| Objective and significance:Infantile hemangiomas (IHs) are the most common benign tumors of infants, affecting females more often than males. IH is a growth with a unique vascular phenotype of new blood vessels forming from circulating stem cells rather than a tumor of ordinary cutaneous blood vessels. They are not randomly distributed and vary enormously from small benign growths to large, function-threatening tumors. There are two important patterns named localized hemangiomas and segmental hemangiomas. The former are spatially confined and the latter distribute over a specific cutaneous territory. The mean size of segmental hemangiomas is 10 times that of localized hemangiomas. Segmental ones meanwhile have an earlier age of presentation. Segmental hemangiomas have a higher risk of complications and usually need for intervention. Approximately 41% of IHs are located on the face. There is a much higher risk of disfigurement for facial IHs than those in other sites since facial IHs lead to increased scarring. And the soft tissue distortion in the face can lead to permanent deformities. A precious study has reported that 20.3% of IHs in facial area involved lips. With respect of the small area, the constituent ratio is quite notable. Perioral hemangiomas are of special concern, since they occur in such a prominent site on the face that could develop ulceration, cause disfigurement, and lead to feeding difficulties. The lip hemangiomas commonly present ulceration. As reported, hemangiomas of the lower lip, neck and anogenital region were significantly more likely to ulcerate than those in other body locations. There have been studies on the characteristics of periocular hemangiomas and extremity hemangiomas. Accordingly, the lip IHs are also have unique features. However, studies on clinical characteristics of lip IHs are still not enough. It is often difficult to decide which condition of lip IH is at higher risk of complications. This cohort study aimed to describe the clinical characteristics of lip IHs, which might aid in understanding risk of complications and deciding intervention time.Methods:All the cases of IHs were collected in the Department of Dermatology at Zhujiang Hospital. Only cases diagnosed as lip hemangiomas visiting between June 1,2010 and October 31,2015 were included in this study, which was defined as any IH covering any part of vermilion. Perioral hemangiomas which did not involve vermilion were excluded. IHs treated before did not meet our criteria, making sure they were in natual state. All cases were diagnosed based on medical history and lesion manifestation. Medical records and photos were sources of data. We collected and reviewed the following information:sex, age, gestational age, birth weight, maternal age at birth, maternal infertility, specific events during pregnancy, family history and other desorders. Clinical characteristics of lip IHs were reviewed, including age at appearance of IHs, growth rate, location, other sites involved, subtype, size, and ulceration. Oral mucosa was checked for all the patients. As lip or perioral IHs can be accompanied by airway or hepatic IHs. We looked out for breath symptoms and liver ultrasound. And all patients were checked with ultrasound cardiogram and electrocardiogram. The size of each hemangioma was measured using "hemispheric" measurement. The surface area of each hemangioma located on the lip, including continuously involved lip external skin and intraoral mucosa, was measured in two perpendicular directions using the maximum diameter in each to give a measurement in cm2, with a soft tape measure. Continuous variables were compared using t-test. Chi-square test was performed to evaluate the relative contribution of clinical features. All statistical tests were two-sided, and all analyses were carried out with the software IBM SPSS 20.Results:A total of 421 patients of IHs were enrolled in an over 5-year period, of which 46 hemangiomas (10.9%) were accorded with the inclusion criteria. The sex distribution was 19 boys and 27 girls, translating to a ratio of about 2:3. Median age of the patients at initial visit was 91 days,75% of which were younger than 136 days of age. Ten (22%) were premature infants with gestational age ranging 32-36 weeks. The mean birth weight was 3.00 kg (range,2.05-5.00 kg). Of the 46 cases,9 (20%) were low birth weight (defined as 1.50-2.49 kg),33 (72%) were normal birth weight (defined as≥2.50 kg and≤4.00 kg), and 4 (9%) were macrosomia (defined as>4.ookg). The average maternal age was 29 years (range,20-39 years), and 4 women were older than 35 years. The median age at the appearance of IHs was 9 days, ranging from 1 day to 60 days of age. Lip hemangiomas were noted at birth in 11 patients (24%). Among the included cases,14 cases first visited doctors without any treatment at least 4 months after hemangiomas appeared. Three infants frequently sucked on the upper lip before their upper lip IHs occurred. The lip IHs grew fastest during the age of 5 to 8 weeks. Nineteen hemangiomas (41%) extended to oral mucosa or gingiva. No airway or hepatic IHs were found. Twenty-two patients (48%) involved other regions simultaneously. The most common sites involved were jaw, cheek and nose. Ten (22%) of lip hemangiomas were segmental, and all the others were localized. The mean size of hemangiomas included was 2.92 cm2 (range,0.15-14.00 cm2). The lesion largest diameter ranged from 0.5 to 5 cm, with an average of 2.0 cm. A total of,24 lip hemangiomas developed ulceration, which occurred in 52% of the included cases. Lip hemangiomas experiencing ulceration had a mean size of 4.42 cm compared with 1.29 cm for those without ulceration. The difference between ulcerated and non-ulcerated hemangiomas was 3.13 cm2 (P0.001; 95%CI:1.82-4.44 cm2). None of the lip IHs with a size less than 1 cm2 develop ulceration and all those with a size over 3 cm2 had ulcers. Hemangiomas located on the lower lip were more likely to ulcerate (P=0.003). Ulceration occurred on 29% of the upper lip and 72% of the lower lip IHs in our cases. Eighteen of the 36 non-segmental hemangiomas were ulcerated (50%). Six of the 10 segmental hemangiomas experienced ulceration (60%). No significant difference of incidence of ulceration between segmental and non-segmental hemangiomas was found in this study (P=0.84). Ulceration was found between 4 days and 70 days after appearance of lip IHs, with an average of 33 days.75% of the lip IHs developed ulceration within 42 days after hemangiomas occurred. Of the ulcerated lip hemangiomas,21 (88%) resulted in bleeding. Hemangioma surface would turn more swollen with grey-white color (71%), or shiny red colour (25%) before developing ulceration.Main conclusions:In this study we reviewed 46 cases of lip hemangiomas, which, we believe, represents the published largest series of lip IHs in China and even in Asia. A total of 421 patients were enrolled within the specified time diagnosed as IHs. The 46 cases with lip involvement accounted for 10.9% of all the cases of IHs. It is worth mentioning that the proportion of vermilion is not more than one thousandth of the whole body surface area. Relatively there was a very high incidence of IHs located in vermilion. In the first week of life,64% of lip IHs had appeared. And totally 85% of lip IHs presented within the first two weeks of life, as was consistent with features of infantile hemangiomas. Namely, most infantile hemangiomas do not appear at birth until several weeks later, which is entirely different from vascular malformations. Though most lip IHs became evident within two weeks after birth, a few cases would not manifest clearly until one month or even two months. Frequently sucking on lip may indicate following formation of lip IHs, which has not be reported by other authors. The reason of the abnormal movement of the upper lip is still unclear. It is nesscery for global specialists to observe it further. We found the lip IHs grew fastest during the age of 5 to 8 weeks. Before, other IHs were usually recorded growing fastest at the age of 3 months. Maybe the peak rate of lip IHs precedes the rate of other IHs. The upper and lower lips were almost equally involved. In this study, the hemangiomas of lower lip are more likely to develop ulceration than those of upper lip, confirming the previous records. The inevitable maceration and friction of the unique location lead to ulceration. IHs involving the chin or lower lip have been associated with airway involvement. But in this study, we discovered none. Over half of the lip IHs were accompanied with other sites of involvement. Jaw, cheek and nose were the most likely involved locations. IHs on the upper lip usually extended to under-nose, nasolabial and narial skins, whereas IHs on the lower lip often extended to jaw and cheek. Importantly, almost 41% of hemangiomas affected oral mucosa, which were most commonly found on the intraoral lip mucosa and gingivae. The ratio of segmental versus localizd hemangiomas was 5:18, a little larger than the previous reported ratio. Segmental IHs were more common in lip hemangiomas. Different from previous studies, there was no significant difference of ulceration between localized and segmental hemangiomas in this study. There was significant difference of size between ulcerated and non-ulcerated lip IHs (P0.001), consistent with another study. Over half of lip IHs developed ulceration (52%), higher than any reported incidence in other sites. In this study, there were 24 cases of ulcerated hemangiomas overall, including 72%(18/25) and 29%(6/21) of the lower and upper lip, respectively. Comparably, only 30%(21/71) of lower and 19%(14/72) of upper lip hemangiomas in the American infants developed ulceration. The most likely explanation for such tremendous difference between two studies was early or late intervention time rather than racial difference. The high incidence of ulceration in this study showed that lip IHs were more likely to develop into ulceration compared with the reported incidence. To sum up, the development of ulceration was highly associated with big size and lower lip, but without association with subtype based on this observational study. The duration of ulcer formation (mean 33 days) was well ahead of reported 93 days and 6.6 months. Lip IHs could form ulceration within merely 4 days of duration. Previous investigation has shown that most of IHs can reach 80% of their maximum size by five months of life, just when the majority of overall IHs develop ulceration. Our finding indicated the ulcerated hemangiomas located on the lip could occur at an earlier stage of IH proliferative phase. In addition, there was a highly incidence of bleeding for ulceration (88%) in lip IHs compared with reported 41%of all ulcerated IHs. We speculated the particular anatomical structure and frequent sucking of the lip might be key factors. The painful ulcerated hemangiomas often caused infants crying, having difficulty in feeding, and having sleeping disorder. Of note, lip IH surface turned pale or bright crimson swollen appearance, which might be very important signal and quickly followed by ulceration. Early white discoloration of IH has been reported as a sign of impending ulceration. There is a high incidence of IHs involving vermilion. Most lip IHs first appear within two weeks after birth. As to the high-risk features of ulceration on lip IHs, we have identified as follows:(1) The incidence of ulceration on hemangiomas of lower lip are more than double of that on hemangiomas of upper lip. (2) The lip hemangiomas with a size over 1 cm2 may develop ulcers, and if the size is more than 3 cm2, all lip IHs form ulcers. (3) Lip IHs are likely to develop ulcers when the duration exceeds 15 days and most ulcers have occurred over 45 days of duration. (4) Regardless size, duration and location, a lip hemangioma shows grey-white, or shiny red swollen appearance, highly suggesting the ulceration occurs quickly. This study highlights the importance of early interventions for the lip IHs. To our knowledge, there are few studies about clinical characteristics of lip hemangiomas with detailed information. This study may be very useful for clinicians. |
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