Proper surgical planning and education of the patient are essential for successful treatment.
Keywords: Lip surgery, orthognathic surgery, phlebolith, vascular malformation
|How to cite this article:|
Balaji S M, Balaji P. Unusual Number of Phleboliths in a Lip Vascular Malformation – A Case Report. Indian J Dent Res 2022;33:110-2
|How to cite this URL:|
Balaji S M, Balaji P. Unusual Number of Phleboliths in a Lip Vascular Malformation – A Case Report. Indian J Dent Res [serial online] 2022 [cited 2022 Oct 4];33:110-2. Available from: https://www.ijdr.in/text.asp?2022/33/1/110/353534
| Background|| |
Vascular malformations (VMs) are benign lesions usually from birth and extend during growth. There are various classification, the simplest being the International Society for Study of Vascular Malformations (ISSVA) classification (2018) for vascular anomalies – as simple (capillary, lymphatic, venous, and arteriovenous), combined, of major named vessels, and associated with other anomalies. Although common soft tissue tumor in the head and neck, they are rare intra-orally. When occurring, they may cause significant osseous overgrowth, sometime necessitating orthognathic surgery.
There are very infrequent reports of VMs and phleboliths being observed together. When occurring, they are often small, solitary calcifications, and/or not in direct contact with the VM. The mechanism of formation of phlebolith in VM could be the changes in blood flow causing a thrombus that act as a nidus, trauma, and dystrophic calcifications. Often such lesions are painless, clinically palpable, and often visualized in imaging modalities. Depending on the site, size, and numbers, the phlebolith could cause esthetical and functional compromise. Surgical removal is only treatment and when asymptomatic, they are followed up.,,, The aim is to present a rare case of numerous phleboliths in a VM case.
| Case Report|| |
A 33-year old female presented for rehabilitation of her facial appearance for the protruding jaws and if possible the lower lip and parts of adjoining buccal mucosa bilaterally. History revealed that she had been diagnosed with VM since childhood and had not undergone any specific treatment for the same because of the risk of bleeding. Considering the diffuse nature of the VM in lip-cheek, with a high risk of iatrogenic nerve injury and bleeding, patient had been advised sclerotherapy in the past but was never done [Figure 1]. On examination, patient had a class III malocclusion, open-bite, macrochelia with crops of bright red, vesicles- strawberry-like clumps involving the lower lip and adjoining buccal mucosa. The lesion was diascopy positive. She was denied treatment because of the VM. Radiographic examination revealed a typical class III malocclusion and numerous phleboliths [Figure 2]a, [Figure 2]b, [Figure 2]c. Because of poor oral hygiene, she had lost many posterior teeth. Panoramic radiography showed radiolucencies at the mandible from the angulus to the corpus. Many phleboliths were also seen in this region. Contrast enhanced MR angiography demonstrated vascular supply to the lesion as well as lesion matrix [Figure 3]a, [Figure 3]b, [Figure 3]c. Treatment to the VM was offered and patient denied consent for any large-scale intervention for the VM. Histopathological examination was not carried because of the absence of consent, however, aspiration helped to narrow the clinical diagnosis.
|Figure 2: (a and b) – Preoperative Orthopantomogram and Lateral Cephalogram revealed a typical class III malocclusion and numerous phleboliths. (c) – Intraoral examination revealed Class III Malocclusion, open bite, macrochelia with crops of bright red, vesicles-strawberry-like clumps involving lower lip and buccal mucosa|
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|Figure 3: Contrast-enhanced MR angiography demonstrates vascular supply to the lesion as well as lesion matrix|
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Standard sclerotherapy was organized to shrink the superficial elements. Standard orthognathic surgery to reduce the Class III malocclusion was carried out along with debulking of superficial lesion. Patient is being followed up for 60 months with no obvious increase in the size of the VM or relapse of the class III malocclusion [Figure 4].
|Figure 4: (a). Orthopantomogram X-ray showing orthognathic surgery was performed. (b). Postoperative photograph|
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| Discussion|| |
VMs may directly or indirectly influence the growth of the adjoining craniofacial bones leading to a host of occlusal issues. Open bite deformities and other orthognathic abnormalities may precipitate difficulties with speech, feeding, mouth breathing, social presentation, and most importantly poor oral hygiene. In this case too, patient had been suffering largely because of the VM and its squeale. However, patient was not willing to get the VM completely treated owing to inherent risk involved. Such issues would be needed to be addressed with the consent of the patient. The malocclusion can be directly attributed to the presence of VM by direct or indirect extension. In this case too, the class III malocclusion could have developed from the effects of the VM. There are sufficient surgical literature that has reported treatment in young age.
Multiple palpable phleboliths in a VM of the head and neck are very rare and the treatment for the same is not yet been discussed.,,, There are reports of VM patients with individual palpable phleboliths of the maxillofacial region that has been managed with surgery or multiple small phleboliths with sclerotherapy. There was one recent report wherein a surgical intervention was indicated for the deformity because of seven phleboliths. In that case, the phleboliths were in close proximity, whereas in this case, the spread of the phleboliths was a huge challenge in surgical treatment. As the patient was not consenting, treatment was not possible. Literature suggests that localized phlebolith has to be excised and there are reports of large ones from cheek being removed.,,,, However, in this case, the distribution is wide and there were numerous structures. The pathogenesis of phleboliths in VM is widely believed to be from thrombi formed from slowing of peripheral blood flow that becomes organized and later calcified. The size of the most of the phlebolith is between 1 and 5 mm in diameter, but rarely exceed 1 cm and may be big as 60 mm. Phlebolith exceeding 20 mm are very rare with a very handful case being reported. This case has numerous phlebolith with many of them exceeding 20 mm.,,
In this case, sclerotherapy was provided to facilitate debulking of the lesion and successful orthognathic surgery was successfully carried out. As the correct treatment algorithm for the complete removal of the phlebolith is not available in literature, the need for one arises such that similar cases in future could be managed effectively. The case is novel in having several phlebolith with several of them exceeding 20 mm in diameter and there is no proper direction in removal of the phlebolith, especially when they are widely placed apart in the lower labial and buccal mucosa.
In rare instance, phleboliths can occur without VMs too. Hence, a careful clinical and radiological examination is needed, especially, if VMs are deeply seated inside muscle or adjacent structures.
| Conclusion|| |
A rare case of numerous phleboliths-associated VM is presented. The orthognathic management in such situation has been discussed. The need for careful evaluation has been stressed.
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.
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Conflicts of interest
There are no conflicts of interest.
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Dr. S M Balaji
Director and Consultant Oral and Maxillofacial Surgeon, Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai - 600 018, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]