The document details various developmental disturbances affecting the lip and palate, including conditions such as Van der Woude’s syndrome and cleft lip/palate malformations, their etiology, clinical features, and associated treatments. It also discusses lesser-known conditions like cheilitis glandularis and cheilitis granulomatosa, highlighting their symptoms, differential diagnoses, and treatment options. The information presented emphasizes the genetic factors, developmental disruptions, and the importance of surgical interventions in these congenital conditions.

1. DEVELOPMENTAL DISTURBANCES
AFFECTING LIP AND PALATE
Dr. Reshma M
Assistant Professor
Dept of Oral and Maxillofacial Pathology

3. Congenital lips and commissural pits
and fistulas
• Malformations of the lip with hereditary pattern
• Van der Woude’s syndrome: pits of lower lip with cleft lip and/or cleft palate.
• Commissural pits: related to lip pits but occurs in commissures lateral to
typical lip pits
• Etiology : in early stages of development fixation of tissue to the base of
notch or from failure of complete union of the embryonic lateral sulci of the
lip.

4. Bilateral lip pits
Commissural lip pits

5. CLINICAL FEATURES
• Unilateral /bilateral
• Vermilion surface of either lip
• Common in lower lip
• Some times appears swollen accentuating appearance of lips
• Commissural pits are unilateral or bilateral
TREATMENT
Surgical excision

6. Van der Woude’s syndrome
• Autosomal dominant
• Cleft lip/cleft palate+ pits of lower lips
• Etiology : deletions in chromosome band 1q32
• Clinical features :
Unilateral/ bilateral
Hyper nasal voice and cleft or bifid uvula
Syngnathia(congenital adhesion of the jaws)
Narrow high arched palate
Ankyloglossia

7. EXTRA ORAL MANIFESTATION
Limb abnormalities
Popliteal webs
Brain abnormalities
Congenital heart defects
TREATMENT
Surgical repair of cleft lip and palate
Surgical removal of lip pits

8. Cleft lip and cleft palate
• Two types of malformation : cleft lip with or without cleft palate and cleft
palate
• Etiology : Hereditary
By single gene/ by number of genes (polygenic inheritance)
Polygenic : Most common type , low risk
Monogenic : Associated with congenital abnormalities
Other factors : Defective vascular supply
Size of the tongue
Alcohol and certain drugs
Infections and lack of inherent developmental force

11. Clinical features
• Males
• Syndromic and non syndromic
• Unilateral or bilateral
• Median cleft face syndrome: hypertelorism, median cleft of premaxilla
and palate and cranium bifidum occultum.
• Clinical significance :
Esthetic, physical and psychological effects
• Treatment :
cosmetic and functional, 10 s

12. Chelitis glandularis
• Uncommon poorly understood inflammatory disease of the lip
• Progressive enlargement and eversion of lower lip
• Altered by environmental influences, leading to erosion, ulceration and
crusting.
• Etiology : chronic irritation
• Clinical features: Distinct deeply suppurative chronic inflammatory condition
of lower lip
Equal gender predilection
classification : simple type- multiple,painless, papular surface lesions with
central depressions and dilated canals

13. • Superficial suppurative type(baelz disease) : painless indurated swelling of
the lower lip with shallow ulceration and crusting.
• Deep suppurative type: deep seated infection with formation of abscesses,
sinus tracts and fistulas
• Histological features: lip biopsy is indicated
• Differential diagnosis: actinic keratosis, chelitis granulamatosa, sarcoidosis
and squamous cell carcinoma
• Treatment : aetiology to be rule out and attempts to alleviate and eradicate
the cause.

14. Chelitis granulomatosa(miescher-
melkersson –Rosenthal syndrome)
• Swelling of lip due to granulomatous inflammation
• Melkersson Rosenthal when chelitis with facial palsy and plicated tongue
• Etiology : Genetic predisposition
• Clinical features : Episodic nontender swelling and enlargement of one or
both lips
Prodromal symptoms
Regional lymph nodes are enlarged

15. • Differential diagnosis: insect bites and sarcoidosis
• Histological features: chronic inflammatory cell infiltration ;peri and para
vascular aggregations of lymphocytes, plasma cells and histiocytes, non
caseating granuloma formation and Langhans type of giant cells
• Treatment : corticosteroid, NSAIDS,Surgery radiation

16. Thank you