Hemangiomas are still one of the most common skin lesions in infancy and early childhood. To cope with such a lesion is a persistent problem for all affected children and parents alike. But not exclusively, it is an ongoing challenge for us carers and the health care system as well. And, it is not only the delicate location of these hemangiomas in a most sensitive area like the genitals, unfortunately, its their (potential) complications like ulceration and hemorrhage, that needs to be taken into consideration quite frequently, too.

Thus, the question, -“How to manage and treat such hemangiomas best…?”-, is still a matter of debate in our allday clinical practice.

Especially, since the former practised benign neglect of such lesions is no longer in favour, thanks to the improved understanding of the natural course and general acceptance of a more accurate classification scheme of such hemangiomas. Respectively, recent advantages in wound care and/or laser technology made it much easier to intervene earlier and more effective as well. And this not only for lesions, that would otherwise leave a grossly unacceptable result behind. Quite contrary, such an early approach will even prevent these children to suffer any of the negative clinical and psychological impacts such lesions will have on their future life.

In the following paragraphes we would like to present our results using minimal access laser therapy (MALT) in the care of this small cohort of patients.

Five little girls (Median age: 6 mos, range: 2.5-118 mos) with complex genitary hemangiomas have been treated with MALT in a Swiss Children`s Hospital over a time period of 3 years. Each hemangioma has been subclassifed either into “capillary”, “cavernous”, or “mixed” in accordance to the hemangioma`s overall morphological appearance and the dominating part of the lesion [1-3]. Ulceration and hemorrhage, either preexisting or appearing in due course, diagnosed alone or in combination, have been documented and treated accordingly and immediately after first notification [4-6].

For details regarding type, size, location and complications please visit (Table 1 and Figure 1).

Table 1: Details of hemangiomas regarding type, size, location and complications.

Figure 1: a) Patient B.C. presenting with an erythematous soft swelling of her nipple area. b) During laser treatment.

A continous Nd:YAG (neodymium:yttrium aluminum garnet) laser (CLMD 220-60; 1064 nm; Surgical Laser Technology (SLT), Montgomery PA/USA) beam of 5-10 W has been applied straight to the capillary parts of the hemangioma in glass plate-compressiontechnique. While the cavernous parts of the hemangioma, indeed, have been treated with a continuous 30-35 W laser beam transmitted via an ice cube (ice cube-cooling-technique). For the deep parts, respectively, the intralesional bare fiber-technique (5-8 W) has been used. Margins or other specific areas of the hemangioma have finally been treated in pulsed spot (0.2-0.5 s), non-contact technique with repeated intermittent cooling of the skin, if deemed necessary (Table 2).

Table 2: Details of surgical laser techniques applied.

All laser sessions have been performed under general anesthesia on a 4 to 6 weeks basis; in the majority in the day surgery department of the Children`s Hospital. Follow-up visits have been scheduled every 4 weeks. On parent’s request intermittent hydrocolloid foam dressings have been applied on the ulcerated areas. No antibiotics have been prescribed on a regular basis.

Temporary and final results have been documented with a standard digital camera (Samsung, Digimax S 1000). Treatment has been graded a success when ulceration or hemorrhage stopped, and the hemangioma showed constant involution.

In Median 2 (range 1-4) laser sessions have been administered to each individual patient to stop ulceration and hemorrhage and to induce constant involution. The Median laser energy applied per cm² and per session ranged widely from 500-13 075 J in our patient sample.

For more details on the final results please see (Table 3 and Figures 1-5). Finally, no laser specific complications have been seen.

Table 3: Results of MALT at-a-glance.

Figure 2: a) Patient K.I. presenting with painful swelling around her nipple. b) After 1 session swelling could be reduced and patient was pain free.

Figure 3: a) Patient Sch.E. at the time of her first presentation. b) After initiation of laser treatment, when involution starts.

Figure 4: a) Patient M.T. on admission presenting with extended glutealperineal- genital hemangiomas. b) Showing ulceration in due course, but responding well to NdYAG laser therapy.

Figure 5: a) Patient H.M. with her isolated ulcerated and bleeding labial hemangioma. b) Ulceration and bleeding stopped immediately after NdYAG laser application.

Hemangiomas are common tumors in infancy and one of the most common congenital anomaly in the human. During the last decades the general and non-specific term “hemangioma” has been traditionally applied to all sorts of benign vascular tissue tumors, vascular birthmarks, and vascular malformations [1,2,7]. In order to develop a more stringent classification for the “real” hemangioma, several revisions and updates have been undertaken over the years, but none has been finally approved yet and the discussion is still controversial [1,7-10].

However, at the time this study started, the final decision has been to classify the simple genitary hemangiomas in either “capillary”, “cavernous” or “mixed” lesions based on the following definitions: A “capillary” hemangioma is represented by a bright red, raised lesion, ranging from millimeters to several centimeters in diameter. It is always well circumscribed, and only partially compressible. A “cavernous” hemangioma instead, is a more nodular one, that could be large in diameter, width and depth as well. It is often bluish or purple and easy compressible. The “mixed” lesions, indeed, have components of both [1,8,9].

Hemangiomas found in developmental segments are refered to as “segmental” lesions. These segmented hemangiomas are commonly associated with other extracutaneous abnormalities. Here the PELVIS/ SACRAL syndrome denotes the association of segmented hemangiomas of the loins (sacrolumbar region, buttocks or perineum = napkin hemangioma) with spinal dysraphia affecting the sacrolumbar spine, the terminal medullary cone, the genitourinary organs and the anal region to a varying degree [11-13]. The acronym “LUMBAR”, indeed, is proposed by several authors to describe the association between Lower body hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, or Arterial and Renal anomalies further. Because of the many similarities between the LUMBAR association and a PHACE syndrome, they might be considered as a regional expression of the same. In general, such segmental lesions have a higher risk of threatening life or function, and when they are located in the perineal area, they are frequently associated with developmental anomalies and even more often complicated by ulceration. The diagnosis of such hemangiomas associated with a PELVIS/SACRAL/LUMBAR syndrome might be delayed or complicated, too, due to the common macular, teleangiectasic or livedoid appearance of these lesions [10- 13], which was noticed in one of our patient as well.

The “rule of thumb”, stating that all hemangiomas appear after a few weeks of life, growing fast, before starting a slow involution is old fashioned and too simplistic, because it does not tell parents or physicians anything about the lesion`s further growth or potential to destruct sensitive surrounding tissues. But, as soon as we are able to stop the hemangioma`s growth early, we can definitely guarantee a final scar or spot not bigger than the primary lesion itself. If we let it grow instead, we definitely will end up with either a completely involuted hemangioma with no real difference to its surrounding tissue (most favourable). Or, a big fibrous scar and fatty tissue remnant, that needs surgical correction (and hopefully can be corrected) later in life, respectively, a huge area of destruction and disfigurement, where the chance of reconstruction is nil (because we are too late) [6,7].

Fortunately, this traditional approach of “benign neglect” is nowadays challenged more and more by considering hemangiomas in locations, like the face, the digits and the perineal and genital area, as real “cosmetic emergencies” [6-8,11,14-18]. Here, in parallel to functional and cosmetic irritation, immediate intervention is of key importance, because of the permanent concerns and psychosocial trauma the child and his/her family is subjected to. So far, this has been one of the most neglected facts in the management of this type of hemangiomas [18-25].

Later in adolescence, the most critical transition period from childhood to adult sexual and social behaviour rules, increasing normative attention to anxiety, worries and concerns regarding self-esteem, overall physical appearance, and body image have to be payed for, too. Many of these adolescents suffer depressive and attendant behavioural disorders like fatigue, poor concentration, and hypochondriasis. Or even self-imposed isolation, suicide thoughts and attempts. In fact, their psychosocial well-being is at risk. However, in comparison to other physical impairments or differences, so far no reports of higher rates of clinical depression, psychosocial disturbances or suicides are available [7,22,24].

The denial is still so great, that many medical textbooks still fail to mention it. And, at least in the past, we have to confess, that this “wait & see” approach, or considering any treatment “just as cosmetic”, left us behind too often. Than, obviously there is a hemangioma, and this hemangioma anyhow will certainly subject this child to several years of emotional trauma. That this usually happens during the formative years of the child`s individual personality, will make it even worse and it will certainly interfere with his/her puberty as well. With no doubt, these effects are profound and most likely irreversible[7,22,24].

Ulceration is one of the most common physical complications seen in up to 35% of patients with centro-facial and genitary hemangiomas [4,5,26-30]. Such an ulceration can cause pain, or making difficult time for dressing or nappy changes. Ulceration can be further complicated by secondary infection (4.9%) [27] and hemorrhage. This hemorrhage can even lead to severe anaemia with the need for a blood transfusion. Finally, often a disfiguring scar follows this wounding [5]. Since ulceration can happen anytime, before, during or after any type of treatment, at least a superficial ulceration is nowadays considered more and more as normal course, or regular part of the treatment than as a complication per se [4,7,18,23,26-33].

But, despite this high overall complication rate, the rate of the “so far not-treated”, and the “traditional wait & see approach” at the time of their first consultation can still reach up to 49% ! [3,27,34], which is in coincidence with the author`s personal experience. In addition, any identified (or unidentified) psychological distress is significantly associated with extra medical complications, but never with the lesion site or surface area. And, with any repeated intervention, regardless if based on a real or just imaginary medical complication, the expectations, worries and concerns of the parents immediately increase manifold as well. That is why several studies end up with the expression of substantial parental dissatisfaction with nearly all aspects of the medical care of their children`s hemangioma [21].

Obviously there is no real doubt that almost all hemangiomas finally “involute”. But, what is really meant by this, and is that grade of involution (nowadays) really satisfying and acceptable enough [7,24] ?? On the other hand, authors advocating in general against any early intervention, may have underestimated the fact, that in reality every hemangioma will definitely leave a “residuum” behind [14,20,25,27,35]. And that both, final “involution” and “residuum” to date might be considered as absolutely inacceptable. Too far away from actual standards raised by patients, parents and the (medical) society for a sensitive body area like the genitals.

In contrast, any over-aggressive treatment or previously ill-planned surgery, when indeed followed by a good involution, will left the child behind with a grossly deformed or deficient body part as soon as he/she gets older [14,19,20,25,27,35]. Which, of course, will be inacceptable, too.

Thus, the risk of a small physical scar, resulting out of a well-planned early active treatment, should always be weighted up against the sequelae a lifelong emotional scar will have. Especially, when nowadays we are able to treat genitary hemangiomas more safe and easily, thanks to the recent advances in medical science. And this not only for lesions, that would otherwise leave a grossly unacceptable result behind. Quite contrary, these advances now permit treatment safe enough to prevent these children from suffering any of the negative psychological impacts such genitary hemangiomas might have on them in their future life [7].

Surgical treatment options for (genitary) hemangiomas are mainly cryosurgery, plastic surgical excision and laser surgery among others [7,16,17,36-38].

While cryosurgery is widely used in Europe, less experience is found in the United States due to its proposed potential for more extensive scarring [16,17,30,39]. In Germany indeed, in order to prevent maceration and infection of hemangiomas in the ano-genital area, and by supporting the philosophy of considering such hemangiomas as a “cosmetic emergency”, the use of cryosurgery in form of Peltier elements or ice cube-cooling is recommended as early as possible, and not found to be associated with an increased scarring potential [16,17,28,38, 40,41].

Indications for surgical hemangioma removal in delicate areas are seen quite restrictive in both, past and present guidelines. In the majority indications are limited to function-threatening lesions, serious complications, non-responders to pharmacotherapy, or other less invasive alternatives. Respectively, when most probably a fibrofatty or involuted residuum will finally stay behind, that can be excised easily without leaving behind a significant cosmetic deformity, or the lesion persists showing atypical (malignant) growth. However, in each individual case the final rate of involution, the most likely prognosis and the parental desires and expectations must be weighted up carefully and seriously against the ultimate results, that can be achieved by other treatment modalities [7,30,33,39,42].

In 1988, Berlien and co-workers, later joined by Apfelberg et al. and Clymer et al., introduced Nd:YAG laser for the treatment of hemangiomas with promising results. Shortly thereafter, it has become a general agreement, that laser therapy will be a valuable tool in rendering an inoperable lesion into a safely resectable one. Respectively in treating lesions, that interfere with body orifices, by markedly decreasing the lesion`s size and functional impact [4,7,8,20,41].

Since the wavelength of the Nd:YAG laser is mainly absorbed in blood in comparison to other tissues, its main effect as the result out of its deep penetration and widespread scattering is below the surface and within the hemangiomatous tissue [8,9]. Therefore, additional cooling and/or compression should be applied to protect the overlying epidermis and all other tissue layers from unwanted thermal injury. Here any type of pre-, intra- and post procedural cooling and/or compression, applied either alone or in combination, will be able to reduce the adverse laser effects on collagen stimulation and the risk of a thermal injury. In addition, by stimulating the vasoconstriction of the vessels, which subsequently leads to diminuished blood flow, Nd:YAG laser administration will be even made more effective [8,28,38,40,41,43,44]. Thus, the combination of these techniques combines all the positive laser- and cryosurgical effects to its best.

All laser parameter settings and application techniques have been adjusted according to the recommendations of globally renowned laser operators [5,8,9,15,20,38,42] and our own experience [7,43,44].

Each individual treatment usually consisted out of multiple Nd:YAG laser sessions performed under general anesthesia. General anesthesia is mandatory, because the laser beam is painful, and will have hazardous effects on ie the eyes, when operated uncontrolled in a struggling and fighting child. For many carers this immanent multisession schedule is sufficient enough to reject the laser option forever. Obviously and for sure, any “restraining-torture”-management is absolutely inappropriate and inacceptable, even when so far no long term psychological effects after repetitive painful treatments to a restrained young child are determined in the medical literature. In addition, the physical and mental health risks of repeated general anesthesias to a young child are still undetermined as well. On the other hand, there are no reports either, that a well-balanced and monitored pediatric anesthesia will have a harmful impact on the regular development of these little patients [19,45]. Last but not least, the decision made for the need of general anesthesia in children has always to be based on a correct indication and its real need for this specific pediatric surgical procedure. Thus, any detainment will be definitely more harmful and more disadvantageous for the child`s well being [19,45].

In the majority of cases our Nd:YAG laser patients have been served safely on a day case basis, which is in coincidence with a literature review [32]. Follow-up schedules after Nd:YAG hemangioma laser treatment are various, with some authors requesting 2-6 weeks intervals during active treatment, followed by 3 months intervals for the first year. Thereafter, yearly visits for a maximum of 8 (Mean 6) years have been recommended [4,28,42]. However, since the exact time, when ulceration and hemorrhage, or “changes” in the genitary hemangioma`s appearance or laser response occur, will be hard to determine in advance, providing the child and his/her parents with a flexible schedule like in this collective might be more advantageous.

Briefly, in coincidence with our results the Nd:YAG laser is a valuable and successful tool in treatment of genitary hemangiomas in girls. Especially, since this laser has proven to prevent excessive growth and to promote involution in all types of hemangiomas with minimal adverse effects. Its effects are even more pronounced, when applied early, and most effective, when used in combination with cooling and compression techniques [7,8,38].

Mainly in the past and despite controversity about their real effectiveness, systemic or intralesional steroids have been considered as a mainstay in hemangioma treatment. But, quite a considerable number reported severe adverse effects, like moon facies, behavioural changes, osteoporosis, irritability, immunosuppression and growth retardation [3,4,6,7,20,24,25,27,28,30-33,35,42,46]. That is why steroids have never been considered seriously in our hemangioma management policies.

By chance, French authors detected in the year 2008, that propranolol has been able to stop hemangiomas` growth. Since it has been acting so powerful, it has been proposed, that it might has the potential to cause a paradigm shift in their future management [11,14,26,46]. So far, common indications are large hemangiomas in endangering localization [11,14,46,47]. Propranolol treatment should always starts as soon as possible during the proliferation phase [46]. In the meantime, promising results after topical application have been found, too [47].

Shortly summarized

Propranolol is currently used worldwide as well as in the author´s daily practice for all types of hemangiomas with an impressive effect and a low toxicity profile. But it has to be kept in mind, that final pediatric pharmaceutical approval is still pending [7,48]. Propranolol is likely to play a substantial role in the treatment of genitary hemangiomas in the near future. Either alone or in combination with the Nd:YAG laser device. However, since propranolol has not been inaugurated when this study started, it has not been tested in this patient collective. The evaluation of the results after Nd:YAG laser therapy is usually based on the following standard criteria: reduction in size and area of the target lesion, the amount of laser energy applied, and the number of treatment sessions needed to achieve this actual result [7,41]. Others, that have to be mentioned are changes in morphological appearance, improvement in function and the elimination of complications, like ulceration and hemorrhage [8,29]. Since especially ulceration and pain are always a major concern in treatment of genitary hemangiomas, many authors has put their focus on it. While in the past, Lacour et al. exclusively favoured conservative management, with local cleaning, application of topical antimicrobials, and systemic antibiotics, they later have changed to pulsed dye laser treatment, because of its rapid alleviation of pain, and the early restoration of micturation and defecation. But in contrast to our results, they experienced Nd:YAG laser treatment or cryosurgery as a complete failure [7,36]. In a more recent publication, Dixon and Gilbertson have recommended specifically the use of the argon laser for ulceration and bleeding complications, or hemangiomas encroaching into body orificies. But, it has to be considered, that the authors described their “successful” results in a way, that “the lesion usually become ulcerated without bleeding”, and that one of the earlier classification scheme has been in use. They mentioned the Nd:YAG laser only briefly and without any further specification [37]. In their current publication Vlachakis and co-workers` objective has been to determine, whether or not the cooling of the epidermis during Nd:YAG laser irridation prevents thermal damage and decreases the number of sessions required to treat such lesions. Like in our study, a comparable proportion of cases complicated by ulceration and bleeding, endangering impairment of important functions, or significant cosmetic disfigurement and psychological problems related to the patient`s appearance has been presented. None of the patients have received any other treatment such as steroids, but antibiotics have been used as a routine. Vlachakis et al., of course, have been intending primarily on cutaneous and facial rather than on genitary and extracutaneous hemangiomas like we did, but complications and cosmetic disfigurement reported for both collectives seem to be comparable enough to support the early treatment for both. Suitable treatment therefore means early Nd:YAG laser irridation in combination with ice protection of the epidermis, since this has produced good cosmetic results and decreased the number of treatment sessions finally needed [38].

As already mentioned, the combination of Nd:YAG laser, cryosurgery and surface cooling is nowadays a valuable tool in the treatment of genitary hemangiomas and to combine it with propranolol might be even more superior in the future.

All genitary hemangioma have been documented and photographed on a regular basis. Because, their real appearance is too quick and easily forgotten in the majority of cases, so that a “before and after” picture right in hand, would always serve and has always served as the perfect “reminder” and great “morale booster” for both, parents and children alike [4,8,23,29,42].

“Lesions in cosmetically strategic locations, such as the face, genitalia and nipple area can also be considered endangering in a sense, that they are more prone to ulceration, superinfection and pain, and that they can produce permanent disfigurement and psychological issues to the patient and his/her family….” [11,49]. For the first time this statement has payed real attention to the particular concerns, patient, parents and carers usually encounters, when they have to face a hemangioma in the genital area. Plus concerns about atrophy or unwanted scaring after involution, especially when the breast, the breast bud, or the nipple area are at risk.

Bava et al. have presented a case report on a capillary vulvar hemangioma, that has been complicated by life-threatening hemorrhage, not responsive to corticosteroid therapy, and that showed no signs of spontaneous involution. Their therapeutic approach consisted out of 3 selective arterial embolization sessions, followed 5 months later by surgical excision and reconstruction of the vulva. Selective embolization has been rarely used in the treatment of hemangiomas, but in this case it has been a good option in preparing for the final surgery [50].

Baetz et al. reported on a 4 month old baby suffering from a 5×3 cm big ulcerating hemangioma on her left labia majora. Systemic treatment with off-label-use of propranolol has been initiated. After a few weeks, the size and color of the hemangioma has been reduced, and 5 ½ months later almost complete regression has been achieved [49].

Both publications re-underline again, that hemangiomas of the genitals are a clear indication for early active treatment.

Iacobas et al. conducted a multi-institutional, retrospective case analysis on the LUMBAR syndrome based on their patients and published cases in order to assess the potential risk for underlying anomalies in regard to the hemangioma`s specific location. Most hemangiomas tended to be “segmental” and “off minimal growth” in morphology. Such lesions often have been extensive, covering the entire leg. Such extensive limb hemangiomas showed a high potential for extracutaneous anomalies, like ulceration, limb underdevelopment or underlying arterial anomalies. The cutaneous hemangioma and its underlying anomalies demonstrated regional correlation and myelopathies have been the most commonly associated [51]. By trying to subclassify our patient, we found female predominance, too, segmental, but “fast and extensive” growth, and involvement of more than one region. In fact, all regions have been involved plus extension across the midline. Like in their patients, ulceration has been extensive, the extracutaneous anomaly found has been tethered cord, and in MRI evidence of myelopathy has been shown.

Main goals in treatment of genitary hemangiomas are minimization of complications, like ie ulceration, hemorrhage or pain, full restoration of function, maximization of the cosmetic appearance, the minimization of the emotional trauma and a significant decrease in time and age at which the final result could be obtained. The MALT and cryotherapy seems to be valuable tools in achieving this.

Limitations of this study are: the small number of cases and their short follow-up. The broad variance within the diagnosis “genitary hemangioma” and the different classification schemes used in literature reviews, making it hard to quantify the outcome in a retrospective study design.