Clinical and radiological findings related to tuberous sclerosis complex: a case report.

نویسندگان

  • Siddharth Gupta
  • Rahul Bhowate
  • Shirish S Degwekar
چکیده

AIM The aim of this report is to present the intraoral and extraoral clinical features of a young female patient with tuberous sclerosis complex (TSC) who exhibited multiple hamartomas of various organ systems including a fibromatous growth on the gingiva. BACKGROUND TSC is a rare neurocutaneous syndrome exhibiting multiple hamartomatous proliferations that may involve multiple organs such as the brain, kidney, heart, eyes, lungs and skin. Oral manifestations such as enamel pitting and fibromatous growth of the gingiva are commonly seen in patients with TSC. REPORT A 16-year-old female patient presented with a painless interdental gingival growth in the maxillary left incisor and canine region that bled occasionally. Associated findings were adenoma sebaceum (angiofibromas) on the face, a Shagreen patch on the lumbosacral region, ash leaf spots on the trunk, and subangual fibromas (Koenen's tumor) on the nails of fingers and toes. The final diagnosis of TSC was made on the basis of the clinical findings of the skin, computerized tomography (CT) findings of the brain and kidney, ultrasonographic findings of kidney, and a histopathologic evaluation of the gingival growth which met the major and minor criteria required for a diagnosis of TSC. SUMMARY This case report is a good example of the complex nature of patients with TSC. The diagnosis and management of these patients varies depending on the specific presentation of the disease. Dentists need to be mindful of the systemic issues as well as oral conditions related to this disease. Dental practitioners are advised to work closely with the patient's physician during treatment.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

A Patient with Tuberous Sclerosis Complex and Spinal Muscular Atrophy; A Case Report

Background Tuberous Sclerosis Complex (TSC), and Spinal Muscular Atrophy (SMA) are two inherited disorders while they are genetically independent. TSC is characterized by the formation of multiple hamartomas in nearly all organs. SMA is a destructive neurological disorder leading to progressive muscular weakness and atrophy. Case Presentation</e...

متن کامل

Cardiac Rhabdomyomas and Congenital Hypothyroidism: A Coincidence or Hamartia

Cardiac rhabdomyomas are the most common primary cardiac tumors in children. These tumors are generally asymptomatic, although they may be associated with neonatal tuberous sclerosis complex. Despite the fact that thyroid dysfunction rarely occurs in tuberous sclerosis, papillary adenomas (hamartomas) of the thyroid gland have been reported in a number of autopsies. Herein, we present the case ...

متن کامل

Association of Pemphigus Vulgaris and Tuberous Sclerosis: A case report

Pemphigus vulgaris is a blistering autoimmune disease with suprabasal cleft formation, which is the most common autoimmune blistering disease in eastern countries, such as Middle East. Predisposition to pemphigus is linked to genetic factors. Tuberous sclerosis is also a genetic disorder of hamartoma formation in many organs, particularly the skin, brain, eye, kidney and heart. We report ...

متن کامل

Radiological appearance of tuberous sclerosis complex: a case report and review of literature

Tuberous sclerosis complex (TSC) is a rare, auto-somal dominant, hereditary neurocutaneous syndrome, in which hamartomas develop in various organs. Cutaneous features and computed tomography (CT) findings are of great value in the diagnosis of TSC. The present study reported an unusual case of multiple organ abnormalities in a patient with TSC. Radiological findings indicated the presence of ce...

متن کامل

Urological conservative management of a patient with tuberous sclerosis complex (Bourneville disease).

OBJECTIVE To report the clinical and radiological data of a 23-year-woman with bilateral angiomyolipoma and Tuberous Sclerosis with conservative treatment. METHODS The patient underwent conservative urological management, based on clinical and complementary tests data (ultrasonography, CT scanning, excretory urogram, and laboratory). Monitoring was carried out in external consultation by the ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • The journal of contemporary dental practice

دوره 9 4  شماره 

صفحات  -

تاریخ انتشار 2008