Plastic Surgery for Capillary Malformations

Plastic Surgery for Capillary Malformations

No Results

No Results

processing….

Vascular malformations have been recognized throughout history as birthmarks. The cause was thought to be maternal cravings for fruit or maternal dreams, moods, and fantasies. Although Blondel disproved these theories in 1727, some persist today. In 1866, Dugas conducted a scientific analysis of birthmarks and concluded that they were caused by defects in embryologic development. Later, Dupuytren classified vascular malformations as erectile tumors. Virchow and Wagner established the first classification system of vascular malformations, based on channel architecture and histomorphologic appearance. See the images below.

Vascular malformations have been categorized using confusing, inaccurate, and inconsistent terminology. A significant number of patients with vascular lesions receive ineffective and potentially harmful treatment based on misclassification. Mulliken classified vascular malformations using understandable and accurate nomenclature. Vascular malformations are subdivided into low-flow (capillary, venous, lymphatic, or a combination thereof) and high-flow (arterial) anomalies. Clinical, histological, histochemical, and biochemical differences and radiographic imaging findings support this classification.

The prevalence at birth of capillary malformations is reported at 0.3% without predisposition to either sex.

Capillary malformations were once referred to as port-wine stains; this term is now outdated. Genetic studies have mapped capillary malformations to chromosome 5q14-21, showing a defect in the RASA1 gene. The RASA1 gene encodes p120 Ras GTPase-activating protein. When mutated, p120 Ras GTPase-activating protein binds to Krev-1/rap1a, an integrin β1–mediated cell adhesion and angiogenesis protein. The pathogenesis of capillary malformations is not understood.

Capillary malformations are the most common cutaneous vascular malformation appearing as a macular stain. Capillary malformations are represented by ectatic capillaries and medium-sized venules with thin walls and flat endothelial cells. The lack of sympathetic innervation regulating blood flow in vessels with capillary malformation is believed to produce progressive ectasia. Consequently, a characteristic red and purple lesion is seen upon examination.

Unlike hemangiomas, capillary malformations do not undergo spontaneous involution. Further, the endothelium does not exhibit hyper-proliferation but has a normal turnover rate. In a study of 415 patients with capillary malformations in the fifth decade of life, Geronemus and Ashinoff found hypertrophy, nodules, or both in 65% of the capillary vasculature. [1] In addition to these features, bridged fenestrations can be seen by scanning electron microscopy. These are normally found in venous capillaries surrounding sweat glands and hair follicles and represent areas of accelerated exchange between circulation and surrounding tissue.

Controversy remains as to whether the thickening of postcapillary venules from basement membrane deposition actually occurs.

Patient history and physical examination findings are important in order to correctly classify these malformations. Appropriate treatment begins with the correct diagnosis.

The general consensus is that capillary malformations are located in the dermis. They present as well-defined patches of red or purple skin discoloration at birth but may be masked by erythema of neonatal skin. Their growth correlates closely with the person’s age, becoming increasingly nodular and covering larger areas with time.

Although capillary malformations can occur anywhere on the body, they are most commonly seen on the face. When capillary malformations occur on the face, they normally have a dermatomal distribution. Fifty-five percent of facial capillary malformations overlap sensory dermatomes, occur bilaterally, or cross the mid line. The remaining 45% occur in one of the three trigeminal dermatomes. Maxillary or mandibular overgrowth with labial hypertrophy and gingival hyperplasia may be seen in the lower and mid face. [2] Soft tissue and skeletal overgrowth may also be seen in cutaneous capillary malformations of the trunk and limbs.

Capillary malformations are commonly associated with developmental defects. For example, a capillary malformation on the posterior chest may be indicative of an underlying arteriovenous malformation (AVM) of the spinal cord (Cobb syndrome), or an occipital capillary malformation may overlie an encephalocele. Spinal dysraphism, lipomeningocele, tethered spinal cord, and diastematomyelia may be present when the capillary malformation overlies the cervical or lumbosacral spine. In these cases, careful neurologic examination, radiographic examination, spinal radiographic imaging, and bladder function studies are indicated because subtle signs of neurogenic bladder dysfunction or lower extremity weakness may be present.

Syndromes involving capillary malformations are discussed below.

Klippel-Trenaunay-Weber syndrome is often associated with the triad of capillary malformations, venous malformations or varicose veins, and hypertrophy of affected tissues. Vascular abnormalities lead to muscle hypertrophy and thickening of the skin, subcutaneous tissues, and bone. Although port-wine stains commonly occur cutaneously, venous and lymphatic malformations are common in the limbs. Most often, they affect a single lower extremity. A persistent lateral vein extending from the lateral malleolus to the gluteal region is common.

Parkes Weber syndrome is the combination of a capillary malformation and an AVM. It is commonly large and pink, with the affected limb being longer and warmer than the contralateral limb.

The hyperkeratotic cutaneous capillary-venous malformation is normally associated with vascular malformation of the brain and affects the tissue around the eye. It is an autosomal dominant abnormality due to a mutation in the KRIT1 gene.

Sturge-Weber syndrome is a capillary malformation in the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve of the face. This vascular malformation may be associated with many other symptoms, such as jacksonian seizures, mental retardation, calcification of the leptomeninges, glaucoma, and contralateral hemiplegia. [3]

Unlike hemangiomas, capillary malformations display ectatic vessels with flat nonproliferative endothelium. Thickening of the postcapillary venule wall is a common finding. In addition, bridged fenestrations can be seen by electron microscopy.

Geronemus RG, Ashinoff R. The medical necessity of evaluation and treatment of port-wine stains. J Dermatol Surg Oncol. 1991 Jan. 17(1):76-9. [Medline].

Lee JW, Chung HY, Cerrati EW, M O T, Waner M. The Natural History of Soft Tissue Hypertrophy, Bony Hypertrophy, and Nodule Formation in Patients With Untreated Head and Neck Capillary Malformations. Dermatol Surg. 2015 Nov. 41 (11):1241-5. [Medline].

Chen L, Wu J, Xu M, Chen N, Yang Y. Sturge-weber syndrome. Ann Dermatol. 2011 Nov. 23(4):551-3. [Medline]. [Full Text].

Arneja JS, Gosain AK. Vascular malformations. Plast Reconstr Surg. 2008 Apr. 121(4):195e-206e. [Medline].

Katugampola GA, Lanigan SW. Five years’ experience of treating port wine stains with the flashlamp-pumped pulsed dye laser. Br J Dermatol. 1997 Nov. 137(5):750-4. [Medline].

Tan OT, Sherwood K, Gilchrest BA. Treatment of children with port-wine stains using the flashlamp-pulsed tunable dye laser. N Engl J Med. 1989 Feb 16. 320(7):416-21. [Medline].

Bjerring P, Christiansen K, Troilius A. Intense pulsed light source for the treatment of dye laser resistant port-wine stains. J Cosmet Laser Ther. 2003 Apr. 5(1):7-13. [Medline].

Tremaine AM, Armstrong J, Huang YC, Elkeeb L, Ortiz A, Harris R, et al. Enhanced port-wine stain lightening achieved with combined treatment of selective photothermolysis and imiquimod. J Am Acad Dermatol. 2012 Jan 13. [Medline].

Rubin IK, Farinelli WA, Doukas A, Anderson RR. Optimal wavelengths for vein-selective photothermolysis. Lasers Surg Med. 2012 Jan 12. [Medline].

Kono T, Groff WF, Sakurai H. Treatment of port wine stains with the pulse dye laser. Ann Plast Surg. 2006 Apr. 56(4):460-3. [Medline].

McGill DJ, Mackay IR. Capillary vascular malformation response to increased ambient temperature is dependent upon anatomical location. Ann Plast Surg. 2007 Feb. 58(2):193-9. [Medline].

Raulin C, Greve B, Hammes S. Cold air in laser therapy: first experiences with a new cooling system. Lasers Surg Med. 2000. 27(5):404-10. [Medline].

Greve B, Hammes S, Raulin C. The effect of cold air cooling on 585 nm pulsed dye laser treatment of port-wine stains. Dermatol Surg. 2001 Jul. 27(7):633-6. [Medline].

Nelson JS, Kimel S. Safety of cryogen spray cooling during pulsed laser treatment of selected dermatoses. Lasers Surg Med. 2000. 26(1):2-3. [Medline].

Chang CJ, Nelson JS. Cryogen spray cooling and higher fluence pulsed dye laser treatment improve port-wine stain clearance while minimizing epidermal damage. Dermatol Surg. 1999 Oct. 25(10):767-72. [Medline].

Chess C. Does simultaneous contact cooling reduce intravascular temperature during laser irradiation and impinge on selective vascular destruction?. Dermatol Surg. 1998 Mar. 24(3):404-5. [Medline].

Waldorf HA, Alster TS, McMillan K, Kauvar AN, Geronemus RG, Nelson JS. Effect of dynamic cooling on 585-nm pulsed dye laser treatment of port-wine stain birthmarks. Dermatol Surg. 1997 Aug. 23(8):657-62. [Medline].

Lee DH, Pyon JK, Mun GH, Bang SI, Oh KS, Lim SY. Reconstruction of Head and Neck Capillary Malformations With Free Perforator Flaps for Aesthetic Purposes. Ann Plast Surg. 2014 Nov 12. [Medline].

Breugem CC, Hennekam RC, van Gemert MJ, van der Horst CM. Are capillary malformations neurovenular or purely neural?. Plast Reconstr Surg. 2005 Feb. 115(2):578-87. [Medline].

Astner S, Anderson RR. Treating vascular lesions. Dermatol Ther. 2005 May-Jun. 18(3):267-81. [Medline].

Barsky SH, Rosen S, Geer DE, Noe JM. The nature and evolution of port wine stains: a computer-assisted study. J Invest Dermatol. 1980 Mar. 74(3):154-7. [Medline].

Bauer BS, Kernahan DA, Hugo NE. Lymphangioma circumscriptum–a clinicopathological review. Ann Plast Surg. 1981 Oct. 7(4):318-26. [Medline].

Eerola I, Boon LM, Mulliken JB, Burrows PE, Dompmartin A, Watanabe S, et al. Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet. 2003 Dec. 73(6):1240-9. [Medline].

Enjolras O, Mulliken JB. The current management of vascular birthmarks. Pediatr Dermatol. 1993 Dec. 10(4):311-3. [Medline].

Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983 Dec. 18(6):894-900. [Medline].

Frech M, John J, Pizon V, Chardin P, Tavitian A, Clark R, et al. Inhibition of GTPase activating protein stimulation of Ras-p21 GTPase by the Krev-1 gene product. Science. 1990 Jul 13. 249(4965):169-71. [Medline].

Gampper TJ, Morgan RF. Vascular anomalies: hemangiomas. Plast Reconstr Surg. 2002 Aug. 110(2):572-85; quiz 586; discussion 587-8. [Medline].

Goldman MP, Fitzpatrick RE, Ruiz-Esparza J. Treatment of port-wine stains (capillary malformation) with the flashlamp-pumped pulsed dye laser. J Pediatr. 1993 Jan. 122(1):71-7. [Medline].

Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics. 1976 Aug. 58(2):218-22. [Medline].

Katugampola GA, Lanigan SW. Five years’ experience of treating port wine stains with the flashlamp-pumped pulsed dye laser. Br J Dermatol. 1997 Nov. 137(5):750-4. [Medline].

Lanigan SW. Port-wine stains unresponsive to pulsed dye laser: explanations and solutions. Br J Dermatol. 1998 Aug. 139(2):173-7. [Medline].

Marler JJ, Mulliken JB. Current management of hemangiomas and vascular malformations. Clin Plast Surg. 2005 Jan. 32(1):99-116, ix. [Medline].

Mulliken JB. Cutaneous vascular anomalies. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders; 1990:. 3191-274.

Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982 Mar. 69(3):412-22. [Medline].

Nelson JS, Applebaum J. Clinical management of port-wine stain in infants and young children using the flashlamp-pulsed dye laser. Clin Pediatr (Phila). 1990 Sep. 29(9):503-8; discussion 509. [Medline].

Orten SS, Waner M, Flock S, Roberson PK, Kincannon J. Port-wine stains. An assessment of 5 years of treatment. Arch Otolaryngol Head Neck Surg. 1996 Nov. 122(11):1174-9. [Medline].

Rak KM, Yakes WF, Ray RL, Dreisbach JN, Parker SH, Luethke JM, et al. MR imaging of symptomatic peripheral vascular malformations. AJR Am J Roentgenol. 1992 Jul. 159(1):107-12. [Medline].

Schneider BV, Mitsuhashi Y, Schnyder UW. Ultrastructural observations in port wine stains. Arch Dermatol Res. 1988. 280(6):338-45. [Medline].

Smoller BR, Rosen S. Port-wine stains. A disease of altered neural modulation of blood vessels?. Arch Dermatol. 1986 Feb. 122(2):177-9. [Medline].

Wang QK. Update on the molecular genetics of vascular anomalies. Lymphat Res Biol. 2005. 3(4):226-33. [Medline].

Ashok Tholpady, MD, MSc Assistant Professor, Section of Transfusion Medicine, Department of Pathology and Laboratory Medicine, University of Texas MD Anderson Cancer Center

Ashok Tholpady, MD, MSc is a member of the following medical societies: AABB, Association of Clinical Scientists, College of American Pathologists, Harris County Medical Society

Disclosure: Nothing to disclose.

Thomas J Gampper, MD, FACS Associate Professor of Plastic Surgery, Associate Clinical Professor of Neurosurgery, Director of Laser Surgery, Vice Chairman, Department of Plastic Surgery, University of Virginia School of Medicine; Director, Virginia Aesthetic Center

Thomas J Gampper, MD, FACS is a member of the following medical societies: American Cleft Palate-Craniofacial Association, American Society for Aesthetic Plastic Surgery, American Society of Plastic Surgeons, American Council of Academic Plastic Surgeons, American College of Surgeons, American Society for Laser Medicine and Surgery, Undersea and Hyperbaric Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Wayne Karl Stadelmann, MD Stadelmann Plastic Surgery, PC

Wayne Karl Stadelmann, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Society of Plastic Surgeons, New Hampshire Medical Society, Northeastern Society of Plastic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Gregory Gary Caputy, MD, PhD, FICS Wound Healing Consultant, Advantage Surgical and Wound Care

Gregory Gary Caputy, MD, PhD, FICS is a member of the following medical societies: American Society for Laser Medicine and Surgery, International College of Surgeons, International College of Surgeons US Section, Wound Healing Society

Disclosure: Nothing to disclose.

Shahin Javaheri, MD Chief, Department of Plastic Surgery, Martinez Veterans Affairs Outpatient Clinic; Consulting Staff, Advanced Aesthetic Plastic & Reconstructive Surgery

Shahin Javaheri, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Society of Plastic Surgeons

Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Emmanuella Joseph, MD, and William O Murtagh, MD, to the development and writing of this article.

Plastic Surgery for Capillary Malformations

Research & References of Plastic Surgery for Capillary Malformations|A&C Accounting And Tax Services
Source