Ординатура / Офтальмология / Английские материалы / Essentials in Ophthalmology Oculoplastics and Orbit_Guthoff, Katowitz_2007

sured that periodic changes in size and internal velocity are normal and do not, per se, form an indication for clinical intervention – the latter being restricted to reducing any factors leading to impairment of visual development.

Summary for the Clinician

■Serial ultrasonographic studies have identified a complex profile of proliferation and decay in pediatric capillary hemangioma, these two processes occurring as a dynamic equilibrium.

■The correlation in many cases between volume and velocity indicates a dynamic relationship between the hemangioma and its vascular supply, persisting for a number of years.

■About three-quarters of hemangiomas will increase in size during the first year of life, this change being up to four-fold in magnitude.

■The clinician may reassure the parents that, while visual development is normal, periodic changes in size and velocity are to be expected, and are not a reason for clinical intervention.

References

References 109

6.Burstein FD, Simms C, Cohen SR, et al. (2000) Intralesional laser therapy of extensive hemangiomas in 100 consecutive pediatric patients. Ann Plast Surg 44:188–194

7.Burton BK, Schulz CJ, Angle B, et al. (1995) An increased incidence of hemangiomas in infants born following chorionic villus sampling (CVS). Prenat Diagn 15:209–214

8.Castillo BV Jr, Kaufman L (2003) Pediatric tumors of the eye and orbit. Pediatr Clin North Am 50:149–172

9.Chang J, Most D, Bresnick S, et al. (1999) Proliferative haemangiomas: analysis of cytokine gene expression and angiogenesis. Plast Reconstr Surg 103:1–9

10.Chau A, Fung K, Yip L, et al. (2004) Orbital development in Hong Kong Chinese subjects. Ophthalmic Physiol Opt 24:436–439

11.Clark DE, Smith SK, He Y, et al. (1998) A vascular endothelial growth factor antagonist is produced by the human placenta and released into the maternal circulation. Biol Reprod 59:1540–1548

12.Coats DK, O’Neil JW, D’Elia VJ, et al. (2003) SubTenon’s infusion of steroids for treatment of orbital hemangiomas. Ophthalmology 110:1255–1259

13.Cogen MS, Elsas FJ (1989) Eyelid depigmentation following corticosteroid injection for infantile ocular adnexal hemangioma. J Pediatr Ophthalmol Strabismus 26:35–38

14.Dadras SS, North PE, Bertoncini J, et al. (2004) Infantile haemangiomas are arrested in an early developmental vascular differentiation state. Mod Pathol 17:1068–1079

cal excision of selected amblyogenic periorbital capillary hemangiomas. Ophthalmic Surg Lasers 30:754–757

(2006) Pulse methylprednisolone therapy for threatening periocular haemangiomas of infancy. Acta Derm Venereol 86:429–432

Ortone JP, et al. (eds) eMedicine dermatology. eMedicine Corp, St. Petersburg. Available at: http://www.emedicine.com/derm/topic201.htm

ear subcutaneous fat atrophy after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol 105:56–59

(2006) The pathogenesis of hemangiomas: a review. Plast Reconstr Surg 117:29e–35e

4.Bree AF, Siegfreid E, Sotelo-Avila C, et al. (2001) Infantile haemangiomas: speculation on placental trophoblastic origin. Arch Dermatol 137:573–577

5.Bruckner AL, Frieden IJ (2003) Hemangiomas of infancy. J Am Acad Dermatol 48:477–493

nosis and treatment of an ophthalmic artery occlusion during an intralesional injection of corticosteroid into an eyelid capillary hemangioma. Am J Ophthalmol 121:638–642

18.Egbert JE, Paul S, Engel WK, et al. (2001) High injection pressure during intralesional injection of corticosteroids into capillary hemangiomas. Arch Ophthalmol 119:677–683

110

6

44.Walker RS, Custer PL, Nerad JA (1994) Surgical excision of periorbital capillary hemangiomas. Ophthalmology 101:1333–1340

45.Waner M, Suen JY (1999) Treatment options for the management of hemangiomas. In: Waner M, Suen JY (eds) Hemangiomas and vascular malformations of the head and neck. Wiley-Liss, New York, pp 233–261

46.Wasserman BN, Medow NB, Homa-Palladino M, et al. (2004) Treatment of periocular capillary hemangiomas. J AAPOS 8:175–181

References 111

47.Yu Y, Varghese J, Brown LF, et al. (2001) Increased TIE2 expression, enhanced response to angiopoi- etin-1, and dysregulated angiopoietin-2 expression in hemangioma-derived endothelial cells. Am J Pathol 159:2271–2280

48.Yu Y, Fuhr J, Boye E, Gyorffy S, et al. (2006) Mesenchymal stem cells and adipogenesis in hemangioma involution. Stem Cells 24:1605–1612

infantile hemangiomas, which are typically small and absent at birth and ultimately involute, from vascular malformations which are present at birth and do not involute [28]. This classification, however, does not recognize the more recently described non-involuting form of congenital hemangioma [4].

The International Society for the Study of Vascular Anomalies (ISSVA) has subdivided vascular anomalies into tumors and malformations (Table 7.1) [10]. VLM fall into the latter category. Rootman et al. described orbital VLM clinically by their location in the orbit. They were categorized as superficial, deep, combined [32], and complex (associated with intracranial malformations) [22], and later as part of a hemodynamic spectrum of vascular malformations [31]. In 1999 the Orbital Society adopted this latter

Fig. 7.3  A 6-year-old boy with recurrent hemorrhage of orbital VLM (this patient had already undergone subtotal resection)

classification system in which VLM are typically associated with type 1 (no flow) [16].

There is support in the radiology literature for use of the term venous-lymphatic malformation, as opposed to lymphangioma [5]. This is really an extension of the classification by Wright et al. in which these lesions are considered to represent an abnormality of the orbital venous system [48]. Whether or not VLM and varices represent a spectrum of venous malformations, or are really separate entities has not as yet been settled [16]. VLM most commonly have distinctive cystic elements that predominate or are exclusive of any apparent solid components. However, there is a small sub-group of VLM that are predominantly solid and well-circumscribed. The latter group can be mistaken on both clinical and radiographic examination for capillary hemangio-

116

7

7.5 Treatment

7.5.1 Treatment Overview

Given the risk of hemorrhage and the challenges to surgical resection, the majority of authors recommend supportive, non-surgical management for most orbital and adnexal venous-lymphatic malformations [17].

7.5.2 Systemic Steroids

Systemic steroids have mixed results in the management of orbital VLM. A brief course of oral corticosteroids can be helpful in cases of acute hemorrhage or when a URI results in lesion expansion due to lymphoid hypertrophy. Corticosteroids can also be used prior to surgery in an effort to shrink the mass. However, no study has suggested that steroids alone can more completely reverse the proptosis resulting from enlargement of VLM than that occurring spontaneously [39, 42].

7.5.3 Surgery

Surgical resection of orbital VLM is challenging due to the lack of encapsulation, transgression of tissue planes, and propensity to hemorrhage both intraoperatively and in the postoperative period. Indications for surgery include compressive optic neuropathy, exposure keratopathy, and untreatable amblyopia. Surgical planning has to include a consideration of the location and the nature of the lesion. Cystic VLM are typically drained through an open orbitotomy to relieve the mass effect of the lesion (Fig. 7.1). One author has used intralesional fibrin glue (Tisseel) to aid dissection and achieve more complete tumor resection [7]. Since recurrence, regardless of the extent of the resection, is the norm, care should be given to avoid injury to vital structures in an effort to complete the resection. Alternatively, decompression of the hemorrhagic cyst may in some cases be achieved by image-guided needle aspiration. Orbital bone decompression, either alone or combined with tumor debulking, has also been used for proptosis reduction [19].

7.5  Treatment 117

Venous-lymphatic malformations that are radiographically more solid, featuring smaller channels, and producing compressive optic neuropathy as a consequence of apical location or large bulk are more amenable to surgical resection. These lesions are typically more adherent to the surrounding tissues and have a greater propensity to hemorrhage than the cystic variant. As a consequence complete removal may be impractical when attempting to preserve vision [23].

Subtotal resection to accomplish the surgical goals may be aided by use of the carbon dioxide laser [21]. Recently, Gündüz et al. reported 18 patients who underwent total or subtotal resection of orbital VLM. These authors found that the surgically well-delineated extraconal lymphangiomas can be resected totally without recurrence at short-term follow-up (average of 20.4 months). In this series, recurrence was noted in 2 out of 13 cases, with subtotal resection at a mean fol- low-up of 29.4 months. Both of these lesions had cystic components [15]. These findings are in contrast to Tunç et al., who reported a higher recurrence rate of 58.3% at a mean follow-up of 9.2 years in their series of 26 patients. These findings highlight the need to follow patients over an extended period of time and the potentially significant chance of recurrence [43].

7.5.4 Laser Treatment

The CO2 laser has been used for subtotal excision of VLM. Advantages of this instrument include good hemostasis from tissue vaporization and less surgical trauma to surrounding tissue. This laser has also been anecdotally effective with anterior lesions, specifically with conjunctival involvement. Complications from the CO2 laser include corneal anesthesia, papillary dilation from ciliary nerve damage, and symblepharon formation [21, 24, 45].

7.5.5 Sclerotherapy

The goal of any sclerosing agent is to close a vessel lumen, either through direct vessel embolization or through incited inflammatory reactions, which lead to gross vessel wall destruction and

118

7

Another concern with any sclerosing agent injected into the orbit is the risk of distant embolization. While this has not been reported as a complication in the small series of patients treated with orbital injections reported thus far, there have been, however, reports of this problem occurring in animal models following injection for esophageal varices [8, 12, 40]. Such concerns should raise a red flag of caution, particularly for the patient with a posterior tumor, and further underscores the need for better radiographic studies to identify feeder vessels with this type of VLM.

7.5.5.3 Other Agents

Alcohol, in varying concentrations, has long been established as a sclerosant in VLM. Ethanol causes endothelial denudation and leads to immediate thrombosis at the site of the injection. If used in the orbit, this carries a significant risk of thrombosis for the ophthalmic vein [38]. Ethibloc contains 60% alcohol and when in contact with blood creates an embolism that induces thrombosis and fibrosis [9]. Sodium tetradecyl sulfate (Sotradecol) containing 2% alcohol has also been used in orbital lymphangioma, but with only moderate success [47]. Other available agents, though not reported for orbital injection of lymphangioma, have included bleomycin [3] and acetic acid [46].

7.5.5.4Image-guided Sclerotherapy

One of the greatest risks to orbital sclerotherapy is ophthalmic vein thrombosis, which can lead to an orbital compartment syndrome, vision loss, and cavernous sinus thrombosis. To properly treat orbital VLM, it is critical to first establish a pre-procedural characterization of the lesion, both to plan the approach and to identify venous drainage pathways. Next, needle guidance with X-ray fluoroscopy, MRI, or CT is an important aid to achieving a more accurate injection technique. Lastly, real-time monitoring of the sclerosing agent is useful for identifying the rate and the distribution of the agent in the orbit [11, 25].

References 119

Summary for the Clinician

■Venous-lymphatic malformations are congenital anomalies of the orbit that should be differentiated from tumors such as hemangioma.

■VLM are often referred to as lymphangioma; however, the term VLM more accurately describes the origin and natural history of this type of lesion.

■VLM can be mostly solid, and thus more amenable to surgical resection, or have large cystic components, which can be drained.

■Given the high rate of recurrence and spontaneous hemorrhage any intervention must be carefully plotted.

■Sclerotherapy remains a viable treatment modality that needs further investigation.

7.6 Future Treatment Modalities

Recent histologic evidence obtained from the examination of persistent VLM lesions suggests a possible role in future treatment [37]. Sidle et al. report higher expression of angiogenic factors in what they defined as recurrent tumors compared with nonrecurrent specimens. Specifically, specimens identified as recurrent lymphangioma stained more avidly with pigment epitheliumderived factor (PEDF) and vascular endothelial growth factor (VEGF) [37]. This study suggests the potential for the use of anti-VEGF agents as an alternative to sclerotherapy in surgically debulked VLM.

References

1.Al-Adnani M, Williams S, Rampling D, et al. (2006) Histopathological reporting of paediatric cutaneous vascular anomalies in relation to proposed multidisciplinary classification system. J Clin Pathol 59:1278–1282

2.Banieghbal B, Davies MR (2003) Guidelines for the successful treatment of lymphangioma with OK-432. Eur J Pediatr Surg 13:103–107