Patient population
A total of 185 patients with a vascular anomaly, of which 85 women (46%) and 100 men (54%), consulted the OMFS department of the university hospitals of Leuven in a 16-year period. Ages ranged from 3 months to 94 years and the mean age was 44.2 (SD 23.8) years.
Diagnostics
Prior to the first contact, 65 patients (35,1%) had consulted a specialist physician and 41 patients (22,2%) had received therapy before. Twenty patients (10.8%) who visited the general consultation, were referred to the multidisciplinary consultation for vascular anomalies.
The initial reason for consultation in three quarters of the patient population (n = 143; 77.3%) was diagnosis and treatment. In 14 patients (7.6%), the vascular anomaly was an incidental finding and 18 patients (9.7%) asked for a second opinion or advise. Six patients (3.2%) asked a reconstructive treatment for a defect caused by a vascular anomaly (e.g. resection of fibrofatty tissue).
The most frequently used diagnostic techniques were histopathological examination (44,9%), MRI (29,7%), CT (11,4%) and ultrasound (9,2%). In a quarter of the patients, no further investigations were conducted. The list of all technical investigations can be found in additional file 2.
Lesion characteristics
Twenty-three lesions (12.4%) were present at birth and 123 lesions (66.5%) developed later in life at a mean age of 41.8 years (SD 22,9 years). The time of emergence was not recorded for 39 lesions (21.1%).
Venous malformations were most frequently diagnosed (47 patients, 25.4%), followed by lobular capillary hemangiomas (17 patients, 9.2%), arteriovenous malformations (13 patients, 7.0%) and combined malformations (10 patients, 5.4%) (Table 2).
Table 2
Frequency of the different vascular anomalies in the study population
Vascular tumors | Vascular malformations | Not specified |
| N | %* | Low flow | N | %* | High flow | N | %* | | N | %* |
LCH | 17 | 9.2 | VM | 47 | 25.4 | AVM | 13 | 7.0 | Vascular anomaly NOS | 39 | 21.1 |
IH | 1 | 0.5 | LM | 3 | 1.6 | Combined | 3 | 1.6 | | | |
CH | 1 | 0.5 | CM | 3 | 1.6 | | | | |
KHE | 1 | 0.5 | Combined | 7 | 3.8 | | | |
IPEH | 1 | 0.5 | Sturge Weber | 2 | 1.1 | | | | |
| Vascular malformation NOS | 47 | 25.4 | | | |
Total | 19 | 10.3 | Total | 125 | 67.6 | Total of all anomalies | 185 | 100 |
*percentage of the total number of anomalies (N = 185); LCH: lobular capillary hemangioma; IH: infantile hemangioma; CH: congenital hemangioma; KHE: kaposiform hemangioendothelioma; IPEH: intravascular papillary endothelial hyperplasia; NOS: not otherwise specified; VM: venous malformation; AVM: arterio-venous malformation; LM: lymphatic malformation; CM: capillary malformation |
Three AVMs were in Schobinger stage I, seven in stage II and three in stage III (none in stage IV). Forty-seven vascular malformations (25.4%) were not further subclassified and 39 vascular anomalies (21,1%) could not be specified according to the ISSVA-classification based on the clinical records. Two patients had Sturge Weber syndrome and one patient had a Kaposiform hemangioendothelioma, which is a locally aggressive tumor. Another patient had a Masson’s tumor, which is a benign tumor.
Most lesions were unifocal (n = 146; 78.9%) and half of the anomalies had diffused borders (n = 97; 52.4%). For 35 lesions (18.9%) there was no information on the delineation.
The lips were most often affected (n = 72; 38.9%), followed by the tongue (n = 46; 24.9%), buccal space (n = 36; 19.5%), sublingual space (n = 13; 7.0%), masticator space and gums (both 12 lesions; 6.5%). The complete list of affected anatomical regions can be found in additional file 3.
Most frequently mentioned symptoms were discomfort of the voluminous lesion (n = 34; 18.4%), unpleasing aesthetics (n = 25; 13.5%), pain (n = 24; 13.0%) and bleeding (n = 21; 11.4%). A third of the patients were asymptomatic (n = 66, 35.7%). All symptoms are listed in additional file 4.
Treatment and outcome
A total of 101 patients (54.6%) received active treatment and 12 of them (6.5%) received multiple therapies. Vascular anomalies were surgically removed with devascularization techniques during 90 surgical procedures in 83 patients (44.9%) and with hemodynamic techniques during 16 procedures in 10 patients (5.4%). Devascularization techniques resulted in a complete symptom relief in 84.4% of the procedures, while hemodynamic techniques achieved in 43.8% complete symptom relief (p = 0.0003). A minority was treated with laser therapy (n = 6, 3.2%), sclerotherapy (n = 5, 2.7%), embolization (n = 3, 1.6%) or sirolimus (n = 1, 0.5%). Thirteen patients (7.0%) got informed about the treatment options but didn’t wish any therapy. A quarter of the patients (n = 50, 27.0%) were treated conservatively. Thirteen patients (7.0%) were referred to other departments for treatment and seven patients (3.8%) were lost for follow-up before starting treatment. All therapies and outcomes are listed in additional file 5.
The treatment or treatment planning was done through multi-disciplinary collaboration in 57 cases (30.8%). Interventional radiology was most often consulted (41 cases, 22.2%) to evaluate the feasibility of embolization or sclerotherapy. Other involved disciplines are listed in additional file 6.
Complications were reported in 18 patients (17.8%) of the 101 patients who received active treatment (Table 3). The most common complications had a Clavien-Dindo grade I or II: impaired wound healing (n = 5, gr. I), wound infection (n = 4, gr. I), temporary local hypoesthesia (n = 7, gr. II) and permanent local hypoesthesia (n = 3, gr. II). During two surgeries the vascular anomaly bled excessively which necessitated admission at the intensive care unit (grade IV).
Table 3
Complications | I | II | IIIa | IIIb | IVa | IVB | V | N (%*) |
Bacterial / fungal infection | 7 | - | - | - | - | - | - | 7 (6.9%) |
Temporary hypoesthesia | - | 4 | - | - | - | - | - | 4 (4.0%) |
Permanent hypoesthesia | - | 3 | - | - | - | - | - | 3 (3.0%) |
Impaired healing | 5 | - | - | - | - | - | - | 5 (5.0%) |
Prolonged reduced mouth opening | 2 | - | - | - | - | - | - | 2 (2.0%) |
Keloid scarring | 2 | - | - | - | - | - | - | 2 (2.0%) |
Excessive intraoperative bleeding | - | - | - | - | 2 | - | - | 2 (2.0%) |
Persistent pain (not due to infection) | 1 | - | - | - | - | -- | - | 1 (1.0%) |
Facial nerve dysfunction | - | 1 | - | - | - | - | - | 1 (1.0%) |
Diarrhea in association with Sirolimus | - | - | - | - | - | - | - | 1 (1.0%) |
Sialocele | - | 1 | - | - | - | - | - | 1 (1.0%) |
Dysphagia | 1 | - | - | - | - | - | - | 1 (1.0%) |
*Percentage of patients receiving active treatment (n = 101) |
Incidence of all complications after active treatment (surgery, sclerotherapy, embolization, laser therapy and sirolimus), according to Clavien-Dindo classification.
In nine of the actively treated patients (8.9%), the vascular anomaly recurred: four with an AVM, three with a venous malformation (VM) and two with a lobular capillary hemangioma. Three patients had multiple relapses, ranging between two and four. Recurrence occurred after embolization of AVMs in three patients and after surgical therapy in the six remaining patients. All recurrences were treated surgically, with or without preoperative embolization. Another twenty-two patients (11.9%) reported a recurrent anomaly at the first consultation. All these lesions were treated again in UZ Leuven with no new recurrences.