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The Role of Surgery in the Management of Congenital Vascular Anomalies
The Role of Surgery in the Management of Congenital Vascular Anomalies Milton Waner, MB,BCh (Wits), FCS(SA), MD, and Teresa Min-Jung O, MD Surgery is one of the modalities used to treat vascular lesions. Its role is to act in concert with interventional radiology and the various pharmacologic agents. The role of surgery in patients with hemangiomas is limited to nonresponders to propranolol, complications, and eyelid lesions. Corrective surgery would also normalize facial distortion and asymmetry which may result from a hemangioma. Concerning vascular malformations, cure may only be obtained in case of small, focal lesions. In larger, extensive lesions, the role of surgery is limited to debulking the mass. This is usually performed after preoperative embolization or sclerotherapy to reduce the amount of intraoperative blood loss. Surgery is usually not curative but would decrease the amount of disease needing to be sclerosed. Surgery is therefore most often an adjuvant therapy. Tech Vasc Interventional Rad 16:45-50 C 2013 Published by Elsevier Inc. KEYWORDS vascular anomaly, vascular malformation, infantile hemangioma, surgery, laser, sclerotherapy, embolization, interventional radiology
T
he past decade has seen major advances in the management of infantile hemangiomas and vascular malformations. Multidisciplinary vascular anomalies clinics have been established in many centers and clearly the way forward is for team members to work in tandem. Each of the disciplines has a role in the management of these patients and we should never practice in a vacuum. Surgery is without a doubt an important modality in the management of these lesions but a surgeon cannot and should not work alone. Close collaboration between a surgeon and an interventional radiologist can prevent mutilating surgery and ensure the best possible outcome for the patient.
Infantile Hemangiomas The recent discovery of effectiveness of propranolol in treating hemangiomas has completely shifted the paradigm of management for these lesions.1-3 Despite this, there is still a role for surgery. A recent study found that 50% of patients, when treated with propranolol, had no need for treatment with another modality.4 This means Vascular Birthmark Institute of New York, New York, NY. Address reprint requests to Teresa M. O, MD, Vascular Birthmark Institute of New York, 126 West 60 St, New York, NY 10023. E-mail: [email protected] 1089-2516/11/$ - see front matter & 2013 Published by Elsevier Inc. http://dx.doi.org/10.1053/j.tvir.2013.01.006
that the other 50% of the patients would need further treatment. Propranolol appears to be more active against segmental hemangiomas and for these lesions, when treated early, there is only rarely a need for surgery. Focal hemangiomas appear to be less responsive to propranolol and although they do respond, at least 50% will not respond sufficiently and will need surgery. Apart from this, there are instances in which surgery is indicated.
Ulceration An ulcerated focal hemangioma is usually extremely painful. Even though propranolol is used as the first line of treatment, it may take months to heal5 and result in scarring. The child, in the meantime, is in severe pain and at risk for hemorrhage and infection. Surgical removal of the lesion can alleviate the problem more rapidly (Fig. 1). This is usually only possible in the presence of an ulcerated focal hemangioma.
Eyelid Hemangiomas Eyelid hemangiomas are often complicated by astigmatism. It has been shown that early removal of the hemangioma often reverses the astigmatism.6 This should be done as soon as possible after the astigmatism has been diagnosed and certainly before the eighth month of life. 45
M. Waner and T.M. O
46
In about 50% of cases of children with focal hemangiomas treated with propranolol, shrinkage of the lesions is insufficient (Fig. 5). In many of these cases, surgery will correct the problem.
Vascular Malformations
Figure 1 An infant with severe ulceration of both cervical hemangiomas, before and 6 months after surgery. (Color version of the figure is available online.)
Eyelid hemangiomas can frequently be removed using simple procedures and with minimal blood loss (Fig. 2).
Anatomical Distortion Ulceration, tissue loss, and mass distortion often need to be corrected surgically. This is especially true for lip and nasal tip hemangiomas (Figs. 3 and 4). Simple procedures aimed at improving function and appearance can greatly improve the quality of life of the patient.
Surgical resection of vascular malformations has evolved. The role of the surgeon in a multidisciplinary team is to work in tandem with the interventional radiologist. Surgical resection is used to debulk large segments of malformation. This is often done with the aid of pre-, intra, or post-operative embolization or scerlotherapy. In general, excision of a vascular malformation is both difficult and hazardous. Preoperative sclerotherapy, performed 24-48 hours before surgery, coagulates a spongy vascular lesion into a firm, solid mass (Fig. 6). The added soft-tissue edema accentuates the tissue planes and thus significantly decreases the difficulty of the dissection, and thereby minimizes intraoperative blood loss. This is especially important in young children whose total circulating blood volume is much less than that of an adult. The hemorrhage that can accompany surgical resection of an unsclerosed lesion can be life threatening. So as to optimize treatment, preoperative planning must be undertaken. A combined multidisciplinary conference should discuss both the clinical needs of the patient and the radiologic images. It is often not possible
Figure 2 An infant with a large upper eyelid hemangioma, before, 1 week after, and 8 weeks after surgery. This child had severe astigmatism which reversed postoperatively. (Color version of the figure is available online.)
Figure 3 An infant with upper lip distortion due to a large, previously untreated hemangioma; before and after surgical excision and after further laser treatment. (Color version of the figure is available online.)
The role of surgery in the management of congenital vascular anomalies
Figure 4 An infant with an untreated hemangioma of her lower lip. The lesion was excised by performing a wedge excision. A small segment of the hemangioma was left at the oral commissure to prevent distortion of the lip anatomy. This remaining portion is treated with laser. (Color version of the figure is available online.)
to remove the entire lesion. In these cases, the objective is to improve the quality of life, whether this means improvement of function, reduction in hemorrhage, reduction or alleviation of pain, or improvement in appearance. When faced with a large lesion, it is often necessary to stage the treatment. This would lessen the difficulty and improve safety.
Venous Malformations Skin and Mucosa Involvement of the skin and mucosa adds additional challenges. The presence of a malformation so close to the skin and mucosa would make both sclerotherapy and surgery hazardous. The risk of necrosis and ulceration postsclerotherapy is significantly increased and the ability of the surgeon to raise a viable skin or mucosal flap during surgery is challenged. Preoperative treatment with a neodynium-doped: yttrium aluminum garnet (Nd:YAG) laser will result in the development of subcutaneous or submucosal fibrosis which reduces these risks. This enables the surgeon to raise a viable flap and also reduces
Figure 5 An infant was treated with 2 mg/kg/d of propranolol for 6 months. The lesion shrunk but not sufficiently and still needed surgical excision. (Color version of the figure is available online.)
47
Figure 6 A sclerosed venous malformation. The lesion is a firm, coagulated mass that bleeds very little. (Color version of the figure is available online.)
the risk of postsclerotherapy necrosis and ulceration. In addition to this, some lesions are entirely superficial and can be treated solely with a Nd:YAG laser. The fairly recent addition of cutaneous cooling during treatment has added to the safety of Nd:YAG laser treatment. As the thermal profile of Nd:YAG laser treatment makes it especially hazardous with respect to skin and mucosa, lasers with cryogen or contact cooling should only be used. Nd:YAG laser treatment can be used to treat skin and mucosal lesions as the sole modality or as a preoperative modality (Fig. 7). In the latter, it should be done at least 6 weeks prior to surgery. This allows adequate post treatment fibrosis to develop.
Arteriovenous Malformations (AVMs) Once again, the objective of intervention should be to improve the quality of life. This may include improvement in appearance, prevention of hemorrhage, improvement of function, and prevention of complications. A multidisciplinary conference should,
Figure 7 A young adult with a venous malformation of her lower lip, before and after treatment with a Nd:YAG laser. (Color version of the figure is available online.)
M. Waner and T.M. O
48
Figure 8 A 45-year-old with an extensive arteriovenous malformation that was resected in stages. (Color version of the figure is available online.)
where possible, precede treatment. At this conference, a decision should be made concerning the treatment objective and the modalities. It may not be possible to treat the entire lesion without mutilating procedures. When faced with a large lesion, staged procedures may be necessary. Surgical resection of AVMs can be challenging. The surgeon must endeavor to differentiate between the primary nidus of the AVM and the secondary changes, although this might be difficult to ascertain. Differentiation may help to avoid mutilating surgery. Surgical removal of the nidus, and not the secondary change, is necessary. The secondary changes are due to the continuous shunting across the nidus. These consist of arterial hypertrophy, venous dilatation, and edema which typically surround the nidus. Often, the nidus can be quite small whereas the secondary changes may be extensive. Preoperative embolization as well as percutaneous sclerotherapy, completed about 24 hours preoperatively can facilitate surgical resection and reduce the risk of intraoperative hemorrhage. Staged surgical resection is acceptable (Figs. 8-10). Postoperatively, necrosis of part of the overlying skin flap is sometimes seen. This may be due to venous congestion or a steal syndrome.
Figure 9 An arteriovenous malformation of the patient’s upper lip before and after a staged excision. There is still AVM present but the patient no longer bleeds and his appearance has improved. From this point, further treatment would primarily consist of embolization and transcutaneous sclerotherapy. (Color version of the figure is available online.)
Lymphatic Malformations The treatment of lymphatic malformations is truly multidisciplinary. Clinicians have classified these lesions as being macrocystic, microcystic, or mixed on the basis of their response to the sclerosant, OK-432.7,8 In reality, most lesions are mixed and the majority of lesions should be treated both surgically and with sclerotherapy. Surgical resection is able to reduce the volume of the tumor as well as remove fibrosis that has resulted from sclerotherapy (Fig. 11). Once a reasonable contour has been achieved, the remaining disease can be sclerosed. As with the preceding categories, treatment should be planned in a multidisciplinary way.
Mucosal Lesions A site of predilection is the tongue. The typical presentation is tongue swelling with vesicle formation. The vesicles may be filled with lymph or mixed lymph and blood. These patients are almost all symptomatic. The vesicles leak blood or lymph fluid or both. The patient’s pillow is almost always stained. Occasionally, bleeding can be quite profuse. These patients also experience a burning sensation with the consumption of spicy foods and carbonated drinks. Macroglossia may also be present and may need to be addressed. The vesicles can be ablated with a CO2 laser (Fig. 12). Although this is rarely curative, it will significantly improve the patients’ quality of life. There may be periods of normal tongue mucosa which may last for months or even years.
Figure 10 A 50-year-old with an AVM of his right buttock, before and 1 year after surgery. (Color version of the figure is available online.)
The role of surgery in the management of congenital vascular anomalies
49
Figure 13 A child with macroglossia and glossoptosis before and after a tongue reduction. (Color version of the figure is available online.)
Conclusion Figure 11 An infant with an extensive lymphatic malformation in the floor of mouth and submaxillary triangle, before and after surgical debulking. The patient clearly has residual disease but further treatment would consist of sclerotherapy. (Color version of the figure is available online.)
Glossoptosis Glossoptosis is sometimes seen with involvement of the tongue or the floor of the mouth or both. It is imperative to determine the true cause of the protrusion because this would affect the treatment choice. Glossoptosis may be due to tongue enlargement resulting from involvement of the tongue, with lymphatic malformation leading to macroglossia. Alternatively, tongue protrusion may result from involvement of the floor of the mouth and a normal tongue size. This needs to be treated with sclerotherapy whereas macroglossia needs to be treated with tongue reduction surgery (Fig. 13).
In general, surgery plays a secondary role in the management of hemangiomas and vascular malformations. Most hemangiomas show spontaneous involution, and the larger or more symptomatic lesions can often be managed with pharmacologic agents such as propranolol. There remain a significant number of lesions which will require surgery due to their size, location, or interference with vital structures or functions, such as respiration, feeding, and vision. Still others may cause pain, ulceration, or such severe disfigurement that they cannot be managed conservatively. Vascular malformations may require surgical intervention when they cannot be managed with embolization or sclerotherapy. In high flow lesions, proximal ligation of feeding vessels is ineffective, and the goal must be removal of the nidus. Completely resectable lesions and those too bulky to respond to embolization are best suited
Klippel-Tre´naunay Syndrome The role of surgery is primarily to debulk the lesion and to remove as much of the angiokeratoma as possible. As most of the disease is superficial to the deep fascia and because muscle involvement is extremely rare, debulking is possible. This is usually done with preoperative sclerotherapy some 24 hours prior to surgery. The tissue heals well and the incidence of wound dehiscence is low. Angiokeratomatous tissue can be removed at the same time as debulking or separately. Staged procedures are common (Fig. 14).
Figure 12 A child with lymphatic malformation of the tongue before and after treatment with a CO2 laser. This patient is not ‘‘cured’’ but is symptom free for a period of time. (Color version of the figure is available online.)
Figure 14 A 1-year-old with Klippel-Tre´naunay syndrome before and after debulking of the lesion. Note that the leg is thinner. (Color version of the figure is available online.)
50 for surgery. Preoperative embolization or sclerotherapy is often employed to shrink the lesion and reduce intraoperative blood loss.
References 1. Le´aute´-Labreze C, Dumas de la Roque E, Hubiche T, et al: Propranolol for severe hemangiomas of infancy. N Engl J Med 358:2649-2651, 2008 2. Frieden IJ, Drolet BA: Propranolol for infantile hemangiomas: Promise, peril, pathogenesis. Pediatr Dermatol 26:642-644, 2009 3. Schiestl C, Neuhaus K, Zoller S, et al: Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas. Eur J Pediatr 170:493-501, 2011
M. Waner and T.M. O 4. Buckmiller LM, Munson PD, Dyamenahalli U, et al: Propranolol for infantile hemangiomas: Early experience at a tertiary vascular anomalies center. Laryngoscope 120:676-681, 2010 5. Naouri M, Schill T, Maruani A, et al: Successful treatment of ulcerated haemangioma with propranolol. J Eur Acad Dermatol Venereol 24:1109-1112, 2010 6. Arneja JS, Mulliken JB: Resection of amblyogenic periocular hemangiomas: Indications and outcomes. Plast Reconstr Surg 125:274-281, 2010 7. Alomari AI, Karian VE, Lord DJ, et al: Percutaneous sclerotherapy for lymphatic malformations: A retrospective analysis of patientevaluated improvement. J Vasc Interv Radiol 17:1639-1648, 2006 8. Greinwald JH Jr, Burke DK, Sato Y, et al: Treatment of lymphangiomas in children: An update of picibanil (OK-432) sclerotherapy. Otolaryngol Head Neck Surg 121:381-387, 1999
T
he past decade has seen major advances in the management of infantile hemangiomas and vascular malformations. Multidisciplinary vascular anomalies clinics have been established in many centers and clearly the way forward is for team members to work in tandem. Each of the disciplines has a role in the management of these patients and we should never practice in a vacuum. Surgery is without a doubt an important modality in the management of these lesions but a surgeon cannot and should not work alone. Close collaboration between a surgeon and an interventional radiologist can prevent mutilating surgery and ensure the best possible outcome for the patient.
Infantile Hemangiomas The recent discovery of effectiveness of propranolol in treating hemangiomas has completely shifted the paradigm of management for these lesions.1-3 Despite this, there is still a role for surgery. A recent study found that 50% of patients, when treated with propranolol, had no need for treatment with another modality.4 This means Vascular Birthmark Institute of New York, New York, NY. Address reprint requests to Teresa M. O, MD, Vascular Birthmark Institute of New York, 126 West 60 St, New York, NY 10023. E-mail: [email protected] 1089-2516/11/$ - see front matter & 2013 Published by Elsevier Inc. http://dx.doi.org/10.1053/j.tvir.2013.01.006
that the other 50% of the patients would need further treatment. Propranolol appears to be more active against segmental hemangiomas and for these lesions, when treated early, there is only rarely a need for surgery. Focal hemangiomas appear to be less responsive to propranolol and although they do respond, at least 50% will not respond sufficiently and will need surgery. Apart from this, there are instances in which surgery is indicated.
Ulceration An ulcerated focal hemangioma is usually extremely painful. Even though propranolol is used as the first line of treatment, it may take months to heal5 and result in scarring. The child, in the meantime, is in severe pain and at risk for hemorrhage and infection. Surgical removal of the lesion can alleviate the problem more rapidly (Fig. 1). This is usually only possible in the presence of an ulcerated focal hemangioma.
Eyelid Hemangiomas Eyelid hemangiomas are often complicated by astigmatism. It has been shown that early removal of the hemangioma often reverses the astigmatism.6 This should be done as soon as possible after the astigmatism has been diagnosed and certainly before the eighth month of life. 45
M. Waner and T.M. O
46
In about 50% of cases of children with focal hemangiomas treated with propranolol, shrinkage of the lesions is insufficient (Fig. 5). In many of these cases, surgery will correct the problem.
Vascular Malformations
Figure 1 An infant with severe ulceration of both cervical hemangiomas, before and 6 months after surgery. (Color version of the figure is available online.)
Eyelid hemangiomas can frequently be removed using simple procedures and with minimal blood loss (Fig. 2).
Anatomical Distortion Ulceration, tissue loss, and mass distortion often need to be corrected surgically. This is especially true for lip and nasal tip hemangiomas (Figs. 3 and 4). Simple procedures aimed at improving function and appearance can greatly improve the quality of life of the patient.
Surgical resection of vascular malformations has evolved. The role of the surgeon in a multidisciplinary team is to work in tandem with the interventional radiologist. Surgical resection is used to debulk large segments of malformation. This is often done with the aid of pre-, intra, or post-operative embolization or scerlotherapy. In general, excision of a vascular malformation is both difficult and hazardous. Preoperative sclerotherapy, performed 24-48 hours before surgery, coagulates a spongy vascular lesion into a firm, solid mass (Fig. 6). The added soft-tissue edema accentuates the tissue planes and thus significantly decreases the difficulty of the dissection, and thereby minimizes intraoperative blood loss. This is especially important in young children whose total circulating blood volume is much less than that of an adult. The hemorrhage that can accompany surgical resection of an unsclerosed lesion can be life threatening. So as to optimize treatment, preoperative planning must be undertaken. A combined multidisciplinary conference should discuss both the clinical needs of the patient and the radiologic images. It is often not possible
Figure 2 An infant with a large upper eyelid hemangioma, before, 1 week after, and 8 weeks after surgery. This child had severe astigmatism which reversed postoperatively. (Color version of the figure is available online.)
Figure 3 An infant with upper lip distortion due to a large, previously untreated hemangioma; before and after surgical excision and after further laser treatment. (Color version of the figure is available online.)
The role of surgery in the management of congenital vascular anomalies
Figure 4 An infant with an untreated hemangioma of her lower lip. The lesion was excised by performing a wedge excision. A small segment of the hemangioma was left at the oral commissure to prevent distortion of the lip anatomy. This remaining portion is treated with laser. (Color version of the figure is available online.)
to remove the entire lesion. In these cases, the objective is to improve the quality of life, whether this means improvement of function, reduction in hemorrhage, reduction or alleviation of pain, or improvement in appearance. When faced with a large lesion, it is often necessary to stage the treatment. This would lessen the difficulty and improve safety.
Venous Malformations Skin and Mucosa Involvement of the skin and mucosa adds additional challenges. The presence of a malformation so close to the skin and mucosa would make both sclerotherapy and surgery hazardous. The risk of necrosis and ulceration postsclerotherapy is significantly increased and the ability of the surgeon to raise a viable skin or mucosal flap during surgery is challenged. Preoperative treatment with a neodynium-doped: yttrium aluminum garnet (Nd:YAG) laser will result in the development of subcutaneous or submucosal fibrosis which reduces these risks. This enables the surgeon to raise a viable flap and also reduces
Figure 5 An infant was treated with 2 mg/kg/d of propranolol for 6 months. The lesion shrunk but not sufficiently and still needed surgical excision. (Color version of the figure is available online.)
47
Figure 6 A sclerosed venous malformation. The lesion is a firm, coagulated mass that bleeds very little. (Color version of the figure is available online.)
the risk of postsclerotherapy necrosis and ulceration. In addition to this, some lesions are entirely superficial and can be treated solely with a Nd:YAG laser. The fairly recent addition of cutaneous cooling during treatment has added to the safety of Nd:YAG laser treatment. As the thermal profile of Nd:YAG laser treatment makes it especially hazardous with respect to skin and mucosa, lasers with cryogen or contact cooling should only be used. Nd:YAG laser treatment can be used to treat skin and mucosal lesions as the sole modality or as a preoperative modality (Fig. 7). In the latter, it should be done at least 6 weeks prior to surgery. This allows adequate post treatment fibrosis to develop.
Arteriovenous Malformations (AVMs) Once again, the objective of intervention should be to improve the quality of life. This may include improvement in appearance, prevention of hemorrhage, improvement of function, and prevention of complications. A multidisciplinary conference should,
Figure 7 A young adult with a venous malformation of her lower lip, before and after treatment with a Nd:YAG laser. (Color version of the figure is available online.)
M. Waner and T.M. O
48
Figure 8 A 45-year-old with an extensive arteriovenous malformation that was resected in stages. (Color version of the figure is available online.)
where possible, precede treatment. At this conference, a decision should be made concerning the treatment objective and the modalities. It may not be possible to treat the entire lesion without mutilating procedures. When faced with a large lesion, staged procedures may be necessary. Surgical resection of AVMs can be challenging. The surgeon must endeavor to differentiate between the primary nidus of the AVM and the secondary changes, although this might be difficult to ascertain. Differentiation may help to avoid mutilating surgery. Surgical removal of the nidus, and not the secondary change, is necessary. The secondary changes are due to the continuous shunting across the nidus. These consist of arterial hypertrophy, venous dilatation, and edema which typically surround the nidus. Often, the nidus can be quite small whereas the secondary changes may be extensive. Preoperative embolization as well as percutaneous sclerotherapy, completed about 24 hours preoperatively can facilitate surgical resection and reduce the risk of intraoperative hemorrhage. Staged surgical resection is acceptable (Figs. 8-10). Postoperatively, necrosis of part of the overlying skin flap is sometimes seen. This may be due to venous congestion or a steal syndrome.
Figure 9 An arteriovenous malformation of the patient’s upper lip before and after a staged excision. There is still AVM present but the patient no longer bleeds and his appearance has improved. From this point, further treatment would primarily consist of embolization and transcutaneous sclerotherapy. (Color version of the figure is available online.)
Lymphatic Malformations The treatment of lymphatic malformations is truly multidisciplinary. Clinicians have classified these lesions as being macrocystic, microcystic, or mixed on the basis of their response to the sclerosant, OK-432.7,8 In reality, most lesions are mixed and the majority of lesions should be treated both surgically and with sclerotherapy. Surgical resection is able to reduce the volume of the tumor as well as remove fibrosis that has resulted from sclerotherapy (Fig. 11). Once a reasonable contour has been achieved, the remaining disease can be sclerosed. As with the preceding categories, treatment should be planned in a multidisciplinary way.
Mucosal Lesions A site of predilection is the tongue. The typical presentation is tongue swelling with vesicle formation. The vesicles may be filled with lymph or mixed lymph and blood. These patients are almost all symptomatic. The vesicles leak blood or lymph fluid or both. The patient’s pillow is almost always stained. Occasionally, bleeding can be quite profuse. These patients also experience a burning sensation with the consumption of spicy foods and carbonated drinks. Macroglossia may also be present and may need to be addressed. The vesicles can be ablated with a CO2 laser (Fig. 12). Although this is rarely curative, it will significantly improve the patients’ quality of life. There may be periods of normal tongue mucosa which may last for months or even years.
Figure 10 A 50-year-old with an AVM of his right buttock, before and 1 year after surgery. (Color version of the figure is available online.)
The role of surgery in the management of congenital vascular anomalies
49
Figure 13 A child with macroglossia and glossoptosis before and after a tongue reduction. (Color version of the figure is available online.)
Conclusion Figure 11 An infant with an extensive lymphatic malformation in the floor of mouth and submaxillary triangle, before and after surgical debulking. The patient clearly has residual disease but further treatment would consist of sclerotherapy. (Color version of the figure is available online.)
Glossoptosis Glossoptosis is sometimes seen with involvement of the tongue or the floor of the mouth or both. It is imperative to determine the true cause of the protrusion because this would affect the treatment choice. Glossoptosis may be due to tongue enlargement resulting from involvement of the tongue, with lymphatic malformation leading to macroglossia. Alternatively, tongue protrusion may result from involvement of the floor of the mouth and a normal tongue size. This needs to be treated with sclerotherapy whereas macroglossia needs to be treated with tongue reduction surgery (Fig. 13).
In general, surgery plays a secondary role in the management of hemangiomas and vascular malformations. Most hemangiomas show spontaneous involution, and the larger or more symptomatic lesions can often be managed with pharmacologic agents such as propranolol. There remain a significant number of lesions which will require surgery due to their size, location, or interference with vital structures or functions, such as respiration, feeding, and vision. Still others may cause pain, ulceration, or such severe disfigurement that they cannot be managed conservatively. Vascular malformations may require surgical intervention when they cannot be managed with embolization or sclerotherapy. In high flow lesions, proximal ligation of feeding vessels is ineffective, and the goal must be removal of the nidus. Completely resectable lesions and those too bulky to respond to embolization are best suited
Klippel-Tre´naunay Syndrome The role of surgery is primarily to debulk the lesion and to remove as much of the angiokeratoma as possible. As most of the disease is superficial to the deep fascia and because muscle involvement is extremely rare, debulking is possible. This is usually done with preoperative sclerotherapy some 24 hours prior to surgery. The tissue heals well and the incidence of wound dehiscence is low. Angiokeratomatous tissue can be removed at the same time as debulking or separately. Staged procedures are common (Fig. 14).
Figure 12 A child with lymphatic malformation of the tongue before and after treatment with a CO2 laser. This patient is not ‘‘cured’’ but is symptom free for a period of time. (Color version of the figure is available online.)
Figure 14 A 1-year-old with Klippel-Tre´naunay syndrome before and after debulking of the lesion. Note that the leg is thinner. (Color version of the figure is available online.)
50 for surgery. Preoperative embolization or sclerotherapy is often employed to shrink the lesion and reduce intraoperative blood loss.
References 1. Le´aute´-Labreze C, Dumas de la Roque E, Hubiche T, et al: Propranolol for severe hemangiomas of infancy. N Engl J Med 358:2649-2651, 2008 2. Frieden IJ, Drolet BA: Propranolol for infantile hemangiomas: Promise, peril, pathogenesis. Pediatr Dermatol 26:642-644, 2009 3. Schiestl C, Neuhaus K, Zoller S, et al: Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas. Eur J Pediatr 170:493-501, 2011
M. Waner and T.M. O 4. Buckmiller LM, Munson PD, Dyamenahalli U, et al: Propranolol for infantile hemangiomas: Early experience at a tertiary vascular anomalies center. Laryngoscope 120:676-681, 2010 5. Naouri M, Schill T, Maruani A, et al: Successful treatment of ulcerated haemangioma with propranolol. J Eur Acad Dermatol Venereol 24:1109-1112, 2010 6. Arneja JS, Mulliken JB: Resection of amblyogenic periocular hemangiomas: Indications and outcomes. Plast Reconstr Surg 125:274-281, 2010 7. Alomari AI, Karian VE, Lord DJ, et al: Percutaneous sclerotherapy for lymphatic malformations: A retrospective analysis of patientevaluated improvement. J Vasc Interv Radiol 17:1639-1648, 2006 8. Greinwald JH Jr, Burke DK, Sato Y, et al: Treatment of lymphangiomas in children: An update of picibanil (OK-432) sclerotherapy. Otolaryngol Head Neck Surg 121:381-387, 1999