Cleft Lip

The  lateral nasal process forms the alar region of the nose. Normally the maxillary process moves medially and fuses with the medial nasal process at the philtrum on both sides of the upper lip. The fact that the cleft involves both the lip and bony primary palate  suggests failure of  the  maxillary process to fuse with the medial nasal process. The mandibular process does not normally fuse with either the lateral or medial nasal processes. That would reduce the size of the mouth. The lateral and medial nasal processes fuse to form a nostril separate from the oral cavity

Cleft lip  with or  without cleft palate can result from teratogenic exposure; it can be part of the fetal alcohol   syndrome; it can be inherited as a multifactorial disorder; or it can be part of an autosomal dominant, autosomal recessive, or  X- linked condition in all, more than 50 syndromes are associated  with  cleft  lip  with  or  without  cleft palate.  Computed  tomography (CT)  scan  of  the head, serum alcohol level, and urine toxic screen might be helpful in specific situations, depending on the physical examination and history. Cleft lip with or without cleft palate is not associated with amino acid disorders. When isolated, cleft lip with or  without  cleft  palate  is  considered  a multifactorial disorder, resulting from the effects of genes   contributed   by   both   parents   (genetic liability) and some nongenetic (environmental) factors, which often cannot be identified.

The incidence of trisomy 13 is about 1:12,000 live births, and 60% of affected individuals are female. Most infants with trisomy 13 have congenital anomalies that are incompatible with survival.

Surviving children demonstrate failure to thrive, cognitive disabilities, apneic spells, seizures, and deafness.

The symptoms and signs include characteristic features, often a normal birth weight, CNS malformations, eye malformations, cleft lip and palate, polydactyly or syndactyly, and congenital heart disease. 

Clefting of the lip and/or palate is felt to occur around the eighth week of embryogenesis, either by failure of fusion of the medial nasal process and the maxillary prominence or by failure of mesodermal migration and penetration between the epithelial bilayer of the face. 

• Increased parental age
• Drug use
• Infections during pregnancy
• Smoking during pregnancy
• Family history of orofacial clefting.

In the formation of the upper lip, the right and left medial nasal prominences merge to form the philtrum of the upper lip. The lateral maxillary prominence then merges with the merged medial nasal prominences. Failure of this merger to occur results in a unilateral cleft lip.

In the formation of the upper lip, the right and left medial nasal prominences merge to form the philtrum of the upper lip. The lateral maxillary prominence then merges with the merged medial nasal prominences. Failure of this merger to occur results in a unilateral cleft lip.

Cleft lip

B i.e. Medial Nasal Process

A i.e. Two medial nasal processes

Midline cleft lip is d/t failure of fusion between two medial nasal processes

Cleft lip

Ans. is `c’ i.e., Cleft lip

Disorders with multifactorial inheritance

o The multifactorial disorders result from the combined actions of environmental influences and two or more mutant genes having additive effects.

o Multifactorial disorders are ‑

1. Cleft lip/cleft palate                3. Coronary heart disease      5. Diabetes melitus          7. Pyloric stenosis
2. Congenital heart diseases 4. Hypertension                             6. Gout

Ans. is ‘a’ i.e., Cleft lip

Ans. is ‘d’ i.e., Isotretinoin

Isotretinon causes

o Craniofacial abnormality o CVS anomaly o CNS anomaly

                Cleft, lip & palate                     ASD                              Neural tube defect

                Microcephaly                                                               Microcephaly

Ans. is ‘c’ i.e., Combined with cleft palate 

Distribution of Cleft types Cleft lip alone   -> 15%Cleft lip & palate —> 45% Isolated cleft palate —> 40%

Ans. is ‘b’ i.e., 4^th day 

Stiches are removed on 5 post -op day

Ans. is ‘a’ i.e., Left side 

Ans. is ‘d’ i.e., Wardill’s method 

Following techniques have been used for cleft lip repair

– Thompson          

– Le Muserier           

– Tennison-Randall

– but ‘Rotation advancement technique’ by Millard is the most widely used method

Ans. is ‘b’ i.e., Maxillary process with medial nasal process 

Ans. is ‘a’ i.e., Posterior displacement of alar cartilage 

Ans. C: Cleft lip and cleft palate

The typical distribution of cleft types is:

• Cleft lip alone-15%
• Isolated cleft palate-40%
• Cleft lip and cleft palate-45%
• Cleft lip alone (hare lip): It is due failure of fusion between the frontonasal (philtrum) and the maxillary processes.

Types :

– Unilateral 85% usually on the left side 75%, bilateral 15%.

– Upper or lower: lower cleft lip is very rare and is due to failure of fusion between the mandibular processes. Complete or incomplete: according to whether it extends to the nostrils or not.

– Simple or alveolar: if it involves the bony alveolus.

– Complicated or not: If it is associated with cleft palate.

Complications:

– Disfigurement.

– Difficult suckling if it is complicated.

Treatment:

– Timing of surgery for cleft lip alone:

• Unilateral-one operation at 5-6 months
• Bilateral-One operation at 4-5 months.

– (Millard’s operation- When the patient is 10 weeks old, Hb = 10 gm% and the weight is 10 pounds (rule of ten)). II. Isolated cleft palate:

The palate is developed from

– Premaxilla from the frontonasal process.

– Two maxillary shelves: from the maxillary process

Cleft palate occurs due to failure (total or partial) of fusion between the maxillary and frontonasal process or the 2 maxillary processes.

Types :

–  Soft palate only, usually asymptomatic.

– Intermaxillary : the cleft is only between the 2 maxillary shelves.

– Bipartate : as intermaxillary but extends to one side of the premaxilla.

 Tripartate : as intermaxillary but extends to both sides of the premaxilla.

Complications:

– Impaired feeding due to:

• Difficult suckling.
• Wide pharynx : so the soft palate can’t close the nasopharynx during swallowing leading to nasal regurgitation of food, leading to pharyngitis, rhinitis, otitis media and deafness.

Difficult speech : specially consonant e.g. D, P, T, B, K, J.

– Teeth malformation.

Pierre Ribon syndrom : Cleft palate with receding lower jaw (micrognathia) and posterior displacement of the tongue which obstruct air way.

– Malformed nose.

Treatment :

In cases of cleft palate alone, surgical correction is indicated

• Soft palate only-One operation at 6 months
• Soft and hard palate-Two operations. One for soft palate at 6 months and another for hard palate at 15-18 months.

– In case of cleft lip and palate, surgical correction is done at;

• Unilateral- two operations-Cleft lip and soft palate at 5-6 months and hard palate and gum pad with or without lip revision at 15-18 months.
• Bilateral- two operations-Cleft lip and soft palate at 4-5 months and hard palate and gum pad with or without lip revision at 15-18 months.

Ans. is ‘d’ i.e., All the above

Following are the fetal or neonatal effects of amphetamines :

• IUGR
• Abruptio placentae
• Glassy eyed look
• Prematurity
• Hypoglycemia
• Lethargy
• Cardiac anamolies
• Sweating 
• Feeding problems
• Cleft palate 
• Poor visual tracing