Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 7  |  Issue : 6  |  Page : 773-776  

Intraoral hemangiomas in Sturge-Weber syndrome


1 Department of Anaesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Navi Mumbai, Maharashtra, India
2 Department of Anaesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India

Date of Web Publication18-Nov-2014

Correspondence Address:
Sonal S Khatavkar
Flat S2, Bldg B1, Tejovalya, Raviraj CHSL, Near Cipla Foundation, Warje, Pune - 411 058, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.144879

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  Abstract 

Sturge-Weber syndrome is a rare congenital disease also called as encephalo-trigeminal-angiomatosis, caused by persistence of transitory primordial arterio-venous connections of fetal intracranial vasculature. It is characterized by vascular malformations with capillary venous angiomas that involve face, choroid of eye, and leptomeninges. The main clinical features of this syndrome are port-wine stains, glaucoma, convulsions and angiomas of the airway. Anesthesia management is directed towards keeping the intracranial and intraocular pressures normal, avoiding trauma to hemangiomas, and anticipating difficult airway. We discuss the case of a 13-year-old boy with intraoral hemangiomas resulting in difficulty in speech. Patient was scheduled for digital subtraction angiography (DSA) and sclerotherapy of hemangiomas under general anesthesia. The anesthetic considerations during the peri-operative management, including airway problems, multiple changes in patient position during DSA procedure, precautions during use of radio-contrast media and sclerosing agents are highlighted.

Keywords: Airway hemangioma, radio-contrast media, sclerotherapy, Sturge-Weber syndrome


How to cite this article:
Iyer HR, Khatavkar SS, Vyas VH, Patil SS. Intraoral hemangiomas in Sturge-Weber syndrome. Med J DY Patil Univ 2014;7:773-6

How to cite this URL:
Iyer HR, Khatavkar SS, Vyas VH, Patil SS. Intraoral hemangiomas in Sturge-Weber syndrome. Med J DY Patil Univ [serial online] 2014 [cited 2024 Mar 29];7:773-6. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2014/7/6/773/144879


  Introduction Top


 Sturge- Weber syndrome More Details More Details (SWS), also called as encephalo-trigeminal-angiomatosis, is a rare congenital disease which occurs sporadically with incidence of 1 in 50,000. [1] It is caused by persistence of transitory primordial arterio-venous connections of fetal intracranial vasculature, characterized by vascular malformations with capillary venous angiomas that involve face, choroid of eye and leptomeninges. The main clinical features are port-wine stains, angiomas, glaucoma and convulsions.

We discuss a case of SWS with intraoral hemangiomas posted for digital subtraction angiography (DSA) and sclerotherapy under general anaesthesia (GA).


  Case Report Top


A 13-year-old boy weighing 24 kg presented with two painless swellings of tongue since 7 months of age. The swellings progressively increased in size and were affecting clarity of speech though swallowing and breathing were unaffected. His birth history and mile stones were normal but he had poor scholastic performance. He had no history of ocular symptoms/allergy/atopy.

He had two episodes of seizures at 6 months of age for which he was on anticonvulsants for a period of 1 year; subsequently child was seizure-free. H/O uneventful excision of a submandibular swelling under GA 6 years back.

On examination, patient had microcephaly (head circumference 45 cm) and had a port-wine stain of 2 cm × 3 cm on right vestibular region. His airway assessment showed an adequate mouth opening with a 3 cm × 3 cm swelling on the right side and a 1 cm × 1 cm swelling on the left side of tongue, both bluish in color, nonpulsatile, noncaloric (temperature was normal) and noncompressible. Neck movements were normal [Figure 1].
Figure 1: Tongue with angiomas

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Routine blood investigations, chest X-ray, coagulation profile and electroencephalogram were normal. Intraocular pressures (IOP) were normal. Ultrasonography tongue showed developmental vascular malformation. Magnetic resonance imaging (MRI) tongue showed veno-lymphatic malformation of both swellings. MRI brain showed gliosis in the right fronto-temporal region with abnormally draining dural and pial veins. A diagnosis of SWS was made. A DSA was planned under GA, to evaluate the flow type (high or low) in the hemangioma and its feeding vessels, followed by sclerotherapy.

Steroids started in the form tablet prednisolone 2 mg/kg two divided doses for 1 month prior to procedure to reduce the size of swelling; but due to edema and raised IOP after 20 days steroids were stopped. [2] Tablet phenytoin 5 mg/kg was started 1 week prior to procedure.

The child was premedicated with intravenous midazolam 0.5 mg, fentanyl 25 μg, hydrocortisone 50 mg and pantoprazole 20 mg. After induction with pentothal sodium 125 mg and atracurium 16 mg gentle, atraumatic, oral intubation was achieved in the first attempt. Injection lignocaine 1 mg/kg was given to attenuate intubation response. Anesthesia was maintained with 50% O 2 : 50% N 2 O and 1-3% sevoflurane. Hydrocortisone and chlorpheniramine given before injection of radio-contrast media (RCM). During DSA position of endotracheal tube (ETT) and circuit was safeguarded as continuous change in a position due to C arm.

Pulse rate, electrocardiography, SpO 2 , EtCO 2 and temperature were monitored throughout the procedure; EtCO 2 was maintained between 25 and 30 mms Hg and systolic blood pressure (BP) between 90 and 100 mms Hg.

Digital subtraction angiography of carotid (external and internal) and vertebral arteries was performed following femoral artery catheterization [Figure 2] with 50 ml of nonionic, water soluble contrast Iohexol (maxview 350, 840 mOsm/kg). Study revealed slow-flow arterio-venous malformations (AVM) on the right side of tongue, in the right masseter muscle and left nasal region (supplied by left facial artery). Intra-lesional sclerotherapy of the AVM on the tongue was performed by injection of 3 ml of a mixture of polidocanol (Asklerol TM, Samarth Life Sciences Pvt. Ltd., Samarth House, Ram Mandir Road, Goregaon (W), Mumbai) and lipiodol [Figure 3].
Figure 2: Digital subtraction angiography tongue hemangioma

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Figure 3: Intra-lesional injection of sclerosant

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A tongue stitch was taken on conclusion of the procedure [Figure 4]. Ringer lactate with 1% dextrose 400 ml was given intra-operatively. Injection ondansetron 2 mg and lignocaine 1 mg/kg was given before reversal and child was extubated uneventfully. Postoperative pain was managed with paracetamol.
Figure 4: Postoperative with tongue stitch

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He was kept under observation in Intensive Care Unit (ICU) for 24 h and was discharged after 5 days.


  Discussion Top


Sturge-Weber syndrome is a congenital neuro-cutaneous disorder characterized by multiple angiomas involving the leptomeninges and skin of face, typically in ophthalmic and maxillary distribution of trigeminal nerve. The main features are port-wine stains, angiomas, convulsions and glaucoma with various degree of mental retardation.

The vascular plexus of embryonal blood vessels normally regresses around 9 th week of gestation. Failure of this regression results in residual vascular tissue which forms angiomas of leptomeninges, face and ipsilateral eye. [3] Both microcephaly and macrocephaly are reported in SWS. [4] Various AVM in brain give rise to stasis and ischemia leading to calcification and laminar cortical necrosis.

Diagnosis is usually confirmed with computed tomography or MRI brain which shows leptomeningeal angiomatosis with calcification and atrophy of cortex. [5] Cerebrospinal fluid analysis may reveal elevated protein due to micro-hemorrhages. Skull films show characteristic pattern of parallel serpentine configuration due to gyriform calcifications called "tram track" or "rail road" sign. [6]

Sturge-Weber syndrome patient may present for facial/cosmetic/or various other surgeries. Our patient presented for DSA and sclerotherapy of haemangiomas. Numerous therapies like steroid therapy, pulsed dye laser (PDL) or embolization with sclerosants have been used in an attempt to treat hemangiomas. Steroid therapy is limited by its systemic side effects as in our case. PDL remains treatment of choice for majority of children with port-wine stain.

Multiple studies have advocated the use of sclerotherapy for low-flow vascular malformation as treatment of choice. [7] DSA might be useful in locating exact angio-architecture and venous dynamics in SWS. [8] Thus in this case, DSA study followed by direct intra-lesional sclerosant injection was performed.

Angiomas may involve the airway (nose, palate, gingiva, tongue, larynx, and trachea) leading to difficult mask ventilation, laryngoscopy and intubation, apart from bleeding caused by rupture of hemangioma. Good premedication is necessary to avoid increase in size of the angiomas due to anxiety. It is reported that stress can cause expansion of hemangiomas leading to excessive bleeding or rupture. [9] Intubation should be done atraumatically. Intra-operative use of light weight circuit is beneficial as it is amenable to continuous changes in patient position during fluoroscopy. Position of ETT, circuit, lines and monitoring equipment are to be safe guarded. Adequate depth of anesthesia and neuromuscular blockade is required to prevent artifacts in films due to patient movement and rise in IOP and Intracranial pressure. BP should be well-controlled peri-operatively as these vessels have abnormal autoregulation. Events such as hypoxemia, hypoglycemia, hypotension, and ischemia should be avoided.

The incidence of reaction induced by RCM is between 5% and 8% and fatal reaction is approximately 1 in 50,000. [10] Combining pretreatment with steroids and diphenhydramine along with use of nonhyperosmolar contrast media will benefit in preventing reactions in high risk patients. In addition, steroids and adequate hydration are necessary to avoid ill-effects of RCM.

A tongue stitch and postoperative observation in ICU is recommended as increase in size of hemangioma and airway edema due to post sclerosant reaction may lead to respiratory distress.


  Conclusion Top


A good knowledge and an understanding of SWS, careful preoperative evaluation and a proper anesthetic management plan are of utmost significance for a successful outcome.

 
  References Top

1.
Delvi MB, Takrouri MS. Anesthesia for encephalo-trigeminal angiomatosis (Sturge-Weber syndrome). Middle East J Anesthesiol 2006;18:785-90.  Back to cited text no. 1
    
2.
Brevière GM, Piette F, Beregi JP, Rey C. Hemangioma and superficial arteriovenous malformations. Arch Mal Coeur Vaiss 1999;92:649-58.  Back to cited text no. 2
    
3.
Ramanathan G, Prasanna, Karunanithi P, Venkatraman R. Anaesthetic management of a case of Sturge-Webers syndrome. Indian J Anaesth 2012;56:302-4.  Back to cited text no. 3
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4.
Chhajed M, Pandit S, Dhawan N, Jain A. Klippel-Trenaunay and Sturge-Weber overlap syndrome with phakomatosis pigmentovascularis. J Pediatr Neurosci 2010;5:138-40.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Arulrajah S, Ertan G, M Comi A, Tekes A, Lin DL, Huisman TA. MRI with diffusion-weighted imaging in children and young adults with simultaneous supra-and infratentorial manifestations of Sturge-Weber syndrome. J Neuroradiol 2010;37:51-9.  Back to cited text no. 5
    
6.
Gandhi M, Iyer H, Sehmbi H, Datir K. Anaesthetic management of a patient with sturge-weber syndrome undergoing oophorectomy. Indian J Anaesth 2009;53:64-7.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Donnelly LF, Adams DM, Bisset GS 3 rd . Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. AJR Am J Roentgenol 2000;174:597-608.  Back to cited text no. 7
    
8.
Smith Pearl M, Abdalla WM, Lin DD, Comi AM, Boltshauser E, Gailloud P, et al. Sturge-Weber syndrome with cerebellar involvement. J Neuroradiol 2009;36:57-60.  Back to cited text no. 8
    
9.
Yamashiro M, Furuya H. Anesthetic management of a patient with Sturge-Weber syndrome undergoing oral surgery. Anesth Prog 2006;53:17-9.  Back to cited text no. 9
    
10.
Holzman R. Anaphylactic reaction and anaesthesia. In: Longnecker DE, editor. Anesthesiology. 2 nd ed. New York: McGraw Hill; 2008. p. 1958.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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