Sturge-Weber syndrome (SWS) also known as

encephalotrigeminal angiomatosis. It is a

neurocutaneous syndrome, characterized by a

facial vascular birthmark and neurological

abnormalities. An ipsilateral or bilateral facial

cutaneous vascular malformation Port Wine Stain

(PWS) usually affects the upper face. Other

clinical manifestations are seizures, glaucoma,

hemiparesis, mental retardation and delayed

developmental milestones. The main objective of

this case report is to unravel such a rarest syndrome

with bilateral port-wine stain, which has

intraoral manifestation of pyogenic granuloma

involving gingiva in an 11 year old boy

Mamata G. P

Rajeshwari G. Annigeri

Shakuntala G. K.

Shantala Arunkumar

Sturge-Weber syndrome (SWS) also known as encephalotrigeminal angiomatosis. It is a neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. An ipsilateral or bilateral facial cutaneous vascular malformation Port Wine Stain (PWS) usually affects the upper face. Other clinical manifestations are seizures, glaucoma, hemiparesis, mental retardation and delayed developmental milestones. The main objective of this case report is to unravel such a rarest syndrome with bilateral port-wine stain, which has intraoral manifestation of pyogenic granuloma involving gingiva in an 11 year old boy

Shakuntala G. K

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CASE REPORT Pyogenic Granuloma in a Patient of Sturge-Weber Syndrome with Bilateral Port Wine Stain- A Rare Case Report

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 Journal of Krishna Institute of Medical Sciences UniversityJKIMSU,    Vol. 3, No. 2, July-Dec 2014CASE REPORTISSN 2231-4261Ó ÓAbstractSturge-Weber  syndrome  (SWS)  also  known  as encephalotrigeminal  angiomatosis.  It  is  a neurocutaneous  syndrome,  characterized  by  a facial  vascular  birthmark  and  neurological abnormalities. An  ipsilateral  or  bilateral  facial cutaneous vascular malformation Port Wine Stain (PWS)  usually  affects  the  upper  face.  Other clinical  manifestations  are  seizures,  glaucoma, hemiparesis,  mental  retardation  and  delayed developmental milestones. The main objective of this case report is to unravel such a rarest syn-drome with bilateral port-wine stain, which has intraoral  manifestation  of  pyogenic  granuloma involving gingiva in an 11 year old boy. Keywords: Bilateral Port-Wine Stain, Pyogenic Granuloma,  Sturge-Weber  Syndrome,  Vascular MalformationsIntroductionSturge-Weber  Syndrome  (SWS)  or  encephalo-trigeminal angiomatosis is a rare nonhereditary developmental condition. It is a sporadic neuro-cutaneous syndrome affecting the cephalic venous micro-vasculature. It occurs, with an estimated frequency of 1 in 50,000 live births [1]. SWS typically  presents  with  a  flat  facial  cutaneous vascular  malformation  in  a  neonate.  The  syn-drome  is  characterized  by  leptomeningeal  and cerebral  angiomas,  typically  ipsilateral  to  the facial lesion, often accompanied by convulsions, 148Pyogenic Granuloma in a Patient of Sturge-Weber Syndrome with Bilateral Port Wine Stain- A Rare Case Report1* 2 2 3 Shantala Arunkumar , Mamata G. P. , Rajeshwari G. Annigeri , Shakuntala G. K.1Department of Oral Medicine and Radiology, SDM College of Dental Sciences and Hospital, Sattur, 2 Dharwad - 580009 (Karnataka) India;  College of Dental Sciences and Hospital, Davangere - 577004, 3 (Karnataka) India;  MAHE Institute of Dental Sciences & Hospital, Mahe - 673310, (Pondicherry) India.mental  retardation,  contralateral  hemiparesis, hemiatrophy,  homonymous  hemianopia,  and glaucoma. The usual cutaneous finding is a uni-lateral nevus flammeus [Port Wine Stain (PWS)], involving an area supplied by trigeminal nerve. The PWS varies from light pink to deep purple. The overall risk of developing SWS in a patient with any kind of cutaneous vascular malformation is approximately 8%. The lesion in SWS, typically is a few centimeters across and involves the face, scalp, and neck. It is virtually a rule that at least part of the PWS extends to the forehead and upper eyelid.  Patients  with  the  involvement  of  the eyelids are at a higher risk of development of eye and brain disease. In upto 40%, the facial PWS is bilateral, although not necessarily symmetrical. The facial nevus flammeus over a period of time may not remain macular, and may develop papular and verrucous changes [2].SWS have a special meaning to oral physician due to its strong head and neck involvement and a possible manifestation in the oral cavity [3]. Many clinical  aspects  for  SWS  are  published  in  the literature. We are reporting a case of SWS with bilateral occurrence of PWS, which also presented with pyogenic granuloma involving gingiva in the lower left premolar region.Case Report:An 11 year old boy, who was a diagnosed case of SWS reported to our department with swelling   over the left lower gum region since 3 months. Initially the swelling started in the left lower gum region, of peanut size and slowly progressed to the present size.   Swelling was not associated with pain,  ulceration  or  pus  discharge.  Patient  had difficulty in mastication, closing the mouth and speaking,  noticed  bleeding  while  brushing  the teeth. There was no history of difficulty in swal-lowing, fever or weight loss and similar swelling elsewhere in the body, or oral cavity. He was born prematurely to nonconsanguineous parents and had  delayed  milestones.  At  birth  cutaneous angiomas were noted on his left and right facial region extending to neck and patient was kept under observation. Patient had first epileptic atta-ck at the age of 8 months and once in a month till the age of 6yrs and he was on continuous treat-ment with low doses of sodium valproate from the time of first attack and currently on 200 mgs twice daily. At present seizures are under control and motor  development  has  been  normal,  but  his mental developments has been subnormal. This has been confirmed by psychological assessment reports showing IQ of 47 on Binet Kamat test of intelligence & below the average on other test indicating the cognitive deficits. Electroencepha-logram reports were abnormal, suggestive of post central dysfunction,  which  necessitated  for primary level institutionalized care. CSF analysis reports were within the normal limits. Magnetic Resonance  Imaging  (MRI)  of  brain  (Fig.1) showed bilateral mild atrophy of occipital and parietal lobes right more than left with the areas of gyriform calcifications.Post contrast images showed dense bilateral lepto-meningeal enhancement involving occipital and post  parietal  lobes  and  minimal  bilateral  gyral enhancement  suggestive  of  ischemic  changes, paucity of cortical veins, enlargement of choroid plexus bilaterally and prominent medullary veins were seen in MRI angiography images. Ophthal-mology opinion was taken, which was normal.  JKIMSU,    Vol. 3, No. 2, July-Dec 2014 Shantala Arunkumar et. al. Journal of Krishna Institute of Medical Sciences University Ó Ó 149Fig. 1: MRI showing gyriform calcifications of parietal and occipital lobesOn clinical examination, patient was in a good general  condition,  had  normal  gait,  was  con-scious, co-operative, less oriented with the time, place and as a person with  normal vital signs. His face  was  markedly  red  and  slightly  swollen bilaterally, covering whole face including the ears and forehead with central part was spared, on left side  extending  to  neck  region  suggestive  of cutaneous angiomas. Hypertrophy of the upper and lower lips was seen (Fig.2).Fig. 2: Bilateral Portwine Stain over the Face and NeckOn intraoral examination, the mucosa covering the oral cavity was reddish in color with no evidence of any hypertrophy, except for a solitary, sessile, well defined swelling in the gingiva of 33, 34, & 75 covering the crowns of 33 & 34, measuring about 2x2cm in size. Anteroposteriorly extending from distal surface of 32 to the distal surface of 75, superoinferiorly  from  lingual  gingiva  by  com-pletely covering the occlusal surfaces of 33 & 34 to the buccal vestibule. Mucosa over the swelling was pale pink in color, covered with the necrotic slough; no evidence of bleeding was present. On palpation it was non-tender, soft to firm in consis-tency, did not bleed on palpation. So clinically, it was diagnosed as pyogenic granuloma of gingiva (Fig. 3). It was planned for excisional biopsy.Fig. 3: Pyogenic Granuloma of GingivaOn intraoral periapical radiography radiolucency was seen in the primary second molar suggestive of  caries  and  bone  loss  present  mesial  to unerrupted 35 & increased interdental space was seen between 33 & 34. On panoramic radiography altered trabecular pattern was seen and width of the mandible was found to be less (Fig.4). His posteroanterior & lateral skull views showed no intracranial calcifications.Pediatric (genetic specialist) opinion was taken and  patient  was  started  on  prednisolone  in  a tapering dosage for one week before excision of the swelling, to prevent bleeding.Later the growth was excised under conscious sedation and sent it  JKIMSU,    Vol. 3, No. 2, July-Dec 2014 Shantala Arunkumar et. al. Journal of Krishna Institute of Medical Sciences University Ó Ó 150for  histopathological  investigations  and  was diagnosed as a pyogenic granuloma of gingiva. Postoperative  period  was  uneventful,  and  no recurrence after four-years of follow up. Fig. 4: Panoramic Ragiograph Showing Bone Resorption From 33-35Discussion:SWS was first described by Schirmer in 1860 and later more specifically by Sturge in 1879, who associated  dermatological  and  ophthalmic changes of the disease to neurologic manifesta-tions. Weber in 1929 complemented it with the documentation  of  radiologic  alterations  where calcifications  are  gyriform  and  curvilinear  and most  commonly  seen  in  parietal  and  occipital lobes  of  brain  [4].  Later  neurocutaneous  syn-drome was first discussed as a clinical entity by the ophthalmologist Van der Hoeve, who included neurofibromatosis,  tuberous  sclerosis,  Sturge-Weber  syndrome  and Von  Hippel-Lindau  syn-drome. Currently 20 to 30 disorders are grouped under the neurocutaneous syndromes [5, 6].The SWS is thought to result from anomalous development of the primodial vascular bed during the early stages of cerebral vascularization. At this stage, the blood supply to the brain, meninges and face  is  undergoing  reorganization  while  the primitive  ectoderm  in  the  region  differentiates into the skin of the upper face and occipital lobe of the cerebrum. The overlying leptomeninges are richly  vascularized,  and  the  brain  beneath becomes atrophic and calcified, particularly in the molecular layer of the cortex [3]. It is presumed that interference at 5 to 8 weeks of gestation with the  development  of  vascular  drainage  of  these areas subsequently affects the face, eyes, lepto-meninges, and brain [7]. These structures share a common embryological basis in the promesen-cephalic and mesencephalic neural crest, where an unknown insult results in malformations in the tissue that differentiates from it. Most common features  are  epilepsy  (80%),  dermal  angiomas resulting in PWS (76%), abnormal findings in skull  radiographs  (63%),  mental  retardation (54%), ocular involvement (37%) and hemiplegia (37%) [3].SWS is referred to as complete when both CNS and facial angiomas are present and incomplete when only one area is affected without the other. The  Roach  Scale  is  used  for  classification,  as follows [5].Ÿ Type  I  -  Both  facial  and  leptomeningeal angiomas; may have glaucomaŸ Type  II  -  Facial  angioma  alone  (no  CNS involvement); may have glaucoma Ÿ Type III - Isolated leptomeningeal angioma; usually no glaucomaIn our case, patient had both facial and leptom-eningeal angiomas, but no glaucoma, so it is SWS of type I. The facial nevus is present at birth, in 40% of case is bilateral as we see in our case. Three-fourth percent of the patients with bilateral facial hemangiomas have a tendency to extension of the hemangioma beyond the face to the neck, trunk  and  extremities  [2].  Our  patient  too  had facial angioma at birth, bilaterally covering the whole face, extending to neck on left side and ear. Seizures develop in most patients during first year of life [5]. They are typically focal tonic clonic and contralateral to the side of the facial nevus. Our patient had partial seizure on left side with second-ary generalization, which was developed within one year of age and he was managed symptomati-cally with low doses of sodium valproate.JKIMSU,    Vol. 3, No. 2, July-Dec 2014 Shantala Arunkumar et. al. Journal of Krishna Institute of Medical Sciences University Ó Ó 151The seizures in SWS may become refractory to anticonvulsants and are associated with a slowly progressive hemiparesis in many cases [3]. About one half of patients are mentally abnormal and have behavioral problems. As seizures of early onset increase in frequency and severity, mental functions and behavior often regress probably the result  of  prolonged  generalized  seizures  and increasing cerebral atrophy. Secondary to local hypoxia and use of numerous anticonvulsants and even in our case also delayed milestones with cognitive deficits were reported.Glaucoma and  bupthalamos  (hydrophthalmia), enlargement  of  the  eye  occurs  from  the  same mechanisms as glaucoma of the ipsilateral eye are a common complication and but not a consistent feature [8]. The ophthalmic examination of our patient did not reveal any abnormalities. The radiographic hallmark of SWS is “tram-line” or gyriform calcifications usually involving the occipital and parietal lobes is seen in plain skull radiographs  usually  demonstrate  intracranial calcification that have a serpentine linear, parallel configuration (tram sign) primarily in the occipi-tal or parieto-occiptal regions in about 90% of patients [9]. Our patient's skull radiographs were normal, but minute changes were highlighted in MRI brain. This is probably because the changes appear usually by the second decade. The intraoral PWS may remain macular or may proliferate to become papular or verrucous growth. Many cases are reported in the literature with hyperplasia of the vascular malformation or disfiguring general-ized gingival enlargement because of long-term phenytoin therapy [8]. In our case a well defined growth  was  present  and  histopathologically diagnosed  as  pyogenic  granuloma,  as  per  our knowledge, this is a rare case of SWS with bilat-eral port-wine stain having localized, well defined gingival  growth  suggestive  of  pyogenic granuloma. Considering patients general condition, we need to modify the type of anesthesia. In our case, the excision was done under intravenous conscious sedation with propofol and N2O-O2 to reduce the patient's emotional stress. Periodontal ligament injection  with  2%  lignocaine  containing 1:80,000U  epinephrine  was  chosen  for  local anesthesia.  Deep  sedation  requiring  airway manipulation should be avoided because there are possible difficulties in airway maintenance. No change  of  antiepileptic  drugs  was  suggested because  of  no  enlarged  gingiva  and  patient remained seizure free for many years.1. Madaan  V,  Dewan  V,  Ramaswamy  S,  Sharma  A. Behavioural manifestations of Sturge-Weber sydrome: A case report. Prim Care Companion J of Clinical Psychiatry 2006; 8:198-200.2. Zaki SA, Lad V. Sturge-Weber syndrome with bilateral facial nevus and early cerebral calcification. J Pediatr Neurosci 2011; 6(2):114-115.3. Sampaio    Gc,  Pereira  Jrd,  Crazal  C,  Sorbral Apv. Chronic apical periodontitis in patient with bilateral Sturge-Weber syndrome: report of a case. Odontologia Clínico-Científica2008;7(1):81-85 4. Khambete  N,  Risbud  M,  Kshar  A.  Sturge-Weber syndrome:  A  case  report.  International  Journal  of Dental Clinics 2011;3(1):79-81    5. Mukhopadhyay  S.  Sturge-Weber  syndrome: A  case References:*Author for Correspondence: Dr. Shantala Arunkumar, Department of Oral Medicine and Radiology, SDM College of Dental Sciences and Hospital, Sattur, Dharwad - 580009 (Karnataka) India. Cell: 9481929790 Email: jinkashanti@gmail.com JKIMSU,    Vol. 3, No. 2, July-Dec 2014 Shantala Arunkumar et. al. Journal of Krishna Institute of Medical Sciences University Ó Ó 152report.  J  Indian  Soc  Pedod  Prev  Dent  2008;  26 (Suppl1):S29-31.6. Jing Z, Nan-yun LI, Xiao-jun Z, Jian-dong W, Heng-hui MA and Ru-song Z. Sturge-Weber syndrome: A case report and review of literatures. Chinese Medical Journal 2010;123(1):117-1217. Maria BN. Sturge-Weber Syndrome. In: Chapter 73: Current  Management  in  Child  Neurology.  Third edition; 2005: 470-4758. Hobson C, Foyaca-Sibat H, Hobson B, L de F Ibanez-Valdes. Sturge-Weber syndrome Type I "Plus": A Case Report. The Internet Journal of Neurology 2006;5(2)    9. V  Kumar,  BK  Prasad.  Sturge  Weber  syndrome. Katmandu  University  Medical  Journal  2004; 2(4)8:372-74.Conclusion:Each  case  of  SWS  is  unique  and  exhibits  the characterizing findings to varying degrees. While social stigma is lessened by the absence of PWS, the unknown natural course of the syndrome is still  frustrating  for  parents  and  professionals treating the condition. So oral physicians should be aware of the signs and symptoms associated with SWS. Many times we may require consulta-tion of neurophysician, dermatologist, a plastic surgeon,  a  neuropsychologist,  pediatrician  and genetic specialist, before attempting any dental procedures, to prevent complication and to give definitive treatment.

Pyogenic Granuloma in a Patient of Sturge-Weber Syndrome with Bilateral Port Wine Stain- A Rare Case Report

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