Intraoral Myolipoma in a 6-Year-Old Child: A Rare Case Report.

Lipoma is the most common benign soft tissue mesenchymal neoplasm, which occasionally occurs in the oral cavity. It has many variants such as infiltrating lipoma, fibrolipoma, angiolipoma, spindle cell/pleomorphic lipoma, myxolipoma, chondroid lipoma, osteolipoma, and myolipoma. Myolipoma is a rare benign lipomatous soft tissue tumor which is diagnosed histologically because of presence of irregularly admixed mature adipose tissue and smooth muscle fibers. The most common site of its occurrence is retroperitoneum, abdominal and pelvic cavity, and the abdominal wall. Rarely myolipomas occur in the oral and maxillofacial region. Here, we present a rare case of intraoral myolipoma present in the buccal mucosa of a 6-year-old child.

Limbal dermoid in Nager acrofacial dysostosis: A rare case report.

Nager syndrome, also called preaxial acrofacial dysostosis, comprises two groups of defects involving the limbs and craniofacial region, respectively. This syndrome is rare and only 70 cases have been reported in the literature. The exact cause of this syndrome is unknown, but there is indication that it is genetically based. Ocular manifestations of this syndrome include widely separated downward slanting eyes, absence of eyelashes, ptosis of upper eyelids and colobomas on the inner aspect of lower eyelids. We report limbal dermoid in a patient with Nager syndrome. We did not find such an association of “Limbal dermoid in Nager acrofacial dysostosis syndrome” on PubMed using Nager acrofacial dysostosis, limbal dermoid and ocular manifestations as the keywords.

Effects of Consanguineous Marriages On Oral And Craniofacial Structures : A Study On Dental Patients In North India.

Background: Consanguinity describes a relationship between two people who share an ancestor, or share blood. These consanguineous marriages are a major risk to the health of offsprings till the extent that they can cause various craniofacial abnormalities, orofacial pigmentations and other abnormal birth defects. We conducted a study on patients visiting a dental college in North India to accesses the possible deleterious effects of consanguineous marriages on oral and craniofacial structures. Methodology: The study group comprised of all the patients visiting outpatient department of Kotiwal Dental college and Research centre, Moradabad in North India from March 2009 to February 2010. 200 patients showed positive history of consanguinity. They were carefully evaluated and all the diagnostic information was noted. Results: 66 out of 200 patients had systemic disorders like cardiovascular, pulmonary and renal disturbances. 56 patients had orofacial manifestations like orofacial pigmentations, craniofacial syndromes and occlusal abnormalities with or without systemic disorders. Conclusion:The prevalence of consanguineous marriages is still high. Consanguineous marriages are a major risk to the health of offsprings till the extent that they can cause various craniofacial abnormalities, orofacial pigmentations and other abnormal birth defects. They increase the autosomal recessive conditions through the expression of recessive deleterious alleles, especially in the offspring of first degree cousins

Prominent Orofacial Findings In Klippel - Trenaunay Syndrome.

Klippel-Trenaunay syndrome is a triad of congenital anomalies characterized by nevus flammeus, varicosities, and unilateral bony and soft tissue hypertrophy. Orofacial manifestations include facial asymmetry, jaw enlargement, and malocclusions as well as premature tooth eruption. Presented here is an illustrative report of Klippel Trenaunay syndrome in a 30 year old male patient showing all the characteristic findings.

Cowden syndrome.

Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

Blue rubber bleb nevus syndrome: Prominent oral findings.

Blue rubber bleb nevus syndrome is a rare disorder characterized by multiple cutaneous venous malformations in association with visceral lesions, most commonly affecting the gastrointestinal tract. Oral cavity lesions occur in 59 to 64% of cases. We report a unique presentation of this syndrome in a 25-year-old male patient with prominent oral findings. This is a sporadic case, started during early childhood, progressively increasing in number and size. Oral vascular lesions were part of gastrointestinal involvement. Associated cardiac abnormalities were also observed. An early diagnosis of this syndrome is required as it gets complicated with bleeding, anemia and other systemic complications.