for orthodontist

1. Gardner Syndrome
 Also known as familial colo-rectal polyposis
 1:15,000 affected
 Autosomal dominantform of polyposis. Typically, one parent has Gardner syndrome. Each
of their children, male and female alike, are at 50% risk of inheriting the gene for Gardner
syndrome.
 Mutation in the APC gene.
Sign and Symptoms
 characterised by the presence of multiple polyps in the colon together
 tumoursoutside the colon (may include osteomas of the skull, thyroid cancer, epidermoid
cysts, fibromas, desmoid tumours) in approximately 15% of affected individuals.
 Desmoid tumors are fibrous tumours covering the intestines. The countless polyps in the
colon predispose to the development of colon cancer; if the colon is not removed, the
chance of colon cancer is considered to be very significant. Polyps may also grow in the
stomach, duodenum, spleen, kidneys, liver, mesentery and small bowel.
 Cancers related to GS commonly appear in the thyroid, liver and kidneys. The number of
polyps increases with age, and hundreds to thousands of polyps can develop in the colon.
 Oral findings present in around 30% of patients: multiple impacted and supernumerary
teeth,multiple jawosteomaswhichgive a"cotton-wool" appearance to the jaws, as well as
multiple odontomas,hypodontia, abnormal tooth morphology and impacted or unerupted
teeth, fibrous dysplasia of the skull.
 Skeletal abnormalities, the most common of which are osteomas, are an essential
component of GS. The benign tumours are characterized by slow, continuous growth, and
occur most frequently in the mandible, the outer cortex of the skull and the paranasal
sinuses. The angle of the mandible is a particularly diagnostic site. The osteomas may be
either exostoses, often referred to as peripheral osteomas, or endostoses, which are
detectable only radiographically. The radiographic appearance of either type is a localized
radiopaque lesion with a sharp border. Another type of lesion has been described, which
appearsas a large and diffuse radiopaque cottonwool-likeareaineitherjaw,andisreferred
to as a widespread radiopaque lesion.
 The principal cutaneouslesionsare multiple epidermoid cysts, present in around 50%–65%
of patients. The cysts arise prior to puberty and occur primarily on the face, scalp and
extremities. Multiple sebaceous cysts of the scalp.
General Treatment
 There isno cure,and in itsmore advancedforms,itis consideredaterminal diagnosis with a
life expectancy of 35–45 years; treatments are surgery and palliative care, although some
chemotherapy has been tried with limited success
 After diagnosing these osteomas and adontomas, they must be resected under general
anesthesia,becauseof the unreachableregionslike paranasal sinuses, condylar and angular

2. region.Also,recurrence of osteomasand odontomasafterinadequate surgerycouldbe seen
and to eliminate possible occurrence, several surgeries could be performed.
 Histopathological evaluationof resectedtissuesfollowingsurgical intervention, followed by
other dental procedures are recommended. Recurrence is rare after surgical intervention.
Orthodontic treatment
 It isnot a valuable optionforthese patientsbecause osteomasand the increased density of
the bone would inhibit tooth movement.
 The densityof the bone isso dense toeruptimpacted tooth so surgical extraction would be
the most suitable alternative.
 Afterextractionof all impactedtooth conventional partial or total prosthetic rehabilitation
must be performed.
 Due to high bone density, long treatment interval is recommended

3. Acromegaly
Acromegalyisa rare syndrome thatresultswhenthe anteriorpituitaryglandproducesexcess
growthhormone afterepiphyseal plate closure atpuberty.If GH isproducedinexcesspriorto
epiphyseal plate closure,the resultisgigantism.A numberof disordersmayincrease the pituitary's
GH output,althoughmostcommonlyitinvolvesatumourcalledpituitaryadenoma. About3-4new
casesper millionpopulation.
Signs and Symptoms
Most commonlyaffectsadultsinmiddle age andcanresultinsevere disfigurementandfatal
complicationsif unchecked.Becauseof itspathogenesisandslow progression,itishardto diagnose
inthe earlystagesandis frequentlymissedforyearsuntil changesinexternal features,especiallyof
the face,become noticeable.
 Softtissue swellingvisiblyresultinginenlargementof the hands,feet,nose,lipsandears,
and a general thickeningof the skin
 Softtissue swellingof internal organs,notablythe heartwithattendantweakeningof its
muscularity,andthe kidneys,alsothe vocal cordsresultinginacharacteristicthick,deep
voice andslowingof speech
 Generalizedexpansionof the skullatthe fontanelle
 Pronouncedbrowprotrusion,oftenwithoculardistension(frontal bossing)
 Pronouncedprognathismwithattendantmacroglossia andteethspacing
 Hypertrichosis,hyperpigmentationandhyperhidrosismayoccurinthese patients
 Skintags
 Carpal tunnel syndrome
Complications
 Severe headache
 Arthritisand carpal tunnel syndrome
 Enlargedheart
 Liverfibrosisandbile duct
hyperplasia.
 Hypertension
 Diabetesmellitus(excessof GHleads
to insulinresistance)
 Heart failure
 Kidneyfailure
 Colorectal cancer
 Sleepapnoea,macroglossia
 Compressionof the opticchiasmleadingtolossof visioninthe outervisual fields
 Increasedpalmarsweatingandsebumproductionoverthe face (seborrhea)are clinical
indicatorsof active GH-producingpituitarytumors.Thesesymptomscanalsobe usedto
monitorthe activityof the tumor aftersurgery,althoughbiochemical monitoringis
confirmatory.
Diagnosis
 IGF1, GH suppresiontestandoral gluscose loadtesting
 MRI testingof sellawithgadoliniumuptake
 Changingfacial features
Treatment
 Somatostatin whichstopsGH production ordopamine agonistsif unresponsive
 Surgeryto excise pituitary tumour
 Radiationtherapy