Dissection of
Surgical
Capillary Hemangiomas
An Alternative to Intralesional Corticosteroids Robin M.
Deans, MD; Gerald J. Harris, MD; Jane
D.
Kivlin, MD
Intralesional corticosteroids are ofreducing the size of functionally significant capillary hemangiomas. While they may have a better benefit-risk ratio than some earlier treatment methods, a number of serious complications have been reported recently. We believe these resulted from the hemodynamic continuity of capillary hemangiomas with the orbital and systemic circulation and that intralesional injections are intravascular. Surgery has generally been
avoided because capillary hemangiomas are not encapsulated and piecemeal resection can produce significant bleeding. In carefully selected patients, we have used a surgical approach that involves dissection on the tumor's surface without entering its substance. Hemangiomas were removed en bloc from five patients with insignificant blood loss and excellent anatomic restoration.
í~i apillary hemangioma is the most com^ mon periocular vascular tumor in in¬ fancy and childhood. Presentation is usu¬ ally before 2 months of age, and most le¬ sions reach their maximum size by 6 to 12 months.1·2 Spontaneous involution fre¬ quently occurs by 4 to 7 years of age, but
the tumor's presence during the vulner¬ able period of ocular development may lead to visual complications. Deprivation
\s=b\
ten successful in
complication.69 These concerns subse¬ quently led to local, intralesional injections.10"13
Ease of administration, general suc¬ in reducing the size of hemangio¬ mas, and relieving visual compromise have contributed to the popularity of intra- lesionai steroids. The technique's simplicity may have even liberalized the treatment indications. However, as the number of treated children has in¬ creased, various complications have emerged. Most have been local prob¬ lems, such as crystalline deposits, depigmentation, subcutaneous fat atrophy, and eyelid necrosis.1418 Of greater con¬ cern, ipsilateral and contralateral vision loss have been reported.19·20 Short-term adrenal suppression and sustained de¬ rangement with significant growth re¬ tardation have also occurred.21 Surgical options for capillary he¬ mangioma have also been reported.22·23 These have generally been avoided by ophthalmologists because the unencapsulated tumors do not shell-out easily, their high-flow vascularity causes concern about hemorrhage in infants with small blood volumes, and inadequate results or unacceptable scarring can follow piecemeal surgery. Incisions into these highly vascular lecess
(Arch Ophthalmol. 1992;110:1743-1747)
anisometropic amblyopia can occur eyelid tumors. Orbital lesions may cause proptosis with corneal exposure, strabismus and amblyopia, optic atro¬ phy, and bony malformation.3·4 Children without visual compromise have generally required only careful ob¬ or
with
Accepted
for publication July 13, 1992. From the Sections of Ophthalmic Plastic and Reconstructive Surgery (Drs Deans and Harris) and Pediatric Ophthalmology (Dr Kivlin), Department of Ophthalmology, Medical College of Wisconsin, Milwaukee. Read in part at the Annual Spring Meeting of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Chatham, Mass, June 17, 1991. Reprint requests to Eye Institute, Medical College of Wisconsin, 8700 W Wisconsin Ave, Milwaukee, WI 53226 (Dr Harris).
servation. When intervention has been necessary, various methods have been used in the past. Significant scarring or collateral damage occurred with many
techniques, including sclerosing agents, cryotherapy, and high-dose radiation therapy.5 Oral prednisolone and pred¬ nisone
found to be
were
growth suppression
effective, but
was
a
potential
Clinical Data in Five Patients With Surgically Dissected Capillary Hemangiomas Preoperative Patient/Sex/ Age at
Surgery
1/M/8
Visual
Acuity No fixation
mo
preference 2/F/2 y 5
3/F/9
mo
L fixation
preference
No fixation
mo
preference 20/20 ' 20/25*
4/F/7 y
Cycloplegic Retinoscopy +1.50 +0.75+2.75x20°
-11.50+8.00x160° 1-1.50
+3.00+0.50x90° +3.00+0.25x90° Plano +0.75x90° Plano +0.50x90°
Postoperative
Site of Lesion L
temporal
upper
Previous
Visual
Therapy
Acuity
None
orbit R nasal upper lid, anterior orbit
R Inferior orbit
No fixation
preference
lid, anterior
Intralesional triamcinolone acetonide and betamethasone x2 (age 1 y), no effect None
20/60 20/30*
No fixation
preference L
temporal lid
upper
Intralesional triamcinolone acetonide and
Cycloplegic Retinoscopy +1.00 f 1.00+0.50x35°
-10.00+4.50x40°
Age at Last Examination 1 y 11 mo 3 y4
mo
+ 1.00+0.50x60°
1 y 7 mo
20/20 20/25
7 y 1 mo
No fixation
1 y 10 mo
betamethasone x2 (age 18
mo), partial
5/F/1 y 3
mo
No fixation
preference
R
superotemporal
effect None
orbital rim, brow
»Snellen.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
preference
1.—Case 1. Top left, An 8-month-old boy with eyelid and anterior orbital mass causing significant astigmatism. Top right, Surgical incision placed in the natural upper eyelid crease. Bottom left, The lesion has been removed by dissection of the multiple lobules from pre-aponeurotic fat, levator muscle, and lacrimal gland. Incisions into and between tumor lobules were avoided to minimize bleeding. Bottom right, Appearance 3 months after surgery. Examination 1 year later showed relief of most of the astigmatism and symmetrical vision.
Fig
was
Fig 2.—Left, Low-power view demonstrates the multilobulated architecture of a capillary hemangioma. In the absence of a capsule, the plane of surgical dissection should be directly on the surface of the most peripheral lobules (hematoxylin-eosin, original magnification x38). Right, The lesion consists of endothelial cell clusters that define blood-filled channels. Hemodynamic continuity with normal blood vessels can permit anterograde and retrograde dissemination of injected material (hematoxylin-eosin, original magnification X240). sions produce brisk and diffuse bleed¬ ing, and heavy treatment with monopolar cautery leads to significant scar¬
ring.
In five selected patients, we have used a surgical approach that involves careful dissection of the tumors from surrounding tissues, without incisions
into the lesions themselves. Surgery was performed by one of us (G.J.H.). PATIENTS AND METHODS Patient Selection
The anterior aspect of the hemangioma must be deep enough that a surgical plane
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
can be developed without devascularizing the more superficial tissues. Lesions that extend into the dermis—those that are bright red and do not allow movement of the overlying skin—are not considered for this approach. At the minimum, an intact layer of subcutaneous tissue should be present. In the eyelids, without a substantial sub¬ cutaneous layer, intact orbicularis muscle-
ules that extend between normal orbital structures. There are vascular connections between these lobules, and efforts are made to remove all portions in continuity to minimize bleeding. Depending on the overall volume and surface area of the tu¬ mor, complete removal may require 2 or 3 hours of operative time.
RESULTS
The clinical data and surgical results presented in the Table and Figs 1 through 4. The surgical indications were significant anisometropia in pa¬ tients 1 and 2, marked orbital asymme¬ try in patient 3, and failure of the eye¬ lid tumor to involute by age 7 years in patient 4. Patient 5 was a 15-month-old black child with a progressively enlarg¬ ing deep mass over the superotemporal orbital rim. There was no discoloration suggestive of a vascular lesion, and surgical exploration was performed for diagnosis. The tumor was completely dissected to avoid incisional hemor¬ rhage. There was no astigmatism or amblyopia in patients 3 through 5. Preoperative and final ocular align¬ ment and motility were normal in all five patients. None had postoperative edema sufficient to obstruct the visual axis, and none were patched for more than a few hours after surgery. are
2. Top, A 2-year-old girl who had been treated twice with intralesional cor¬ ticosteroids 1 year before referral. There had been no shrinkage of the mass, and marked anisometropia remained. Center, Capillary hemangioma was dissected intact. Scarring between dermis and tumor was noted at the sites of previous injection. Bottom, Three months after surgery. Residual anisometro¬ pia may have been due to relatively late intervention.
Fig 3.—Case
anterior to the tumor is preferable. While there are no theoretical limits to the size of tumors that can be managed by en bloc removal, the time-consuming techniques im¬ pose some practical limitations.
Surgical Technique Skin incisions are made within the natural upper or lower eyelid creases. Sharp dissec¬ tion continues through orbicularis muscle, parallel to its fibers, and then behind muscle, superiorly or inferiorly, until over the tumor. Dissection proceeds on the lesion's surface without entering its substance. The most peripheral lobules are dissected slowly and carefully from surrounding normal struc¬ tures, with small snips of a sharp Westcott scissors and intermittent, prophylactic pin¬ point bipolar cautery. Major and minor feed¬ ing arteries and draining veins are thus obliterated before they are severed, and cautery damage to surrounding normal structures is negligible. Incisions into the tumor itself are avoided throughout the pro¬ cedure, since the lesion remains perfused by intact vessels at undissected poles. While some lesions appear well circumscribed on imaging studies, surgical dissection often demonstrates an irregular pattern of lob-
COMMENT
The local complications of cortico¬ steroid injection of periocular hemangi¬ omas may be exaggerations of the desired therapeutic response. The mechanism of action of both systemic and local therapy remains unclear, but sensitization of terminal vascular beds to circulating vasoconstrictors—with resultant narrowing, thrombosis, and involution—has been implicated.24·25 In¬ tralesional administration might also be effective because of embolization of the vascular channels of a hemangioma by the larger particles in corticosteroid suspensions. The particle size in these preparations ranges from 10 to 200 µ , while a red blood cell has a diameter of 6 µ .15·26 If the particles embolize the blood supply of contiguous normal tis¬ sue, the result may be subcutaneous fat atrophy or eyelid necrosis. Other complications of intralesional corticosteroids may be related to more distant vascular connections. Capillary hemangiomas consist of proliferating endothelial cells that circumscribe small blood-filled channels (Fig 2, right). While there may be some variation in degree, these vascular beds are hemodynamically continuous with the normal orbital vessels. Doppler studies,27 contrast-enhanced computed tomogra¬ phy,4 and arteriography28·29—as well as
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
surgical findings—all demonstrate high-flow perfusion of capillary he¬ mangiomas. In this respect, the tumors differ from the lymphangiomas30 that also occur as congenital vascular lesions and from the cavernous hemangiomas31 the the
that appear in adulthood. Because capillary hemangiomas are continuous with the orbital and sys¬ temic vasculature, all intralesional in¬ jections might be thought of as intra¬ vascular. Direct or anterograde flow of the smaller particles into the venous system could explain the transient and long-term adrenal suppression that has been documented with careful monitor¬ ing.21 Retrograde (intra-arterial) flow can lead to the retinal arterioles.19·20 While the frequency of reported com¬ plications of intralesional corticoster¬ oids has been low, their gravity must be considered. In a real sense, the treat¬ ment is a form of systemic administra¬ tion, and some authors favor the oral route over intralesional injections when comparing the two.32 We believe that careful surgery may offer the best overall benefit-risk ratio for selected patients. This belief re¬ quires clear distinction between piece¬ meal resection, which leads to brisk hemorrhage, and meticulous dissection of the tumor from surrounding normal tissues. Because there is no capsule, dissection must proceed slowly on the surface lobules (Fig 2, left), with pre¬ emptive pin-point bipolar cautery of scores of feeding and draining vessels. Normal anatomy has been surpris¬ ingly well restored after removal of the lesions, and it has not been necessary to re-create eyelid creases or to alter eye¬ lid levels secondarily. The relief of in¬ duced astigmatism may be related to surgical timing (cases 1 and 2). Later intervention may be less successful be¬ cause of permanent molding of the globe as scierai rigidity increases with age.33 Surgical dissection is not appropriate for all capillary hemangiomas. Lesions that extend into the dermis—those that are bright red and do not allow move¬ ment of the overlying skin—should not be approached in this manner. These superficial tumors may be amenable to transcutaneous treatment with argon or tunable dye lasers, exploiting the se¬ lective absorption of the lasers' energy. The benefit-risk ratio for relatively flat, superficial lesions is favorable.34 The neodymium-YAG laser is more effec¬ tive than the argon for deep or bulky tumors, but complications can be se¬ vere. The carbon dioxide laser requires surgical dissection for exposure and of¬ fers no tumor-specific absorption. Surgical dissection is also not appro¬ priate for massive, diffuse facial lesions.
Fig 4.—Case 3. Top left, A 9-month-old girl with progressive globe displacement. Top right, Tumor-induced expansion of the right bony orbit caused concern about permanent asymmetry. The lesion was isodense with the extraocular muscles. Bottom left, Despite the roentgenographic characteristics, the tumor could be sharply dissected from normal structures. Surgery had been performed through a lower eyelid crease in¬ cision (straight arrow), and a small drain (curved arrow) was maintained overnight. Bottom right, Three months after surgery, without functional or cosmetic residua. In these unfortunate patients, any ef¬ fective therapy carries some risk. We favor low-dose oral prednisone (1 to 4 mg/kg per day)35 as the initial treat¬ ment, administered under the careful supervision of a pediatrician. If local injections are to be used in any patients, it is possible that perilesional—rather than intralesional—
administration might limit intravascu¬ lar dissemination. While many periocular hemangiomas require no treatment, those that do present a therapeutic challenge. Efforts may ultimately be directed at those en¬ dogenous factors that stimulate their growth or inhibit their involution, as suggested by the recent use of inter-
feron alfa.36 At present, we believe that surgical dissection offers acceptably low risks compared with current treat¬ ment options, when performed care¬ fully in selected patients. This investigation was supported in part by an unre¬ stricted grant from Research to Prevent Blindness Ine, New York, NY, and by core grant EY01931 from the National Eye Institute, Bethesda, Md.
References 1. Margileth AM, Museles M. Cutaneous hemangiomas in children. JAMA. 1965;194:523-526. 2. Bowers RE, Graham EA, Tomlinson KM. The natural history of the strawberry nevus. Arch
Dermatol. 1960;82:667-680. 3. Stigmar G, Crawford JS, Ward CM, Thomson HG. Ophthalmic sequelae of infantile hemangiomas of the eyelids and orbit. Am J Ophthalmol. 1978; 85:806-813. 4. Haik BG, Jakobiec FA, Ellsworth RM, Jones IS. Capillary hemangioma of the eyelids and orbit: an analysis of the clinical features and therapeutic results in 101 cases. Ophthalmology. 1979;86:760\x=req-\ 789. 5. Henderson JW. Orbital Tumors. 2nd ed. New York, NY: Brian C Decker; 1980;117-128.
6. Zarem HA, Edgerton MT. Induced resolution of cavernous hemangiomas following prednisolone therapy. Plast Reconstr Surg. 1967;39:76-83. 7. Fost NC, Esterly NB. Successful treatment of juvenile hemangiomas with prednisone. J Pediatr.
1968;72:351-357.
G, Lobeck CC. Successful treathemangioma with prednisone. Arch
8. deVenecia
ment of eyelid
Ophthalmol. 1970;84:98-102. 9. Hiles DA, Pilchard WA. Corticosteroid control of neonatal hemangiomas of the orbit and ocular adnexa. Am J Ophthalmol. 1971;71:1003\x=req-\
1008. 10. Mazzola RF. Treatment of haemangiomas in children by intralesional injection of steroids. Chir Plast (Berlin). 1978;4:161-171.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
11. Kushner BJ. Local steroid therapy in adnexal hemangioma. Ann Ophthalmol. 1979;11: 1005-1009. 12. Zak TA, Morin JD. Early local steroid therapy of infantile eyelid hemangiomas. J Pediatr Ophthalmol Strabismus. 1981;18:25-27. 13. Brown BZ, Huffaker G. Local injection of steroids for juvenile hemangiomas which disturb the visual axis. Ophthalmic Surg. 1982;13:630\x=req-\ 636. 14. Dyment PG. Local atrophy following triamcinolone injection. Pediatrics. 1970;46:136-137. 15. Sutula FC, Glover AT. Eyelid necrosis following intralesional corticosteroid injection for
capillary hemangioma. Ophthalmic Surg. 1987;18: 103-105.
16. Droste PJ, Ellis FD, Sondhi N, Helveston EM. Linear subcutaneous fat atrophy after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol. 1988;105:65-69. 17. Townshend LM, Buckley EG. Linear subcutaneous fat atrophy after a single corticosteroid injection for ocular adnexal hemangioma. Am J Ophthalmol. 1990;109:102-103. 18. Cogen MS, Elsas FJ. Eyelid depigmentation following corticosteroid injection for infantile ocular adnexal hemangioma. J Pediatr Ophthalmol Strabismus. 1989;26:35-38. 19. Shorr N, Seiff SR. Central retinal artery occlusion associated with periocular corticosteroid injection for juvenile hemangioma. Ophthalmic
Surg. 1986;17:229-231. 20. Ruttum MS, Abrams GW,
Harris GJ, Ellis MK. Bilateral retinal embolization associated with intralesional corticosteroid injection for capillary hemangioma of infancy. J Pediatr Ophthalmol Strabismus. In press. 21. Weiss AH. Adrenal suppression after corticosteroid injection of periocular hemangiomas. Am J Ophthalmol. 1989;107:518-522. 22. Thomson HG, Ward CM, Crawford JS, Stigmar G. Hemangiomas of the eyelid: visual complications and prophylactic concepts. Plast Reconstr
Surg. 1979;63:641-647.
23. Pasyk KA, Dingman RO, Argenta LC, Sandall GS. The management of hemangiomas of the eyelid and orbit. Head Neck Surg. 1984;6: 851-857. 24. Zweifach BW, Shorr E, Black MM. The influence of the adrenal cortex on behavior of the terminal vascular bed. Ann N Y Acad Sci. 1953;56: 626-642. 25. Wyman LC, Fulton GP, Shulman MH. Direct observations on the circulation in the hamster cheek pouch in adrenal insufficiency and experimental hypercorticalism. Ann N Y Acad Sci. 1953; 56:643-656. 26. Wilson RS, McGrew RN, White HJ. Bilateral retinal artery and choriocapillaris occlusion following the injection of long-acting corticosteroid suspensions in combination with other drugs, II: animal experimental studies. Ophthalmology. 1978;85: 975-985. 27. Bingham HG. Predicting the course of a congenital hemangioma. Plast Reconstr Surg. 1979;63: 161-166. 28. Aron-Rosa D, Doyon D, Dassonville J. Angiography in vascular malformations of the orbit. Modern Probl Ophthalmol. 1975;14:146-155. 29. Dilenge D. Arteriography in angiomas of the orbit. Radiology. 1974;113:355-361. 30. Harris GJ, Sakol PJ, Bonavolont\l=a`\ G,
Currently
analysis of 30 cases of orbital lymphangioma: pathophysiological considerations DeConciliis C. An
and management recommendations. Ophthalmology. 1990;97:1583-1592. 31. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: a clinicopathologic analysis of 66 cases. In: Jakobiec FA, ed. Ocular and Adnexal Tumors. New York, NY: Aesculapius Publishers Inc; 1978:741-781. 32. Edgerton MT. Discussion: the treatment of periorbital infantile hemangiomas with intralesional corticosteroid. Plast Reconstr Surg. 1985; 76:525-526. 33. Bogan S, Simon JW, Krohel GB, Nelson LB. Astigmatism associated with adnexal masses in infancy. Arch Ophthalmol. 1987;105:1368-1370. 34. Achauer BM, Vander Kam VM. Capillary hemangioma (strawberry mark) of infancy: comparison of argon and Nd:YAG laser treatment. Plast Reconstr Surg. 1989;84:60-70. 35. Esterly NB, Margileth AM, Kahn G, et al. The management of disseminated eruptive hemangiomata in infants. Pediatr Dermatol. 1989;1:312\x=req-\ 317. 36. Orchard PJ, Smith CM, Woods WG, Day DL, Dehner LP, Shapiro R. Treatment of haemangioendotheliomas with alpha interferon. Lancet. 1989;2: 565-567.
in Other AMA Journals
AMERICAN JOURNAL OF DISEASES OF CHILDREN
Hemorrhages in Newborn Piglets Following Cardiopulmonary Resuscitation James C. Fackler, MD; Ivor D. Berkowitz, MD; W. Richard Green, Retinal
(AJDC. 1992;146:1294-1296)
Are We Educating a Medical Professional Who Cares? Robert G. Petersdorf, MD, Kathleen Turner, MPA
(AJDC. 1992;146:1338-1341)
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
MD
Surgical
Capillary Hemangiomas
An Alternative to Intralesional Corticosteroids Robin M.
Deans, MD; Gerald J. Harris, MD; Jane
D.
Kivlin, MD
Intralesional corticosteroids are ofreducing the size of functionally significant capillary hemangiomas. While they may have a better benefit-risk ratio than some earlier treatment methods, a number of serious complications have been reported recently. We believe these resulted from the hemodynamic continuity of capillary hemangiomas with the orbital and systemic circulation and that intralesional injections are intravascular. Surgery has generally been
avoided because capillary hemangiomas are not encapsulated and piecemeal resection can produce significant bleeding. In carefully selected patients, we have used a surgical approach that involves dissection on the tumor's surface without entering its substance. Hemangiomas were removed en bloc from five patients with insignificant blood loss and excellent anatomic restoration.
í~i apillary hemangioma is the most com^ mon periocular vascular tumor in in¬ fancy and childhood. Presentation is usu¬ ally before 2 months of age, and most le¬ sions reach their maximum size by 6 to 12 months.1·2 Spontaneous involution fre¬ quently occurs by 4 to 7 years of age, but
the tumor's presence during the vulner¬ able period of ocular development may lead to visual complications. Deprivation
\s=b\
ten successful in
complication.69 These concerns subse¬ quently led to local, intralesional injections.10"13
Ease of administration, general suc¬ in reducing the size of hemangio¬ mas, and relieving visual compromise have contributed to the popularity of intra- lesionai steroids. The technique's simplicity may have even liberalized the treatment indications. However, as the number of treated children has in¬ creased, various complications have emerged. Most have been local prob¬ lems, such as crystalline deposits, depigmentation, subcutaneous fat atrophy, and eyelid necrosis.1418 Of greater con¬ cern, ipsilateral and contralateral vision loss have been reported.19·20 Short-term adrenal suppression and sustained de¬ rangement with significant growth re¬ tardation have also occurred.21 Surgical options for capillary he¬ mangioma have also been reported.22·23 These have generally been avoided by ophthalmologists because the unencapsulated tumors do not shell-out easily, their high-flow vascularity causes concern about hemorrhage in infants with small blood volumes, and inadequate results or unacceptable scarring can follow piecemeal surgery. Incisions into these highly vascular lecess
(Arch Ophthalmol. 1992;110:1743-1747)
anisometropic amblyopia can occur eyelid tumors. Orbital lesions may cause proptosis with corneal exposure, strabismus and amblyopia, optic atro¬ phy, and bony malformation.3·4 Children without visual compromise have generally required only careful ob¬ or
with
Accepted
for publication July 13, 1992. From the Sections of Ophthalmic Plastic and Reconstructive Surgery (Drs Deans and Harris) and Pediatric Ophthalmology (Dr Kivlin), Department of Ophthalmology, Medical College of Wisconsin, Milwaukee. Read in part at the Annual Spring Meeting of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Chatham, Mass, June 17, 1991. Reprint requests to Eye Institute, Medical College of Wisconsin, 8700 W Wisconsin Ave, Milwaukee, WI 53226 (Dr Harris).
servation. When intervention has been necessary, various methods have been used in the past. Significant scarring or collateral damage occurred with many
techniques, including sclerosing agents, cryotherapy, and high-dose radiation therapy.5 Oral prednisolone and pred¬ nisone
found to be
were
growth suppression
effective, but
was
a
potential
Clinical Data in Five Patients With Surgically Dissected Capillary Hemangiomas Preoperative Patient/Sex/ Age at
Surgery
1/M/8
Visual
Acuity No fixation
mo
preference 2/F/2 y 5
3/F/9
mo
L fixation
preference
No fixation
mo
preference 20/20 ' 20/25*
4/F/7 y
Cycloplegic Retinoscopy +1.50 +0.75+2.75x20°
-11.50+8.00x160° 1-1.50
+3.00+0.50x90° +3.00+0.25x90° Plano +0.75x90° Plano +0.50x90°
Postoperative
Site of Lesion L
temporal
upper
Previous
Visual
Therapy
Acuity
None
orbit R nasal upper lid, anterior orbit
R Inferior orbit
No fixation
preference
lid, anterior
Intralesional triamcinolone acetonide and betamethasone x2 (age 1 y), no effect None
20/60 20/30*
No fixation
preference L
temporal lid
upper
Intralesional triamcinolone acetonide and
Cycloplegic Retinoscopy +1.00 f 1.00+0.50x35°
-10.00+4.50x40°
Age at Last Examination 1 y 11 mo 3 y4
mo
+ 1.00+0.50x60°
1 y 7 mo
20/20 20/25
7 y 1 mo
No fixation
1 y 10 mo
betamethasone x2 (age 18
mo), partial
5/F/1 y 3
mo
No fixation
preference
R
superotemporal
effect None
orbital rim, brow
»Snellen.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
preference
1.—Case 1. Top left, An 8-month-old boy with eyelid and anterior orbital mass causing significant astigmatism. Top right, Surgical incision placed in the natural upper eyelid crease. Bottom left, The lesion has been removed by dissection of the multiple lobules from pre-aponeurotic fat, levator muscle, and lacrimal gland. Incisions into and between tumor lobules were avoided to minimize bleeding. Bottom right, Appearance 3 months after surgery. Examination 1 year later showed relief of most of the astigmatism and symmetrical vision.
Fig
was
Fig 2.—Left, Low-power view demonstrates the multilobulated architecture of a capillary hemangioma. In the absence of a capsule, the plane of surgical dissection should be directly on the surface of the most peripheral lobules (hematoxylin-eosin, original magnification x38). Right, The lesion consists of endothelial cell clusters that define blood-filled channels. Hemodynamic continuity with normal blood vessels can permit anterograde and retrograde dissemination of injected material (hematoxylin-eosin, original magnification X240). sions produce brisk and diffuse bleed¬ ing, and heavy treatment with monopolar cautery leads to significant scar¬
ring.
In five selected patients, we have used a surgical approach that involves careful dissection of the tumors from surrounding tissues, without incisions
into the lesions themselves. Surgery was performed by one of us (G.J.H.). PATIENTS AND METHODS Patient Selection
The anterior aspect of the hemangioma must be deep enough that a surgical plane
Downloaded From: http://archopht.jamanetwork.com/ by a University of Iowa User on 06/06/2015
can be developed without devascularizing the more superficial tissues. Lesions that extend into the dermis—those that are bright red and do not allow movement of the overlying skin—are not considered for this approach. At the minimum, an intact layer of subcutaneous tissue should be present. In the eyelids, without a substantial sub¬ cutaneous layer, intact orbicularis muscle-
ules that extend between normal orbital structures. There are vascular connections between these lobules, and efforts are made to remove all portions in continuity to minimize bleeding. Depending on the overall volume and surface area of the tu¬ mor, complete removal may require 2 or 3 hours of operative time.
RESULTS
The clinical data and surgical results presented in the Table and Figs 1 through 4. The surgical indications were significant anisometropia in pa¬ tients 1 and 2, marked orbital asymme¬ try in patient 3, and failure of the eye¬ lid tumor to involute by age 7 years in patient 4. Patient 5 was a 15-month-old black child with a progressively enlarg¬ ing deep mass over the superotemporal orbital rim. There was no discoloration suggestive of a vascular lesion, and surgical exploration was performed for diagnosis. The tumor was completely dissected to avoid incisional hemor¬ rhage. There was no astigmatism or amblyopia in patients 3 through 5. Preoperative and final ocular align¬ ment and motility were normal in all five patients. None had postoperative edema sufficient to obstruct the visual axis, and none were patched for more than a few hours after surgery. are
2. Top, A 2-year-old girl who had been treated twice with intralesional cor¬ ticosteroids 1 year before referral. There had been no shrinkage of the mass, and marked anisometropia remained. Center, Capillary hemangioma was dissected intact. Scarring between dermis and tumor was noted at the sites of previous injection. Bottom, Three months after surgery. Residual anisometro¬ pia may have been due to relatively late intervention.
Fig 3.—Case
anterior to the tumor is preferable. While there are no theoretical limits to the size of tumors that can be managed by en bloc removal, the time-consuming techniques im¬ pose some practical limitations.
Surgical Technique Skin incisions are made within the natural upper or lower eyelid creases. Sharp dissec¬ tion continues through orbicularis muscle, parallel to its fibers, and then behind muscle, superiorly or inferiorly, until over the tumor. Dissection proceeds on the lesion's surface without entering its substance. The most peripheral lobules are dissected slowly and carefully from surrounding normal struc¬ tures, with small snips of a sharp Westcott scissors and intermittent, prophylactic pin¬ point bipolar cautery. Major and minor feed¬ ing arteries and draining veins are thus obliterated before they are severed, and cautery damage to surrounding normal structures is negligible. Incisions into the tumor itself are avoided throughout the pro¬ cedure, since the lesion remains perfused by intact vessels at undissected poles. While some lesions appear well circumscribed on imaging studies, surgical dissection often demonstrates an irregular pattern of lob-
COMMENT
The local complications of cortico¬ steroid injection of periocular hemangi¬ omas may be exaggerations of the desired therapeutic response. The mechanism of action of both systemic and local therapy remains unclear, but sensitization of terminal vascular beds to circulating vasoconstrictors—with resultant narrowing, thrombosis, and involution—has been implicated.24·25 In¬ tralesional administration might also be effective because of embolization of the vascular channels of a hemangioma by the larger particles in corticosteroid suspensions. The particle size in these preparations ranges from 10 to 200 µ , while a red blood cell has a diameter of 6 µ .15·26 If the particles embolize the blood supply of contiguous normal tis¬ sue, the result may be subcutaneous fat atrophy or eyelid necrosis. Other complications of intralesional corticosteroids may be related to more distant vascular connections. Capillary hemangiomas consist of proliferating endothelial cells that circumscribe small blood-filled channels (Fig 2, right). While there may be some variation in degree, these vascular beds are hemodynamically continuous with the normal orbital vessels. Doppler studies,27 contrast-enhanced computed tomogra¬ phy,4 and arteriography28·29—as well as
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surgical findings—all demonstrate high-flow perfusion of capillary he¬ mangiomas. In this respect, the tumors differ from the lymphangiomas30 that also occur as congenital vascular lesions and from the cavernous hemangiomas31 the the
that appear in adulthood. Because capillary hemangiomas are continuous with the orbital and sys¬ temic vasculature, all intralesional in¬ jections might be thought of as intra¬ vascular. Direct or anterograde flow of the smaller particles into the venous system could explain the transient and long-term adrenal suppression that has been documented with careful monitor¬ ing.21 Retrograde (intra-arterial) flow can lead to the retinal arterioles.19·20 While the frequency of reported com¬ plications of intralesional corticoster¬ oids has been low, their gravity must be considered. In a real sense, the treat¬ ment is a form of systemic administra¬ tion, and some authors favor the oral route over intralesional injections when comparing the two.32 We believe that careful surgery may offer the best overall benefit-risk ratio for selected patients. This belief re¬ quires clear distinction between piece¬ meal resection, which leads to brisk hemorrhage, and meticulous dissection of the tumor from surrounding normal tissues. Because there is no capsule, dissection must proceed slowly on the surface lobules (Fig 2, left), with pre¬ emptive pin-point bipolar cautery of scores of feeding and draining vessels. Normal anatomy has been surpris¬ ingly well restored after removal of the lesions, and it has not been necessary to re-create eyelid creases or to alter eye¬ lid levels secondarily. The relief of in¬ duced astigmatism may be related to surgical timing (cases 1 and 2). Later intervention may be less successful be¬ cause of permanent molding of the globe as scierai rigidity increases with age.33 Surgical dissection is not appropriate for all capillary hemangiomas. Lesions that extend into the dermis—those that are bright red and do not allow move¬ ment of the overlying skin—should not be approached in this manner. These superficial tumors may be amenable to transcutaneous treatment with argon or tunable dye lasers, exploiting the se¬ lective absorption of the lasers' energy. The benefit-risk ratio for relatively flat, superficial lesions is favorable.34 The neodymium-YAG laser is more effec¬ tive than the argon for deep or bulky tumors, but complications can be se¬ vere. The carbon dioxide laser requires surgical dissection for exposure and of¬ fers no tumor-specific absorption. Surgical dissection is also not appro¬ priate for massive, diffuse facial lesions.
Fig 4.—Case 3. Top left, A 9-month-old girl with progressive globe displacement. Top right, Tumor-induced expansion of the right bony orbit caused concern about permanent asymmetry. The lesion was isodense with the extraocular muscles. Bottom left, Despite the roentgenographic characteristics, the tumor could be sharply dissected from normal structures. Surgery had been performed through a lower eyelid crease in¬ cision (straight arrow), and a small drain (curved arrow) was maintained overnight. Bottom right, Three months after surgery, without functional or cosmetic residua. In these unfortunate patients, any ef¬ fective therapy carries some risk. We favor low-dose oral prednisone (1 to 4 mg/kg per day)35 as the initial treat¬ ment, administered under the careful supervision of a pediatrician. If local injections are to be used in any patients, it is possible that perilesional—rather than intralesional—
administration might limit intravascu¬ lar dissemination. While many periocular hemangiomas require no treatment, those that do present a therapeutic challenge. Efforts may ultimately be directed at those en¬ dogenous factors that stimulate their growth or inhibit their involution, as suggested by the recent use of inter-
feron alfa.36 At present, we believe that surgical dissection offers acceptably low risks compared with current treat¬ ment options, when performed care¬ fully in selected patients. This investigation was supported in part by an unre¬ stricted grant from Research to Prevent Blindness Ine, New York, NY, and by core grant EY01931 from the National Eye Institute, Bethesda, Md.
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Hemorrhages in Newborn Piglets Following Cardiopulmonary Resuscitation James C. Fackler, MD; Ivor D. Berkowitz, MD; W. Richard Green, Retinal
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Are We Educating a Medical Professional Who Cares? Robert G. Petersdorf, MD, Kathleen Turner, MPA
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MD
