Our case is the Gardner's syndrome
, which is a life-threatening disease, because it holds 100% risk of malignant transformation.
Absence of colorectal polyps, skeletal abnormalities and multiple impacted or supernumerary teeth in this case, was against labelling it as Gardner's syndrome
. Table 1 shows the summary of clinical and radiological features present in our case and some recent studies (7-11).
(12) A relationship also exists between aggressive fibromatosis and Gardner's syndrome
; patients with Gardner's syndrome
can also exhibit multiple desmoid tumors.
Lack of abnormal skin elasticity and hypermobile joints ruled out the possibility of Ehlers-Danlos syndrome clinically which may be associated with multiple supernumerary teeth.8 Other syndromes associated with multiple supernumerary teeth are Gardner's syndrome
, Down syndrome and noonan's syndrome.9 Both clavicles were absent in our patient which allowed approximation of shoulders in front of chest.
They can be divided into five subgroups: extraabdominal, intraabdominal, multiple, multiple familial and as part of Gardner's syndrome
. Extraabdominal desmoid tumors have a wide distribution; the shoulder girdle, abdominal wall and lower extremities are most commonly involved.
(4) Other associated conditions include familial polyposis syndrome, Gardner's syndrome
, (1) estrogen therapy and trauma.
Differential gene expressions by dental follicle needed for osteoclastogenesis, osteogenesis and pressure from underlying succedaneous teeth are responsible for timely shedding and eruption process of succedaneous teeth (3) and several local, systemic causes including syndromes (Cleidocranial dysplasia, Gardner's syndrome
) and metabolic or hormonal diseases have been attributed in literature for impacted and embedded teeth.
is a genetic disorder that causes adenomatous colon polyps and leaves patients prone to epidermoid cysts, desmoid tumors, and congenital hypertrophy of the retina.
. Intra-abdominal desmoid tumors are often associated with Gardner's syndrome
, which is an autosomal condition that is characterized by polyposis coli, osteomas, cutaneous or follicular cysts, and fibromas.
(1,2) Rare familial cases have also been reported which can be associated with familial conditions like myotonic dystrophy and Gardner's syndrome
(43) Epidermoid cysts of the head and neck can be associated with Gardner's syndrome
, with the lesions frequently affecting the scalp, face, and soft tissues of the neck.