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Peripheral giant cell granuloma in a child with ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome: a case

Aman Kumar1, Vinay Kumar Srivastava2, Sannu Sonal2

  • 1Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India. aman.aman.kumar44@gmail.com.

BMC Oral Health
|August 12, 2024
PubMed

Insights

Ectrodactyly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome can present with oral lesions like peripheral giant cell granuloma. Early dental intervention and surgical excision are key for successful management in affected children.

Area of Science:

  • Oral pathology
  • Pediatric dentistry
  • Clinical genetics

Background:

  • Ectrodactyly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome is a rare genetic disorder affecting ectodermal and mesodermal tissues.
  • Manifestations include split hands/feet, ectodermal dysplasia, and orofacial clefting, necessitating multidisciplinary care.
  • Dentists are crucial for identifying and managing oral conditions associated with EEC syndrome.

Observation:

  • A case report details a pediatric patient with EEC syndrome presenting with peripheral giant cell granuloma (PGCG) in the anterior mandible.
  • Diagnosis involved thorough medical/family history and clinical examination.
  • The PGCG lesion was surgically excised under local anesthesia.

Findings:

  • Surgical excision of the peripheral giant cell granuloma was performed successfully.
  • The patient experienced no recurrence of the lesion during a 24-month follow-up period.
  • This case underscores the importance of dental surveillance in children with EEC syndrome.

Implications:

  • Highlights the significant role of dentists in managing complex oral conditions in patients with EEC syndrome.
  • Demonstrates the effectiveness of surgical intervention for PGCG in this patient population.
  • Emphasizes the need for continued monitoring and early dental care for individuals with EEC syndrome to ensure optimal oral health outcomes.
Abstract

Keywords:
Cleft lipEctodermal dysplasiaPeripheral giant cell Granuloma

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