Cardiovasc Intervent Radiol DOI 10.1007/s00270-014-0971-5
PICTORIAL ESSAY
Percutaneous Sclerotherapy of Congenital Slow-Flow Vascular Malformations of the Orbit George Koshy Chiramel • Shyamkumar Nidugala Keshava Vinu Moses • Suraj Mammen • Sarada David • Sudipta Sen
•
Received: 4 May 2014 / Accepted: 14 July 2014 Ó Springer Science+Business Media New York and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) 2014
Abstract Purpose This manuscript describes the clinical features, imaging findings, treatment details, and short-term outcomes of a series of congenital slow-flow vascular malformations. Methods This was a prospective study of congenital slow-flow vascular malformations involving the orbital region treated at a single institution with percutaneous sclerotherapy. Results Ten patients presented during the study period, comprising eight venous malformations, one lymphatic malformation, and one veno-lymphatic malformation. Nine patients underwent percutaneous sclerotherapy under digital subtraction angiography guidance, of which three developed marked rise in intraocular pressure requiring lateral canthotomy. The treatments were performed in the presence of an ophthalmologist who G. K. Chiramel (&) S. N. Keshava V. Moses S. Mammen Department of Radiology, Christian Medical College, Vellore 632004, Tamil Nadu, India e-mail: [email protected] S. N. Keshava e-mail: [email protected] V. Moses e-mail: [email protected] S. Mammen e-mail: [email protected] S. David Department of Ophthalmology, Christian Medical College, Vellore, Tamil Nadu, India e-mail: [email protected] S. Sen Department of Pediatric Surgery, Christian Medical College, Vellore, Tamil Nadu, India e-mail: [email protected]
measured the intraorbital pressure during and after the procedure. On follow-up, some of the patients required repeat sessions of sclerotherapy. All patients had improvement of symptoms on follow up after the procedure. Conclusion Congenital slow-flow vascular malformations of the orbital region are rare lesions that should be treated using a multidisciplinary approach. Monitoring of the intraorbital pressure is required both during and after the procedure to decide about the need for lateral canthotomy to reduce the transiently increased intraorbital pressure. Keywords Orbital malformations Lymphatic malformations Venous malformations Sclerotherapy Lateral canthotomy Inferior cantholysis
Introduction Orbital venous and lymphatic malformations are congenital slow flow vascular malformations that involve the orbit and occur due to aberrations in vasculogenesis during embryonic development [1]. The patient seeks treatment due to symptoms, such as increased orbital pressure, intractable pain, profound orbital hemorrhage leading to visual deterioration, and cosmetic disfigurement [2]. Because these are challenging to treat, a conservative approach is sometimes used [3]. Percutaneous sclerotherapy is considered to be the treatment of choice, because surgical and other options result in varied and suboptimal results. Although this procedure is performed by a few centers worldwide, there is little published literature on vascular malformations of the orbital region. This pictorial essay illustrates our experience in performing percutaneous sclerotherapy
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Fig. 1 Measurement of intraocular pressure using a tonometer immediately after the sclerotherapy procedure
Fig. 3 Clinical photograph showing the appearance of the periorbital region after injection of the sclerosant foam
Fig. 2 Contents of the lymphatic malformation being aspirated with a syringe to ascertain the contents
for orbital venous and lymphatic malformations and describes the procedure and the possible complications.
Materials and Methods This prospective study included patients with venous and lymphatic malformations involving the orbital region who were referred for treatment with percutaneous sclerotherapy during a period of 5 years (March 2009 to March 2014). This study was approved by our institutional research ethics board, and informed consent was obtained from all patients.
123
Fig. 4 A, B Clinical photographs showing lateral canthotomy and inferior cantholysis being performed
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Fig. 5 Flowchart showing the intraorbital changes that occur during percutaneous sclerotherapy. The red boxes show the dangerous effects of a raised intraorbital pressure and the green boxes depict
how lateral canthotomy could reduce the intraocular pressure and preserve the patient’s vision
Procedural Details
portions of the malformation. The malformation was partially aspirated to ascertain its contents as either blood or lymph (Fig. 2). A nonionic contrast agent (Omnipaque-300) diluted with normal saline (1:1 ratio) was gradually injected by hand through each needle under blank roadmap fluoroscopy in the anteroposterior and lateral views to ascertain the anatomy, venous drainage, and volume of the malformation. If there was no communication with the intracerebral veins or cavernous sinus, sclerotherapy was performed by slowly instilling the previously ascertained volume of the sclerosant mixture through each needle, while monitoring the size of the malformation (Fig. 3).
Pre-Procedural Evaluation Before treatment, each patient underwent a complete clinical and ophthalmological evaluation that included photographs, vision tests, pupillary reflexes, and intraocular pressure measurement. MRI (T2W axial and coronal sequences and fat saturated T1W sequences) and ultrasound studies were performed to document the extent of the malformation and to identify patent vascular spaces. Sclerotherapy Procedure
Postprocedural Care All patients underwent percutaneous sclerotherapy under general anaesthesia using aseptic precautions in the digital subtraction angiography suite (Multistar DSA and Artis Zee biplane DSA, Siemens, Germany). Dexamethasone (0.1 mg/kg IV) was given prior to the procedure for all patients. An ophthalmologist was present in the room to measure the intraocular pressure of the involved eye using a tonometer both before and after the procedure (Fig. 1). Using ultrasound guidance, one or more 23G needles were inserted through the overlying normal skin into different
The intraocular pressure and pupillary reflexes were evaluated immediately after the procedure, and the patients were admitted for overnight observation. An ophthalmologist monitored the patient and measured the intraocular pressure measurements at regular intervals. After discharge, the patients were followed up and further sessions of sclerotherapy were performed if there was persistence of symptoms and if vascular spaces were demonstrable on ultrasonography.
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Table 1 Details of each patient’s lesion and treatment performed S. no.
Age (yr)/ gender
Type of malformation and location
Appearance on MRI
Sclerosant mixture composition and volume injected
Lateral canthotomy required?
Further sessions of sclerotherapy?
Outcomes
1
3/M
Venous
Retrobulbar extension
STS:Iohexol (2:1) liquid, 1.5 ml
No
None
Symptoms have improved. Has not required further sclerotherapy.
2
4/F
Venous
Retrobulbar extension
STS:Iohexol:air (2:1:1) foam, 6 ml
Yes
After 4 years, STS:air (1:1) foam 2 ml foam. Lateral canthotomy required
Symptoms have improved after the second session of sclerotherapy.
3
11/M
Venous
Superficial
STS:air (2:1) foam, 3 ml
No
None
Symptoms have improved. Has not required further sclerotherapy.
4
6/M
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 4 ml
No
Two more sessions, 1 year apart. STS:air (1:1) foam 7 and 2 ml. Lateral canthotomy not required.
Symptoms have improved after the third session of sclerotherapy.
5
2/F
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 1.5 ml
Yes
None
Symptoms have improved. Has not required further sclerotherapy.
6
17/F
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 6 ml
No
After 1 year, STS:air (1:1) foam 5.5 ml. Lateral canthotomy not required.
Symptoms have improved after the second session of sclerotherapy.
7
26/F
Lymphatic
Superficial and retrobulbar extension
STS:air:oxytetracycline (1:1:1) foam, 3 ml
Yes
None
Symptoms have improved. Has not required further sclerotherapy.
8
29/F
Venous
Superficial
None
–
No treatment since it was seen to communicate with the intracranial veins
9
14/M
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 4.5 ml
No
After 3 months, STS:air (1:1) foam 4.5 ml. Lateral canthotomy not required.
Symptoms persist since sclerotherapy was not performed. Symptoms have improved after the second session of sclerotherapy.
10
8/M
Venolymphatic
Superficial
STS:air (1:1) foam, 12 ml
No
Two more sessions 6 months apart. Second session STS:air (1:1) foam 12 ml. Third session STS:air (1:1) foam 7 ml with 50 mg of oxytetracycline as 1 ml. Lateral canthotomy was not required for either session.
123
Symptoms have improved after the third session of sclerotherapy.
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Intraocular Pressure Measurement After Sclerotherapy Procedure Sclerotherapy causes inflammatory changes that increase the intraorbital pressure and could result in orbital compartment syndrome [4]. This can be identified early by detecting reduced visual acuity and an afferent pupillary defect. The intraocular pressure has a normal range of 10–21 mm Hg [5]. A value more than 40 mm Hg is an indication for an orbital decompressive surgery, such as lateral canthotomy and inferior cantholysis (Fig. 4), on an emergency basis to reduce the intraorbital pressure [6]. If performed in a timely manner, this procedure can restore the blood flow and can avoid permanent loss of vision (Fig. 5). Fig. 6 Preparation of the sclerosant foam using two syringes and a three-way stopcock
Fig. 7 A Clinical photograph of a 4-year-old girl (Patient 2 as given in Table 1) with a venous malformation involving the right orbit. B Ultrasonography shows large venous spaces. C DSA image (frontal view) showing a sequestered malformation with no communication with the intracerebral veins. D Clinical photograph taken immediately after lateral canthotomy, which was performed due to marked periorbital swelling and loss of eye movements with an intraocular pressure of 60 mm Hg and grade 3 afferent pupillary defect. After
24 hours, there was reduction of periorbital swelling with improved pupillary reflexes and partial restoration of eye movements. E Clinical photograph taken on the third day when there was normalisation of the vision, pupillary reflexes and intraocular pressure. F Clinical photograph taken at 6 months when the patient was asymptomatic with significant reduction of the malformation. There were no residual venous spaces within the malformation
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Fig. 8 A venous malformation around the right eye (Patient 1 as given in Table 1) with retrobulbar location seen on T2W axial MRI images (A–D) and opacified by contrast agent during DSA (E–I)
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Fig. 9 A venous malformation around the left eye (Patient 4 as given in Table 1) with both superficial and retrobulbar components as seen on the T2W axial MRI images (A–C). Both components were opacified by contrast agent during DSA (D–G)
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Fig. 10 A venous malformation around the left eye (Patient 5 as given in Table 1) with both superficial and retrobulbar components as seen on the T2 W axial MRI images (A–F). Only the superficial portion was seen to opacify by contrast agent during DSA and this was treated (G–I)
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Sclerosant Mixture Preparation In the first patient, the sclerosant mixture was injected as a liquid solution (Table 1). Following this case, all sclerosant mixtures were instilled as foam by mixing equal volumes of 3 % sodium tetradecyl sulphate (Setrol) and room air (Fig. 6). For the first two patients, a small amount of a nonionic contrast agent (Omnipaque-300, GE Healthcare) was added to the sclerosant mixture to improve visualisation (Figs. 7, 8). From the third patient onward, no contrast agent was added due to good visualisation provided by the foam mixture (Figs. 9, 10). The lymphatic malformations were treated in a similar manner except that 50 mg of Oxytetracycline (1 ml) was added to the sclerosant foam mixture. The details are provided in Table 1.
Results Over a period of 5 years, this study included ten patients aged 2–26 years with congenital slow flow malformations involving the orbital region. Eight patients had venous malformations, one had a lymphatic malformation, and one had a mixed veno-lymphatic malformation. None had undergone sclerotherapy previously, but two patients had undergone surgery elsewhere with suboptimal results. In one patient, sclerotherapy was not performed since there was venous drainage into the intracerebral veins. On follow up, four patients underwent another session of sclerotherapy, three patients underwent two sessions, and two patients had three further sessions of sclerotherapy. Overall, three of the nine patients who underwent sclerotherapy required lateral canthotomy due to a marked rise in intraocular pressure.
Discussion Congenital vascular malformations that involve the orbital region are rare and considered difficult to treat due to their location. Other centers have used other sclerosants, such as ethanol, polidocanol, OK-432, and Bleomycin for vascular malformations. Our center preferred to use Sodium tetradecyl sulphate as the sclerosant agent for these lesions. Fifty milligrams of tetracycline was added to the sclerosant mixture for sclerotherapy of the lymphatic malformations. Documentation of the intraocular pressure prior to the procedure and the imaging studies helped to prepare for the procedure and to evaluate the anatomy and character of the malformation. Because it is difficult to predict which patient would require lateral canthotomy, this option was kept available for all patients. An ophthalmologist was present to measure the intraocular pressure after the procedure and in the ward. This helped to increase the safety of this procedure.
It could be hypothesized that the factors that predispose to a significant increase in intraorbital pressure include: the overall size of the malformation, type of malformation (venous/lymphatic), morphology (sequestered vs diffuse), drainage pattern, presence of a retrobulbar component, the dynamics of flow within the malformation, volume of sclerosant used, increased concentration of sclerosant, and addition of additional sclerosants. Evaluation of the significance of each of these factors would require a much larger number of patients, which may not be practical in a single center. The small sample size and limited follow-up could be considered as limitations of this study. However, congenital slow flow vascular malformations in the orbital and periorbital region are known to be rare and only a subset of these patients would be symptomatic enough to present to a physician for consideration of percutaneous sclerotherapy as a treatment option. The treatment of vascular malformations of the orbital region should be undertaken in centers that have a multidisciplinary approach to treatment. A comprehensive knowledge of the treatment options and the possible complications is needed before embarking on the treatment of such lesions. An incorrect assessment or treatment procedure may result in serious harm and could limit future options for treatment.
Conclusions Congenital slow-flow vascular malformations involving the orbital region are rare lesions and require a multidisciplinary approach for percutaneous sclerotherapy. Orbital decompressive procedures, such as lateral canthotomy and inferior cantholysis, should be kept as an option if there is a significant rise in intraocular pressure after sclerotherapy. Conflict of Interest Dr. George Koshy Chiramel, Dr. Shyamkumar Nidugala Keshava, Dr. Vinu Moses, Dr. Suraj Mammen, Dr. Sarada David, Dr. Sudipta Sen have no conflict of interest. Disclaimers No grant support to acknowledge. No conflict of interest exists. Informed Consent Consent was obtained from all individual participants included in the study. Human and Animal Rights All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
References 1. Lacey B, Rootman J, Marotta TR (1999) Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management. Ophthalmology 106(6):1197–1209
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy 2. Rootman J, Hay E, Graeb D, Miller R (1986) Orbital-adnexal lymphangiomas. A spectrum of hemodynamically isolated vascular hamartomas. Ophthalmology 93(12):1558–1570 3. Harris GJ, Sakol PJ, Bonavolonta` G, De Conciliis C (1990) An analysis of thirty cases of orbital lymphangioma. Pathophysiologic considerations and management recommendations. Ophthalmology 97(12):1583–1592 4. Lima V, Burt B, Leibovitch I, Prabhakaran V, Goldberg RA, Selva D (2009) Orbital compartment syndrome: the ophthalmic surgical emergency. Surv Ophthalmol 54(4):441–449
123
5. McInnes G, Howes DW (2002) Lateral canthotomy and cantholysis: a simple, vision-saving procedure. CJEM 4(1):49–52 6. Carrim ZI, Anderson IW, Kyle PM (2007) Traumatic orbital compartment syndrome: importance of prompt recognition and management. Eur J Emerg Med 14(3):174–176
PICTORIAL ESSAY
Percutaneous Sclerotherapy of Congenital Slow-Flow Vascular Malformations of the Orbit George Koshy Chiramel • Shyamkumar Nidugala Keshava Vinu Moses • Suraj Mammen • Sarada David • Sudipta Sen
•
Received: 4 May 2014 / Accepted: 14 July 2014 Ó Springer Science+Business Media New York and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) 2014
Abstract Purpose This manuscript describes the clinical features, imaging findings, treatment details, and short-term outcomes of a series of congenital slow-flow vascular malformations. Methods This was a prospective study of congenital slow-flow vascular malformations involving the orbital region treated at a single institution with percutaneous sclerotherapy. Results Ten patients presented during the study period, comprising eight venous malformations, one lymphatic malformation, and one veno-lymphatic malformation. Nine patients underwent percutaneous sclerotherapy under digital subtraction angiography guidance, of which three developed marked rise in intraocular pressure requiring lateral canthotomy. The treatments were performed in the presence of an ophthalmologist who G. K. Chiramel (&) S. N. Keshava V. Moses S. Mammen Department of Radiology, Christian Medical College, Vellore 632004, Tamil Nadu, India e-mail: [email protected] S. N. Keshava e-mail: [email protected] V. Moses e-mail: [email protected] S. Mammen e-mail: [email protected] S. David Department of Ophthalmology, Christian Medical College, Vellore, Tamil Nadu, India e-mail: [email protected] S. Sen Department of Pediatric Surgery, Christian Medical College, Vellore, Tamil Nadu, India e-mail: [email protected]
measured the intraorbital pressure during and after the procedure. On follow-up, some of the patients required repeat sessions of sclerotherapy. All patients had improvement of symptoms on follow up after the procedure. Conclusion Congenital slow-flow vascular malformations of the orbital region are rare lesions that should be treated using a multidisciplinary approach. Monitoring of the intraorbital pressure is required both during and after the procedure to decide about the need for lateral canthotomy to reduce the transiently increased intraorbital pressure. Keywords Orbital malformations Lymphatic malformations Venous malformations Sclerotherapy Lateral canthotomy Inferior cantholysis
Introduction Orbital venous and lymphatic malformations are congenital slow flow vascular malformations that involve the orbit and occur due to aberrations in vasculogenesis during embryonic development [1]. The patient seeks treatment due to symptoms, such as increased orbital pressure, intractable pain, profound orbital hemorrhage leading to visual deterioration, and cosmetic disfigurement [2]. Because these are challenging to treat, a conservative approach is sometimes used [3]. Percutaneous sclerotherapy is considered to be the treatment of choice, because surgical and other options result in varied and suboptimal results. Although this procedure is performed by a few centers worldwide, there is little published literature on vascular malformations of the orbital region. This pictorial essay illustrates our experience in performing percutaneous sclerotherapy
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Fig. 1 Measurement of intraocular pressure using a tonometer immediately after the sclerotherapy procedure
Fig. 3 Clinical photograph showing the appearance of the periorbital region after injection of the sclerosant foam
Fig. 2 Contents of the lymphatic malformation being aspirated with a syringe to ascertain the contents
for orbital venous and lymphatic malformations and describes the procedure and the possible complications.
Materials and Methods This prospective study included patients with venous and lymphatic malformations involving the orbital region who were referred for treatment with percutaneous sclerotherapy during a period of 5 years (March 2009 to March 2014). This study was approved by our institutional research ethics board, and informed consent was obtained from all patients.
123
Fig. 4 A, B Clinical photographs showing lateral canthotomy and inferior cantholysis being performed
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Fig. 5 Flowchart showing the intraorbital changes that occur during percutaneous sclerotherapy. The red boxes show the dangerous effects of a raised intraorbital pressure and the green boxes depict
how lateral canthotomy could reduce the intraocular pressure and preserve the patient’s vision
Procedural Details
portions of the malformation. The malformation was partially aspirated to ascertain its contents as either blood or lymph (Fig. 2). A nonionic contrast agent (Omnipaque-300) diluted with normal saline (1:1 ratio) was gradually injected by hand through each needle under blank roadmap fluoroscopy in the anteroposterior and lateral views to ascertain the anatomy, venous drainage, and volume of the malformation. If there was no communication with the intracerebral veins or cavernous sinus, sclerotherapy was performed by slowly instilling the previously ascertained volume of the sclerosant mixture through each needle, while monitoring the size of the malformation (Fig. 3).
Pre-Procedural Evaluation Before treatment, each patient underwent a complete clinical and ophthalmological evaluation that included photographs, vision tests, pupillary reflexes, and intraocular pressure measurement. MRI (T2W axial and coronal sequences and fat saturated T1W sequences) and ultrasound studies were performed to document the extent of the malformation and to identify patent vascular spaces. Sclerotherapy Procedure
Postprocedural Care All patients underwent percutaneous sclerotherapy under general anaesthesia using aseptic precautions in the digital subtraction angiography suite (Multistar DSA and Artis Zee biplane DSA, Siemens, Germany). Dexamethasone (0.1 mg/kg IV) was given prior to the procedure for all patients. An ophthalmologist was present in the room to measure the intraocular pressure of the involved eye using a tonometer both before and after the procedure (Fig. 1). Using ultrasound guidance, one or more 23G needles were inserted through the overlying normal skin into different
The intraocular pressure and pupillary reflexes were evaluated immediately after the procedure, and the patients were admitted for overnight observation. An ophthalmologist monitored the patient and measured the intraocular pressure measurements at regular intervals. After discharge, the patients were followed up and further sessions of sclerotherapy were performed if there was persistence of symptoms and if vascular spaces were demonstrable on ultrasonography.
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Table 1 Details of each patient’s lesion and treatment performed S. no.
Age (yr)/ gender
Type of malformation and location
Appearance on MRI
Sclerosant mixture composition and volume injected
Lateral canthotomy required?
Further sessions of sclerotherapy?
Outcomes
1
3/M
Venous
Retrobulbar extension
STS:Iohexol (2:1) liquid, 1.5 ml
No
None
Symptoms have improved. Has not required further sclerotherapy.
2
4/F
Venous
Retrobulbar extension
STS:Iohexol:air (2:1:1) foam, 6 ml
Yes
After 4 years, STS:air (1:1) foam 2 ml foam. Lateral canthotomy required
Symptoms have improved after the second session of sclerotherapy.
3
11/M
Venous
Superficial
STS:air (2:1) foam, 3 ml
No
None
Symptoms have improved. Has not required further sclerotherapy.
4
6/M
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 4 ml
No
Two more sessions, 1 year apart. STS:air (1:1) foam 7 and 2 ml. Lateral canthotomy not required.
Symptoms have improved after the third session of sclerotherapy.
5
2/F
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 1.5 ml
Yes
None
Symptoms have improved. Has not required further sclerotherapy.
6
17/F
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 6 ml
No
After 1 year, STS:air (1:1) foam 5.5 ml. Lateral canthotomy not required.
Symptoms have improved after the second session of sclerotherapy.
7
26/F
Lymphatic
Superficial and retrobulbar extension
STS:air:oxytetracycline (1:1:1) foam, 3 ml
Yes
None
Symptoms have improved. Has not required further sclerotherapy.
8
29/F
Venous
Superficial
None
–
No treatment since it was seen to communicate with the intracranial veins
9
14/M
Venous
Superficial and retrobulbar extension
STS:air (1:1) foam, 4.5 ml
No
After 3 months, STS:air (1:1) foam 4.5 ml. Lateral canthotomy not required.
Symptoms persist since sclerotherapy was not performed. Symptoms have improved after the second session of sclerotherapy.
10
8/M
Venolymphatic
Superficial
STS:air (1:1) foam, 12 ml
No
Two more sessions 6 months apart. Second session STS:air (1:1) foam 12 ml. Third session STS:air (1:1) foam 7 ml with 50 mg of oxytetracycline as 1 ml. Lateral canthotomy was not required for either session.
123
Symptoms have improved after the third session of sclerotherapy.
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Intraocular Pressure Measurement After Sclerotherapy Procedure Sclerotherapy causes inflammatory changes that increase the intraorbital pressure and could result in orbital compartment syndrome [4]. This can be identified early by detecting reduced visual acuity and an afferent pupillary defect. The intraocular pressure has a normal range of 10–21 mm Hg [5]. A value more than 40 mm Hg is an indication for an orbital decompressive surgery, such as lateral canthotomy and inferior cantholysis (Fig. 4), on an emergency basis to reduce the intraorbital pressure [6]. If performed in a timely manner, this procedure can restore the blood flow and can avoid permanent loss of vision (Fig. 5). Fig. 6 Preparation of the sclerosant foam using two syringes and a three-way stopcock
Fig. 7 A Clinical photograph of a 4-year-old girl (Patient 2 as given in Table 1) with a venous malformation involving the right orbit. B Ultrasonography shows large venous spaces. C DSA image (frontal view) showing a sequestered malformation with no communication with the intracerebral veins. D Clinical photograph taken immediately after lateral canthotomy, which was performed due to marked periorbital swelling and loss of eye movements with an intraocular pressure of 60 mm Hg and grade 3 afferent pupillary defect. After
24 hours, there was reduction of periorbital swelling with improved pupillary reflexes and partial restoration of eye movements. E Clinical photograph taken on the third day when there was normalisation of the vision, pupillary reflexes and intraocular pressure. F Clinical photograph taken at 6 months when the patient was asymptomatic with significant reduction of the malformation. There were no residual venous spaces within the malformation
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Fig. 8 A venous malformation around the right eye (Patient 1 as given in Table 1) with retrobulbar location seen on T2W axial MRI images (A–D) and opacified by contrast agent during DSA (E–I)
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Fig. 9 A venous malformation around the left eye (Patient 4 as given in Table 1) with both superficial and retrobulbar components as seen on the T2W axial MRI images (A–C). Both components were opacified by contrast agent during DSA (D–G)
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy Fig. 10 A venous malformation around the left eye (Patient 5 as given in Table 1) with both superficial and retrobulbar components as seen on the T2 W axial MRI images (A–F). Only the superficial portion was seen to opacify by contrast agent during DSA and this was treated (G–I)
123
G. K. Chiramel et al.: Orbital Malformation Sclerotherapy
Sclerosant Mixture Preparation In the first patient, the sclerosant mixture was injected as a liquid solution (Table 1). Following this case, all sclerosant mixtures were instilled as foam by mixing equal volumes of 3 % sodium tetradecyl sulphate (Setrol) and room air (Fig. 6). For the first two patients, a small amount of a nonionic contrast agent (Omnipaque-300, GE Healthcare) was added to the sclerosant mixture to improve visualisation (Figs. 7, 8). From the third patient onward, no contrast agent was added due to good visualisation provided by the foam mixture (Figs. 9, 10). The lymphatic malformations were treated in a similar manner except that 50 mg of Oxytetracycline (1 ml) was added to the sclerosant foam mixture. The details are provided in Table 1.
Results Over a period of 5 years, this study included ten patients aged 2–26 years with congenital slow flow malformations involving the orbital region. Eight patients had venous malformations, one had a lymphatic malformation, and one had a mixed veno-lymphatic malformation. None had undergone sclerotherapy previously, but two patients had undergone surgery elsewhere with suboptimal results. In one patient, sclerotherapy was not performed since there was venous drainage into the intracerebral veins. On follow up, four patients underwent another session of sclerotherapy, three patients underwent two sessions, and two patients had three further sessions of sclerotherapy. Overall, three of the nine patients who underwent sclerotherapy required lateral canthotomy due to a marked rise in intraocular pressure.
Discussion Congenital vascular malformations that involve the orbital region are rare and considered difficult to treat due to their location. Other centers have used other sclerosants, such as ethanol, polidocanol, OK-432, and Bleomycin for vascular malformations. Our center preferred to use Sodium tetradecyl sulphate as the sclerosant agent for these lesions. Fifty milligrams of tetracycline was added to the sclerosant mixture for sclerotherapy of the lymphatic malformations. Documentation of the intraocular pressure prior to the procedure and the imaging studies helped to prepare for the procedure and to evaluate the anatomy and character of the malformation. Because it is difficult to predict which patient would require lateral canthotomy, this option was kept available for all patients. An ophthalmologist was present to measure the intraocular pressure after the procedure and in the ward. This helped to increase the safety of this procedure.
It could be hypothesized that the factors that predispose to a significant increase in intraorbital pressure include: the overall size of the malformation, type of malformation (venous/lymphatic), morphology (sequestered vs diffuse), drainage pattern, presence of a retrobulbar component, the dynamics of flow within the malformation, volume of sclerosant used, increased concentration of sclerosant, and addition of additional sclerosants. Evaluation of the significance of each of these factors would require a much larger number of patients, which may not be practical in a single center. The small sample size and limited follow-up could be considered as limitations of this study. However, congenital slow flow vascular malformations in the orbital and periorbital region are known to be rare and only a subset of these patients would be symptomatic enough to present to a physician for consideration of percutaneous sclerotherapy as a treatment option. The treatment of vascular malformations of the orbital region should be undertaken in centers that have a multidisciplinary approach to treatment. A comprehensive knowledge of the treatment options and the possible complications is needed before embarking on the treatment of such lesions. An incorrect assessment or treatment procedure may result in serious harm and could limit future options for treatment.
Conclusions Congenital slow-flow vascular malformations involving the orbital region are rare lesions and require a multidisciplinary approach for percutaneous sclerotherapy. Orbital decompressive procedures, such as lateral canthotomy and inferior cantholysis, should be kept as an option if there is a significant rise in intraocular pressure after sclerotherapy. Conflict of Interest Dr. George Koshy Chiramel, Dr. Shyamkumar Nidugala Keshava, Dr. Vinu Moses, Dr. Suraj Mammen, Dr. Sarada David, Dr. Sudipta Sen have no conflict of interest. Disclaimers No grant support to acknowledge. No conflict of interest exists. Informed Consent Consent was obtained from all individual participants included in the study. Human and Animal Rights All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
References 1. Lacey B, Rootman J, Marotta TR (1999) Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management. Ophthalmology 106(6):1197–1209
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