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9. Galor A, Karp CL, Oellers P, et al. Predictors of ocular surface squamous neoplasia recurrence after excisional surgery. Ophthalmology 2012;119:1974–81. 10. Reifler DM, Hornblass A. Squamous cell carcinoma of the eyelid. Surv Ophthalmol 1986;30:349–65. 11. Shulman J. Treatment of malignant tumours of the eyelids by plastic surgery. Br J Plast Surg 1962;15:37–47. 12. Tunc M, Char DH, Crawford B, et al. Intraepithelial and invasive squamous cell carcinoma of the conjunctiva: analysis of 60 cases. Br J Ophthalmol 1999;83:98–103. 13. Iliff WJ, Marback R, Green WR. Invasive squamous cell carcinoma of the conjunctiva. Arch Ophthalmol 1975;93:119–22. 14. Donaldson MJ, Sullivan TJ, Whitehead KJ, et al. Squamous cell carcinoma of the eyelids. Br J Ophthalmol 2002;86:1161–5.
Orbital Varix Thrombosis and Review of Orbital Vascular Anomalies in Blue Rubber Bleb Nevus Syndrome Pari N. Shams, BSc., M.R.C.P, F.R.C.Ophth*, Sudha Cugati, M.S. Ph.D.*, Tim Wells, M.B.B.S.†, Shyamala Huilgol, F.A.C.D.‡, and Dinesh Selva, F.R.A.C.S., F.R.A.N.Z.C.O.*‡ Abstract: A 57-year-old woman with cutaneous manifestations of (BRBNS) presented with acute left proptosis and venous congestion secondary to thrombosis within a superior ophthalmic vein varix. Multiple phleboliths were noted in the contralateral right orbit, and an incidental right middle cerebral artery aneurysm. Her symptoms spontaneously resolved within a few days. An English literature review found 7 reported cases of orbital vascular lesions in association with BRBNS from 1950 to 2012. All lesions showed contrast enhancement on CT or MRI: 4 had small orbital calcifications and 3 were distensible with raised venous pressure. The occurrence of a thrombosed orbital varix or cerebral artery aneurysm in BRBNS, to the best of the authors’ knowledge, has not been previously reported. Although orbital vascular lesions in BRBNS have been described as hemangiomas, the biologic behavior and histology of most of the reported orbital lesions are most compatible with venous malformations.
B
lue rubber bleb nevus syndrome (BRBNS) is an uncommon multifocal venous vascular malformation syndrome that primarily affects the skin and the gastrointestinal tract. Ocular structures are rarely affected,1 and there have been only 7 reported cases involving the orbit2–7; the majority described as cavernous hemangiomas. In this report, the authors present a case of a thrombosed orbital varix of the superior ophthalmic vein (SOV). They discuss the clinical presentation and review the literature on orbital vascular lesions in BRBNS. This report has adhered to the principles outlined in the Declaration of Helsinki.
*South Australian Institute of Ophthalmology and Visual Sciences, Royal Adelaide Hospital; †Department of Radiology, Royal Adelaide Hospital; and ‡Adelaide Skin and Eye Centre, Adelaide, Australia Accepted for publication June 17, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Pari N. Shams, m.r.c.p., f.r.c.ophth., South Australian Ophthalmology Network, Level 8, East Wing, Royal Adelaide Hospital, Adelaide, South Australia, Australia 5000. E-mail [email protected] DOI: 10.1097/IOP.0000000000000107
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CASE REPORT A 57-year-old woman with known BRBNS presented 24 hours after awakening with sudden left superior orbital and retrobulbar pain and proptosis. She was born with an asymptomatic vascular anomaly on the right shoulder and as a teenager developed multiple painless cutaneous vascular malformations, which often appeared several weeks following a knock or injury to the skin. She underwent surgical excision of numerous lesions from her back and hands for cosmesis and from her tongue to help with speech and mastication. She had no extracutaneous manifestations of BRBNS. Her full blood count and coagulation screen were normal. Her family history was unremarkable. On examination, she had the characteristic bluish, soft, rubber-like, compressible and slowly refilling lesions on her hands (Fig. 1A and 1B), lower lip, buccal mucosa, and tongue (Fig. 1C). She had a 4-mm left proptosis with 2 mm of inferotemporal globe displacement but no change with valsalva (Fig. 1D). There was resistance of the globe to retropulsion; however, the intraocular pressure was normal. There was moderate limitation of left supraduction with mild vertical diplopia on upgaze. She had mild left periorbital oedema, and diffuse conjunctival injection. The remainder of her ocular examination was normal. A CT scan showed a large, nonenhancing serpiginous lesion following the course of SOV, which was hyperdense to brain and isodense to muscle (Fig. 2A and 2B). She received conservative management. One week later, her retrobulbar pain had resolved, and left proptosis had reduced by 2 mm. Her extraocular movements were full with no diplopia. There was complete resolution of her conjunctival injection and periocular oedema. One month later, MRI confirmed an abnormally dilated SOV, which failed to enhance with contrast and had evidence of chronic blood products (Fig. 2C and 2D). In the right orbit, multiple small round hyperdense lesions were seen on CT (Fig. 2E), consistent with phleboliths in the medial extraconal space and also seen on the MRI (Fig. 2F). The MRI scan of the brain revealed an extradural vascular anomaly—a thrombosed sinus pericranii (Fig. 3A) with an overlying bony defect of the left parietotemporal bone seen on the CT scan (Fig. 3B). Multiple venous anomalies were evident within the cerebellum, the basal cistern (Fig. 3C), and involving the supratentorial brain. No dural venous sinus thrombosis was identified on MR venography. Multiple vascular anomalies were noted within the tongue and also the base of skull. A CT angiogram did not show a carotid cavernous fistula. However, it did reveal an incidental 3-mm right middle cerebral artery saccular aneurysm (Fig. 3D), and neurosurgery recommended observation and biannual review. Four months later she remained stable with no recurrence.
DISCUSSION Originally described in 1860 by Gascoyan,8 the term “blue rubber bleb nevus syndrome” was coined by William B. Bean in 19589; BRBNS is a rare syndrome characterized by distinctive bluish, rubbery, cutaneous venous malformations, varying in size from 0.1 to 50 mm and numbering up to several hundred. These malformations can be partly or completely emptied on compression and refill slowly. They are usually present from birth or early childhood and increase in size and frequency with age. A few cases have had an autosomal dominant inheritance10,11 but most are sporadic. Associated ocular findings include vascular malformations of the conjunctiva, iris, and macula.1 Seven BRBNS cases with associated orbital vascular malformations have been reported with an average age of 40 years (7–71), and all were women.2–7 Four patients experienced sudden onset proptosis, and the other 3 patients had intermittent proptosis associated with a rise in intrathoracic pressure. One
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Case Reports
FIG. 1. A–C, The characteristic bluish, soft, rubber-like, compressible lesions of blue rubber bleb nevus syndrome seen on the patient’s hands (A and B), lower lip, buccal mucosa, and tongue (C). D, Photograph of the patient’s eyes 1 week following symptom onset.
FIG. 2. A and B, Axial (A) and coronal CT (B) CT of the orbits, performed 36 hours following the onset of acute left proptosis, showing a large serpiginous lesion following the course of superior ophthalmic vein (SOV) and no enhancement with contrast. C and D, Axial (C) and coronal (D) T1-weighted contrast-enhanced orbital MRI with fat suppression, 3 weeks following acute left proptosis confirming thrombosis within a left SOV varix. E and F, Axial orbital CT scan (E) showing multiple small round hyperdense lesions within the right medial extraconal space consistent with phleboliths, which are also seen on the postcontrast T1-weighted axial MRI scan (F).
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Case Reports
FIG. 3. A, Axial T2-weighted fat suppressed MRI of the brain showing a region of hyperintensity within the left lateral temporal lobe (arrow), which also appears to involve the adjacent calvarium. There is some associated gliosis within the underlying brain consistent with a sinus pericranii. B, Axial CT scan of the brain showing the defect of the left lateral temporal bone. C, Axial T1-weighted postcontrast MRI of the brain showing multiple venous anomalies within the basal cisterns (arrows) and the cerebellum and supratentorial cortex. D, CT angiogram of the brain demonstrating a 3-mm saccular cerebral aneurysm (arrow), located at the right middle cerebral artery bifurcation.
case underwent surgical excision of the vascular malformations due to decreased vision; the other 6 patients recovered spontaneously. Only 2 patients had a known diagnosis of BRBNS at the time of orbital presentation. Early diagnosis of BRBNS is important because it can involve the gastrointestinal tract with the risk of life-threatening hemorrhage,12 consumptive coagulopathy,13 or iron-deficiency anemia14 secondary to occult bleeding. Visceral lesions commonly occur in the gastrointestinal tract, although, the stomach, liver, spleen, heart, thyroid, kidneys, and lungs may also be involved.15 Neurologic deficits from central nervous system lesions have also been reported16–20: an aneurysm of vein of Galen,16 multiple cerebral vascular malformations,16,19 a dural arteriovenous fistula,20 and previous report of a sinus pericranii.21 To the authors’ knowledge, this is the first case of cerebral artery aneurysm reported in association with BRBNS. Other systemic angiomatoses to be considered in the differential diagnosis of cutaneous and orbital vascular malformations include Maffuci syndrome22 characterized by multiple endochondromas but absent in this case, diffuse neonatal angiomatosis24 in which visceral hemangiomas are a prominent feature but not detected in this patient, Klippel–Trenaunay syndrome25 characterized by nevus flammeus, hereditary hemorrhagic telangiectasia associated with visceral telangiectasia and severe iron-deficiency anemia, multiple cutaneous and mucosal venous malformations,26 and glomuvenous malformations.27 A thorough workup is necessary as many of these disorders can be complicated by life-threatening gastrointestinal hemorrhage or cardiac failure.
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In the past, the vascular lesions of BRBNS were erroneously described as hemangiomas. Most reports have referred to cavernous hemangiomas,3–6 now recognized as venous malformations.2,28 Histologically, vascular lesions in BRBNS are similar to other venous malformations. They are characterized by irregular cavernous channels lined by a thin layer of endothelial cells in the deep dermis and subcutis. Surrounding fibrous stroma and occasional smooth-muscle cells form the walls of the lesion. The walls of the vascular structures vary in thickness, ranging from thick and fibrous to thin and delicate. The patient in this study with BRBNS and sudden onset of left proptosis is similar to the case described by Rennie et al.,3 which was nondistensible and improved rapidly. However, Rennie’s case resolved clinically and radiologically within 24 hours, which is atypical for hemorrhage or thrombosis. All 7 orbital vascular lesions reported in BRBNS showed contrast enhancement on CT or MRI. Three lesions were distensible, suggesting a venous vascular lesion, 2 of which were enophthalmic, a sign associated with orbital varices29 suggesting a long-standing lesion with orbital fat atrophy or bony remodeling. The cases reported by Mojon et al.,4 McCannel et al.,7 and Chang and Rubin6 and the asymptomatic right orbit in the patient of this study had calcifications consistent with phleboliths again suggestive of venous lesions.30 The authors believe that their patient developed a thrombosis within an existing orbital varix, arising from the SOV, leading to an acute rise in orbital volume with mass effect and moderate venous congestion. At least 4 previous cases of isolated orbital varix thrombosis have been reported, 3 due to thrombosis of the SOV but not in
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Ophthal Plast Reconstr Surg, Vol. 31, No. 4, 2015
association with BRBNS.31,32 Stasis of pooled blood within the orbital varix for an extended period is thought to result in thrombosis. These cases of orbital varix thrombosis, including our own, presented with symptoms of acute orbital mass effect rather than the severe venous congestion seen in cases of superior ophthalmic vein thrombosis. Thrombosis of the SOV may also occur due to arteriovenous malformation (carotid cavernous or dural sinus fistula),33,34 septic,35–40 or inflammatory41,42 causes. Coagulopathy43,44 and use of the oral contraceptive pill43,45,46 have also been reported as causes of superior ophthalmic vein thrombosis. Orbital varices are usually located on the SOV, manifesting as intermittent proptosis induced by raised orbital venous pressure29 but sometimes as sudden orbital pain and proptosis due to rapid thrombosis.32,47–49 The varix in this case may have been partially thrombosed prior to her acute presentation, allowing a collateral venous circulation to develop maintaining orbital venous drainage and allowing for rapid recovery.
CONCLUSION The patient of this study presented with acute proptosis due to thrombosis within a SOV varix, which spontaneously and rapidly resolved, and she was incidentally found to have a cerebral artery aneurysm; neither lesion has been previously reported in association with BRBNS. Orbital vascular lesions described in BRBNS are clinically and histologically compatible with venous malformations.
REFERENCES 1. Crompton JL, Taylor D. Ocular lesions in the blue rubber bleb naevus syndrome. Br J Ophthalmol 1981;65:133–7. 2. Tuncer S, Peksayar G, Tefekli EG, et al. Multiple orbital venous malformations presenting with enophthalmos in a patient with presumed blue rubber bleb nevus syndrome. Ophthal Plast Reconstr Surg 2008;24:331–4. 3. Rennie IG, Shortland JR, Mahood JM, et al. Periodic exophthalmos associated with the blue rubber bleb naevus syndrome: a case report. Br J Ophthalmol 1982;66:594–9. 4. Mojon D, Odel JG, Rios R, et al. Presumed orbital hemangioma associated with the blue rubber bleb nevus syndrome. Arch Ophthalmol 1996;114:618–9. 5. Sobottka Ventura AC, Remonda L, Mojon DS. Intermittent visual loss and exophthalmos due to the Blue rubber bleb nevus syndrome. Am J Ophthalmol 2001;132:132–5. 6. Chang EL, Rubin PA. Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome. Ophthalmology 2002;109:537–41. 7. McCannel CA, Hoenig J, Umlas J, et al. Orbital lesions in the blue rubber bleb nevus syndrome. Ophthalmology 1996;103:933–6. 8. Gascoyen M. Case of naevus involving the parotid gland and causing death from suffocation: naevi of the viscera. Trans Pathol Soc (Lond) 1860;11:267. 9. Bean WB. Blue rubber bleb nevi of the skin and the gastointerstinal tract. Vascular Spiders and Related Lesions of the Skin. Springfield, IL: Charles C Thomas, 1958:178–85. 10. Walshe MM, Evans CD, Warin RP. Blue rubber bleb naevus. Br Med J 1966;2:931–2. 11. Berlyne GM, Berlyne N. Anaemia due to ‘blue-rubber bleb’ naevus disease. Lancet 1960;2:1275–7. 12. Crosher RF, Blackburn CW, Dinsdale RC. Blue rubber-bleb naevus syndrome. Br J Oral Maxillofac Surg 1988;26:160–4. 13. Hofhuis WJ, Oranje AP, Bouquet J, et al. Blue rubber-bleb naevus syndrome: report of a case with consumption coagulopathy complicated by manifest thrombosis. Eur J Pediatr 1990;149:526–8. 14. Yacoub M, Gnaoui A, Abroug S, et al. The “blue rubber bleb nevus” (Bean’s syndrome): uncommon cause of gastrointestinal bleeding. Ann Pediatr (Paris) 1993;40:157–61. 15. Moodley M, Ramdial P. Blue rubber bleb nevus syndrome: case report and review of the literature. Pediatrics 1993;92:160–2.
Case Reports
16. Waybright EA, Selhorst JB, Rosenblum WI, et al. Blue rubber bleb nevus syndrome with CNS involvement and thrombosis of a vein of galen malformation. Ann Neurol 1978;3:464–7. 17. Garen PD, Sahn EE. Spinal cord compression in blue rubber bleb nevus syndrome. Arch Dermatol 1994;130:934–5. 18. den Heijer T, Boon AJ. Blue rubber bleb nevus syndrome. Neurology 2007;68:1075. 19. Rosenblum WI, Nakoneczna I, Konderding HS, et al. Multiple vascular malformation in the “blue rubber bleb naevus” syndrome: a case with aneurysm of vein of Galen and vascular lesions suggesting a link to the Weber-Osler-Rendu syndrome. Histopathology 1978;2:301–11. 20. Carvalho S, Barbosa V, Santos N, et al. Blue rubber-bleb nevus syndrome: report of a familial case with a dural arteriovenous fistula. AJNR Am J Neuroradiol 2003;24:1916–8. 21. Gabikian P, Clatterbuck RE, Gailloud P, et al. Developmental venous anomalies and sinus pericranii in the blue rubber-bleb nevus syndrome. Case report. J Neurosurg 2003;99:409–11. 22. Sakurane HF, Sugai T, Saito T. The association of blue rubber bleb nevus and Maffucci’s syndrome. Arch Dermatol 1967;95:28–36. 23. Albregts AE, Rapini RP. Malignancy in Maffucci’s syndrome. Dermatol Clin 1995;13:73–8. 24. Haik BG, Clancy P, Ellsworth RM, et al. Ocular manifestations in diffuse neonatal hemangiomatosis. J Pediatr Ophthalmol Strabismus 1983;20:101–5. 25. Rathbun JE, Hoyt WF, Beard C. Surgical management of or bitofrontal varix in Klippel-Trénaunay-Weber syndrome. Am J Ophthalmol 1970;70:109–12. 26. Boon LM, Vikkula M. Multiple cutaneous and mucosal venous malformations. September 18, 2008 [updated August 23, 2012]. In: Pagon RA, Adam MP, Bird TD, Dolan CR, Fong CT, Stephens K, editors. GeneReviews™ [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2013. 27. Henning JS, Kovich OI, Schaffer JV. Glomuvenous malformations. Dermatol Online J 2007;13:17. 28. Nahm WK, Moise S, Eichenfield LF, et al. Venous malformations in blue rubber bleb nevus syndrome: variable onset of presentation. J Am Acad Dermatol 2004;50(5 suppl):S101–6. 29. Handa H, Mori K. Large varix of the superior ophthalmic vein: demonstration by angular phlebography and removal by electrically induced thrombosis. Case report. J Neurosurg 1968;29:202–5. 30. Lloyd GA. Phleboliths in the orbit. Clin Radiol 1965;16:339–46. 31. Iseki S, Ito Y, Nakao Y, et al. Proptosis caused by partially thrombosed orbital varix of the superior orbital vein associated with traumatic carotid-cavernous sinus fistula–case report. Neurol Med Chir (Tokyo) 2010;50:33–6. 32. Bullock JD, Goldberg SH, Connelly PJ. Orbital varix thrombosis. Ophthalmology 1990;97:251–6. 33. Sergott RC, Grossman RI, Savino PJ, et al. The syndrome of paradoxical worsening of dural-cavernous sinus arteriovenous malformations. Ophthalmology 1987;94:205–12. 34. Bavinzski G, Killer M, Gruber A, et al. Treatment of post-traumatic carotico-cavernous fistulae using electrolytically detachable coils: technical aspects and preliminary experience. Neuroradiology 1997;39:81–5. 35. Sanchez TG, Cahali MB, Murakami MS, et al. Septic thrombosis of orbital vessels due to cutaneous nasal infection. Am J Rhinol 1997;11:429–33. 36. Flaharty PM, Phillips W, Sergott RC, et al. Color Doppler imaging of superior ophthalmic vein thrombosis. Arch Ophthalmol 1991;109:582–3. 37. Berenholz L, Kessler A, Shlomkovitz N, et al. Superior ophthalmic vein thrombosis: complication of ethmoidal rhinosinusitis. Arch Otolaryngol Head Neck Surg 1998;124:95–7. 38. Schmitt NJ, Beatty RL, Kennerdell JS. Superior ophthalmic vein thrombosis in a patient with dacryocystitis-induced orbital cellulitis. Ophthal Plast Reconstr Surg 2005;21:387–9. 39. Grassi MA, Lee AG, Kardon R, et al. A lot of clot. Surv Ophthalmol 2003;48:555–61. 40. Walker JC, Sandhu A, Pietris G. Septic superior ophthalmic vein thrombosis. Clin Experiment Ophthalmol 2002;30:144–6. 41. Spirn FH, Wolintz AH, Tenner MS, et al. Tolosa-Hunt syndrome. Ann Ophthalmol 1975;7:1087–90.
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42. Carrim ZI, Ahmed TY, Wykes WN. Isolated superior ophthalmic vein thrombosis with orbital congestion: a variant of idiopathic orbital inflammatory disease? Eye (Lond) 2007;21:665–6. 43. Michaelides M, Aclimandos W. Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 2003;81:88–90. 44. Shinder R, Oellers P, Esmaeli B, et al. Superior ophthalmic vein thrombosis in a patient with chronic myeloid leukemia receiving antifibrinolytic and thrombopoietin receptor agonist therapy. J Ocul Pharmacol Ther 2010;26:293–6. 45. Suissa S, Spitzer WO, Rainville B, et al. Recurrent use of newer oral contraceptives and the risk of venous thromboembolism. Hum Reprod 2000;15:817–21. 46. Jaais F, Habib ZA. Unilateral superior ophthalmic vein thrombosis in a user of oral contraceptives. Med J Malaysia 1994;49:416–8. 47. Wright JE. Orbital vascular anomalies. Trans Am Acad Ophthalmol Otolaryngol 1974;78:OP606–16. 48. Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol Soc 1984;82:134–57. 49. Rootman J. Diseases of the Orbit. Philadelphia, PA: JB Lippincott, 1988.
Disaster in Cosmetic Surgery: Inadvertent Formalin Injection During Blepharoplasty Mohammad Taher Rajabi, M.D.*, Ali Makateb, M.D.*, Hassan Hashemi, M.D.*, Edward J. Holland, M.D.†, Ali Djalilian, M.D.‡, and Jeffrey A. Nerad, M.D.§ Abstract: The authors report a case where formalin was accidentally injected into the eyelids of a 71-year-old woman undergoing blepharoplasty, causing full thickness necrosis of both upper eyelids and ocular complications that required multiple reconstructive surgical procedures.
A
ccidental drug injection in medicine is not a common incident; however, the complications can be disastrous and tragic. Formalin, a 35% to 40% aqueous solution of formaldehyde, is routinely used as a tissue fixative in operation rooms when sending tissues for pathology investigations. On contact with tissues, formalin binds with proteins and denatures them while also causing DNA cleavage and RNA structure inhibition.1 In some operating rooms, this colorless fluid is kept in empty vials of lidocaine that are labeled as formalin. There are several previous reports of accidental formalin injection in blepharoplasty, cataract, and maxillofacial surgery.2–6 This report describes another case of inadvertent formalin injection in blepharoplasty surgery, which led to severe ocular complications.
CASE REPORT A patient was referred to Farabi Eye Hospital, Tehran, Iran, with a history of upper eyelid blepharoplasty 40 days earlier. She was a 71-year-old woman with full thickness necrosis *Department of Ophthalmic Plastic and Reconstructive Surgery, Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran; †Department of Cornea and Refractive Surgery Service, Cincinnati Eye Institute, University of Cincinnati, Cincinnati, Ohio; ‡Department of Ophthalmology and Visual Sciences, Illinois Eye and Ear Infirmary, University of Illinois at Chicago, Chicago, Illinois; and §Department of Ophthalmic Plastic And Reconstructive Surgery, Cincinnati Eye Institute, Ohio, U.S.A. Accepted for publication December 25, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ali Makateb, M.D., Eye Research Center, Farabi Eye Hospital, Qazvin Square, Tehran, Iran 1336616351. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000110
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of both upper eyelids. At her first operation, the surgeon was mistakenly handed a syringe filled with formalin instead of lidocaine, which was then injected subcutaneously into both upper eyelids. The injection was severely painful, and the patient had severe pain during the procedure; however, the surgeon did not understand the cause of ineffective injection of presumed anesthetic injection during the surgery. At her presentation to the authors, the visual acuity was hand motion in the OD and light perception in the OS. The upper eyelids of OU were severely edematous with a thick membranous necrotic tissue (Fig. 1). Both globes were frozen, which presumably had resulted from extraocular muscle and nerve involvement (Fig. 2). On slit lamp examination, there was significant corneal exposure due to inadequate eyelid closure, corneal epithelial defects, and severe corneal edema with bilateral anterior chamber reaction and hypopyon. The anterior segment displayed signs of severe toxicity and ischemia including corneal anesthesia, fixed and dilated pupil in the OD (minimally reactive in the OS), and bilateral cataracts. Fundus details were poorly visible, and there was bilateral papillitis. For evaluation of optic nerves, visually-evoked potential was performed and showed severe delay bilaterally. To control corneal exposure, bilateral blepharorrhaphy was performed through the eyebrow and upper and lower eyelid margins so that the sutures could be opened to evaluate the cornea as needed (Fig. 3). Systemic methylprednisolone 500 mg per day was prescribed for 3 days and then changed to oral prednisolone 50 mg per day to help reduce tissue edema in the eyelids and around the optic nerve. Ciprofloxacin tablets 500 mg 2 times a day and chloramphenicol eye drops were prescribed prophylactically along with artificial tears to control dry eye. After 5 days, betamethasone eye drops were added. After 14 days, the corneal epithelial defect was healed, and by 1 month, corneal sensation had improved, and the patient’s vision improved to count fingers at 4 m in the OD and 2 m in the OS. However, eyelid problems were too extensive to allow any kind of surgery at that time. During this first 2 months, OU were closed with blepharorrhaphy and opened every 3 to 4 days to evaluate the cornea and vision. After 2 months, the necrotic eyelid tissue that had shrunk was removed under local anesthesia. The bed of the tissue was full of purulent necrotic material all the way through to the bone with some necrotic periosteum along the superior orbital rim. On the opposite side, necrotic material over the eyelid extended all the way to the conjunctiva. It was thought that a skin flap might not work properly due to the lack of the orbicularis muscle at the involved area. The authors decided to cover the conjunctiva and some residual parts of the levator muscle with the orbicularis muscle. The orbicularis muscle was prepared as a bucket-handle flap from the orbital part of the upper eyelid and used to cover the conjunctiva and residual levator muscle. As the last step, a skin graft was harvested from the right medial arm and sutured over the eyelid. The graft was secured with sponge bandage for 1 week. The same procedure was performed for the other eye (Fig. 4). Three months later, both corneas had opacified, and the visual acuity was deteriorating, so it was planned to do a triple procedure on the OD to remove cataractous lens also. After the surgery, OD vision was improved to 2/10. During follow up, the patient developed corneal exposure again, so lateral and medial tarsorrhaphy was performed. Moreover, to control lower eyelid retraction, retractor release with spacer implantation was performed on the OS. Meanwhile, due to the high intraocular pressure (IOP), 24 mm·Hg at OD and 28 mm·Hg at OS, the patient was prescribed acetazolamide tablets and timolol drops. The IOP decreased to 16 mm·Hg in the OD and 19 mm·Hg in the OS. However, the OS IOP gradually increased to 26 mm·Hg, and
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Case Reports
9. Galor A, Karp CL, Oellers P, et al. Predictors of ocular surface squamous neoplasia recurrence after excisional surgery. Ophthalmology 2012;119:1974–81. 10. Reifler DM, Hornblass A. Squamous cell carcinoma of the eyelid. Surv Ophthalmol 1986;30:349–65. 11. Shulman J. Treatment of malignant tumours of the eyelids by plastic surgery. Br J Plast Surg 1962;15:37–47. 12. Tunc M, Char DH, Crawford B, et al. Intraepithelial and invasive squamous cell carcinoma of the conjunctiva: analysis of 60 cases. Br J Ophthalmol 1999;83:98–103. 13. Iliff WJ, Marback R, Green WR. Invasive squamous cell carcinoma of the conjunctiva. Arch Ophthalmol 1975;93:119–22. 14. Donaldson MJ, Sullivan TJ, Whitehead KJ, et al. Squamous cell carcinoma of the eyelids. Br J Ophthalmol 2002;86:1161–5.
Orbital Varix Thrombosis and Review of Orbital Vascular Anomalies in Blue Rubber Bleb Nevus Syndrome Pari N. Shams, BSc., M.R.C.P, F.R.C.Ophth*, Sudha Cugati, M.S. Ph.D.*, Tim Wells, M.B.B.S.†, Shyamala Huilgol, F.A.C.D.‡, and Dinesh Selva, F.R.A.C.S., F.R.A.N.Z.C.O.*‡ Abstract: A 57-year-old woman with cutaneous manifestations of (BRBNS) presented with acute left proptosis and venous congestion secondary to thrombosis within a superior ophthalmic vein varix. Multiple phleboliths were noted in the contralateral right orbit, and an incidental right middle cerebral artery aneurysm. Her symptoms spontaneously resolved within a few days. An English literature review found 7 reported cases of orbital vascular lesions in association with BRBNS from 1950 to 2012. All lesions showed contrast enhancement on CT or MRI: 4 had small orbital calcifications and 3 were distensible with raised venous pressure. The occurrence of a thrombosed orbital varix or cerebral artery aneurysm in BRBNS, to the best of the authors’ knowledge, has not been previously reported. Although orbital vascular lesions in BRBNS have been described as hemangiomas, the biologic behavior and histology of most of the reported orbital lesions are most compatible with venous malformations.
B
lue rubber bleb nevus syndrome (BRBNS) is an uncommon multifocal venous vascular malformation syndrome that primarily affects the skin and the gastrointestinal tract. Ocular structures are rarely affected,1 and there have been only 7 reported cases involving the orbit2–7; the majority described as cavernous hemangiomas. In this report, the authors present a case of a thrombosed orbital varix of the superior ophthalmic vein (SOV). They discuss the clinical presentation and review the literature on orbital vascular lesions in BRBNS. This report has adhered to the principles outlined in the Declaration of Helsinki.
*South Australian Institute of Ophthalmology and Visual Sciences, Royal Adelaide Hospital; †Department of Radiology, Royal Adelaide Hospital; and ‡Adelaide Skin and Eye Centre, Adelaide, Australia Accepted for publication June 17, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Pari N. Shams, m.r.c.p., f.r.c.ophth., South Australian Ophthalmology Network, Level 8, East Wing, Royal Adelaide Hospital, Adelaide, South Australia, Australia 5000. E-mail [email protected] DOI: 10.1097/IOP.0000000000000107
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CASE REPORT A 57-year-old woman with known BRBNS presented 24 hours after awakening with sudden left superior orbital and retrobulbar pain and proptosis. She was born with an asymptomatic vascular anomaly on the right shoulder and as a teenager developed multiple painless cutaneous vascular malformations, which often appeared several weeks following a knock or injury to the skin. She underwent surgical excision of numerous lesions from her back and hands for cosmesis and from her tongue to help with speech and mastication. She had no extracutaneous manifestations of BRBNS. Her full blood count and coagulation screen were normal. Her family history was unremarkable. On examination, she had the characteristic bluish, soft, rubber-like, compressible and slowly refilling lesions on her hands (Fig. 1A and 1B), lower lip, buccal mucosa, and tongue (Fig. 1C). She had a 4-mm left proptosis with 2 mm of inferotemporal globe displacement but no change with valsalva (Fig. 1D). There was resistance of the globe to retropulsion; however, the intraocular pressure was normal. There was moderate limitation of left supraduction with mild vertical diplopia on upgaze. She had mild left periorbital oedema, and diffuse conjunctival injection. The remainder of her ocular examination was normal. A CT scan showed a large, nonenhancing serpiginous lesion following the course of SOV, which was hyperdense to brain and isodense to muscle (Fig. 2A and 2B). She received conservative management. One week later, her retrobulbar pain had resolved, and left proptosis had reduced by 2 mm. Her extraocular movements were full with no diplopia. There was complete resolution of her conjunctival injection and periocular oedema. One month later, MRI confirmed an abnormally dilated SOV, which failed to enhance with contrast and had evidence of chronic blood products (Fig. 2C and 2D). In the right orbit, multiple small round hyperdense lesions were seen on CT (Fig. 2E), consistent with phleboliths in the medial extraconal space and also seen on the MRI (Fig. 2F). The MRI scan of the brain revealed an extradural vascular anomaly—a thrombosed sinus pericranii (Fig. 3A) with an overlying bony defect of the left parietotemporal bone seen on the CT scan (Fig. 3B). Multiple venous anomalies were evident within the cerebellum, the basal cistern (Fig. 3C), and involving the supratentorial brain. No dural venous sinus thrombosis was identified on MR venography. Multiple vascular anomalies were noted within the tongue and also the base of skull. A CT angiogram did not show a carotid cavernous fistula. However, it did reveal an incidental 3-mm right middle cerebral artery saccular aneurysm (Fig. 3D), and neurosurgery recommended observation and biannual review. Four months later she remained stable with no recurrence.
DISCUSSION Originally described in 1860 by Gascoyan,8 the term “blue rubber bleb nevus syndrome” was coined by William B. Bean in 19589; BRBNS is a rare syndrome characterized by distinctive bluish, rubbery, cutaneous venous malformations, varying in size from 0.1 to 50 mm and numbering up to several hundred. These malformations can be partly or completely emptied on compression and refill slowly. They are usually present from birth or early childhood and increase in size and frequency with age. A few cases have had an autosomal dominant inheritance10,11 but most are sporadic. Associated ocular findings include vascular malformations of the conjunctiva, iris, and macula.1 Seven BRBNS cases with associated orbital vascular malformations have been reported with an average age of 40 years (7–71), and all were women.2–7 Four patients experienced sudden onset proptosis, and the other 3 patients had intermittent proptosis associated with a rise in intrathoracic pressure. One
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Case Reports
FIG. 1. A–C, The characteristic bluish, soft, rubber-like, compressible lesions of blue rubber bleb nevus syndrome seen on the patient’s hands (A and B), lower lip, buccal mucosa, and tongue (C). D, Photograph of the patient’s eyes 1 week following symptom onset.
FIG. 2. A and B, Axial (A) and coronal CT (B) CT of the orbits, performed 36 hours following the onset of acute left proptosis, showing a large serpiginous lesion following the course of superior ophthalmic vein (SOV) and no enhancement with contrast. C and D, Axial (C) and coronal (D) T1-weighted contrast-enhanced orbital MRI with fat suppression, 3 weeks following acute left proptosis confirming thrombosis within a left SOV varix. E and F, Axial orbital CT scan (E) showing multiple small round hyperdense lesions within the right medial extraconal space consistent with phleboliths, which are also seen on the postcontrast T1-weighted axial MRI scan (F).
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FIG. 3. A, Axial T2-weighted fat suppressed MRI of the brain showing a region of hyperintensity within the left lateral temporal lobe (arrow), which also appears to involve the adjacent calvarium. There is some associated gliosis within the underlying brain consistent with a sinus pericranii. B, Axial CT scan of the brain showing the defect of the left lateral temporal bone. C, Axial T1-weighted postcontrast MRI of the brain showing multiple venous anomalies within the basal cisterns (arrows) and the cerebellum and supratentorial cortex. D, CT angiogram of the brain demonstrating a 3-mm saccular cerebral aneurysm (arrow), located at the right middle cerebral artery bifurcation.
case underwent surgical excision of the vascular malformations due to decreased vision; the other 6 patients recovered spontaneously. Only 2 patients had a known diagnosis of BRBNS at the time of orbital presentation. Early diagnosis of BRBNS is important because it can involve the gastrointestinal tract with the risk of life-threatening hemorrhage,12 consumptive coagulopathy,13 or iron-deficiency anemia14 secondary to occult bleeding. Visceral lesions commonly occur in the gastrointestinal tract, although, the stomach, liver, spleen, heart, thyroid, kidneys, and lungs may also be involved.15 Neurologic deficits from central nervous system lesions have also been reported16–20: an aneurysm of vein of Galen,16 multiple cerebral vascular malformations,16,19 a dural arteriovenous fistula,20 and previous report of a sinus pericranii.21 To the authors’ knowledge, this is the first case of cerebral artery aneurysm reported in association with BRBNS. Other systemic angiomatoses to be considered in the differential diagnosis of cutaneous and orbital vascular malformations include Maffuci syndrome22 characterized by multiple endochondromas but absent in this case, diffuse neonatal angiomatosis24 in which visceral hemangiomas are a prominent feature but not detected in this patient, Klippel–Trenaunay syndrome25 characterized by nevus flammeus, hereditary hemorrhagic telangiectasia associated with visceral telangiectasia and severe iron-deficiency anemia, multiple cutaneous and mucosal venous malformations,26 and glomuvenous malformations.27 A thorough workup is necessary as many of these disorders can be complicated by life-threatening gastrointestinal hemorrhage or cardiac failure.
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In the past, the vascular lesions of BRBNS were erroneously described as hemangiomas. Most reports have referred to cavernous hemangiomas,3–6 now recognized as venous malformations.2,28 Histologically, vascular lesions in BRBNS are similar to other venous malformations. They are characterized by irregular cavernous channels lined by a thin layer of endothelial cells in the deep dermis and subcutis. Surrounding fibrous stroma and occasional smooth-muscle cells form the walls of the lesion. The walls of the vascular structures vary in thickness, ranging from thick and fibrous to thin and delicate. The patient in this study with BRBNS and sudden onset of left proptosis is similar to the case described by Rennie et al.,3 which was nondistensible and improved rapidly. However, Rennie’s case resolved clinically and radiologically within 24 hours, which is atypical for hemorrhage or thrombosis. All 7 orbital vascular lesions reported in BRBNS showed contrast enhancement on CT or MRI. Three lesions were distensible, suggesting a venous vascular lesion, 2 of which were enophthalmic, a sign associated with orbital varices29 suggesting a long-standing lesion with orbital fat atrophy or bony remodeling. The cases reported by Mojon et al.,4 McCannel et al.,7 and Chang and Rubin6 and the asymptomatic right orbit in the patient of this study had calcifications consistent with phleboliths again suggestive of venous lesions.30 The authors believe that their patient developed a thrombosis within an existing orbital varix, arising from the SOV, leading to an acute rise in orbital volume with mass effect and moderate venous congestion. At least 4 previous cases of isolated orbital varix thrombosis have been reported, 3 due to thrombosis of the SOV but not in
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Ophthal Plast Reconstr Surg, Vol. 31, No. 4, 2015
association with BRBNS.31,32 Stasis of pooled blood within the orbital varix for an extended period is thought to result in thrombosis. These cases of orbital varix thrombosis, including our own, presented with symptoms of acute orbital mass effect rather than the severe venous congestion seen in cases of superior ophthalmic vein thrombosis. Thrombosis of the SOV may also occur due to arteriovenous malformation (carotid cavernous or dural sinus fistula),33,34 septic,35–40 or inflammatory41,42 causes. Coagulopathy43,44 and use of the oral contraceptive pill43,45,46 have also been reported as causes of superior ophthalmic vein thrombosis. Orbital varices are usually located on the SOV, manifesting as intermittent proptosis induced by raised orbital venous pressure29 but sometimes as sudden orbital pain and proptosis due to rapid thrombosis.32,47–49 The varix in this case may have been partially thrombosed prior to her acute presentation, allowing a collateral venous circulation to develop maintaining orbital venous drainage and allowing for rapid recovery.
CONCLUSION The patient of this study presented with acute proptosis due to thrombosis within a SOV varix, which spontaneously and rapidly resolved, and she was incidentally found to have a cerebral artery aneurysm; neither lesion has been previously reported in association with BRBNS. Orbital vascular lesions described in BRBNS are clinically and histologically compatible with venous malformations.
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42. Carrim ZI, Ahmed TY, Wykes WN. Isolated superior ophthalmic vein thrombosis with orbital congestion: a variant of idiopathic orbital inflammatory disease? Eye (Lond) 2007;21:665–6. 43. Michaelides M, Aclimandos W. Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 2003;81:88–90. 44. Shinder R, Oellers P, Esmaeli B, et al. Superior ophthalmic vein thrombosis in a patient with chronic myeloid leukemia receiving antifibrinolytic and thrombopoietin receptor agonist therapy. J Ocul Pharmacol Ther 2010;26:293–6. 45. Suissa S, Spitzer WO, Rainville B, et al. Recurrent use of newer oral contraceptives and the risk of venous thromboembolism. Hum Reprod 2000;15:817–21. 46. Jaais F, Habib ZA. Unilateral superior ophthalmic vein thrombosis in a user of oral contraceptives. Med J Malaysia 1994;49:416–8. 47. Wright JE. Orbital vascular anomalies. Trans Am Acad Ophthalmol Otolaryngol 1974;78:OP606–16. 48. Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol Soc 1984;82:134–57. 49. Rootman J. Diseases of the Orbit. Philadelphia, PA: JB Lippincott, 1988.
Disaster in Cosmetic Surgery: Inadvertent Formalin Injection During Blepharoplasty Mohammad Taher Rajabi, M.D.*, Ali Makateb, M.D.*, Hassan Hashemi, M.D.*, Edward J. Holland, M.D.†, Ali Djalilian, M.D.‡, and Jeffrey A. Nerad, M.D.§ Abstract: The authors report a case where formalin was accidentally injected into the eyelids of a 71-year-old woman undergoing blepharoplasty, causing full thickness necrosis of both upper eyelids and ocular complications that required multiple reconstructive surgical procedures.
A
ccidental drug injection in medicine is not a common incident; however, the complications can be disastrous and tragic. Formalin, a 35% to 40% aqueous solution of formaldehyde, is routinely used as a tissue fixative in operation rooms when sending tissues for pathology investigations. On contact with tissues, formalin binds with proteins and denatures them while also causing DNA cleavage and RNA structure inhibition.1 In some operating rooms, this colorless fluid is kept in empty vials of lidocaine that are labeled as formalin. There are several previous reports of accidental formalin injection in blepharoplasty, cataract, and maxillofacial surgery.2–6 This report describes another case of inadvertent formalin injection in blepharoplasty surgery, which led to severe ocular complications.
CASE REPORT A patient was referred to Farabi Eye Hospital, Tehran, Iran, with a history of upper eyelid blepharoplasty 40 days earlier. She was a 71-year-old woman with full thickness necrosis *Department of Ophthalmic Plastic and Reconstructive Surgery, Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran; †Department of Cornea and Refractive Surgery Service, Cincinnati Eye Institute, University of Cincinnati, Cincinnati, Ohio; ‡Department of Ophthalmology and Visual Sciences, Illinois Eye and Ear Infirmary, University of Illinois at Chicago, Chicago, Illinois; and §Department of Ophthalmic Plastic And Reconstructive Surgery, Cincinnati Eye Institute, Ohio, U.S.A. Accepted for publication December 25, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ali Makateb, M.D., Eye Research Center, Farabi Eye Hospital, Qazvin Square, Tehran, Iran 1336616351. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000110
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of both upper eyelids. At her first operation, the surgeon was mistakenly handed a syringe filled with formalin instead of lidocaine, which was then injected subcutaneously into both upper eyelids. The injection was severely painful, and the patient had severe pain during the procedure; however, the surgeon did not understand the cause of ineffective injection of presumed anesthetic injection during the surgery. At her presentation to the authors, the visual acuity was hand motion in the OD and light perception in the OS. The upper eyelids of OU were severely edematous with a thick membranous necrotic tissue (Fig. 1). Both globes were frozen, which presumably had resulted from extraocular muscle and nerve involvement (Fig. 2). On slit lamp examination, there was significant corneal exposure due to inadequate eyelid closure, corneal epithelial defects, and severe corneal edema with bilateral anterior chamber reaction and hypopyon. The anterior segment displayed signs of severe toxicity and ischemia including corneal anesthesia, fixed and dilated pupil in the OD (minimally reactive in the OS), and bilateral cataracts. Fundus details were poorly visible, and there was bilateral papillitis. For evaluation of optic nerves, visually-evoked potential was performed and showed severe delay bilaterally. To control corneal exposure, bilateral blepharorrhaphy was performed through the eyebrow and upper and lower eyelid margins so that the sutures could be opened to evaluate the cornea as needed (Fig. 3). Systemic methylprednisolone 500 mg per day was prescribed for 3 days and then changed to oral prednisolone 50 mg per day to help reduce tissue edema in the eyelids and around the optic nerve. Ciprofloxacin tablets 500 mg 2 times a day and chloramphenicol eye drops were prescribed prophylactically along with artificial tears to control dry eye. After 5 days, betamethasone eye drops were added. After 14 days, the corneal epithelial defect was healed, and by 1 month, corneal sensation had improved, and the patient’s vision improved to count fingers at 4 m in the OD and 2 m in the OS. However, eyelid problems were too extensive to allow any kind of surgery at that time. During this first 2 months, OU were closed with blepharorrhaphy and opened every 3 to 4 days to evaluate the cornea and vision. After 2 months, the necrotic eyelid tissue that had shrunk was removed under local anesthesia. The bed of the tissue was full of purulent necrotic material all the way through to the bone with some necrotic periosteum along the superior orbital rim. On the opposite side, necrotic material over the eyelid extended all the way to the conjunctiva. It was thought that a skin flap might not work properly due to the lack of the orbicularis muscle at the involved area. The authors decided to cover the conjunctiva and some residual parts of the levator muscle with the orbicularis muscle. The orbicularis muscle was prepared as a bucket-handle flap from the orbital part of the upper eyelid and used to cover the conjunctiva and residual levator muscle. As the last step, a skin graft was harvested from the right medial arm and sutured over the eyelid. The graft was secured with sponge bandage for 1 week. The same procedure was performed for the other eye (Fig. 4). Three months later, both corneas had opacified, and the visual acuity was deteriorating, so it was planned to do a triple procedure on the OD to remove cataractous lens also. After the surgery, OD vision was improved to 2/10. During follow up, the patient developed corneal exposure again, so lateral and medial tarsorrhaphy was performed. Moreover, to control lower eyelid retraction, retractor release with spacer implantation was performed on the OS. Meanwhile, due to the high intraocular pressure (IOP), 24 mm·Hg at OD and 28 mm·Hg at OS, the patient was prescribed acetazolamide tablets and timolol drops. The IOP decreased to 16 mm·Hg in the OD and 19 mm·Hg in the OS. However, the OS IOP gradually increased to 26 mm·Hg, and
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