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cleft lip

16
Q. 1 You assist to deliver a full term baby boy who is otherwise normal but has a left cleft of the upper lip that extends upward toward the left nostril and left anterior cleft of the primary palate just deep to the cleft lip. These defects are most likely due to a failure of which of the following?
 A Mandibular process to fuse with the lateral nasal process
 B Mandibular process to fuse with the medial nasal process
 C Maxillary process to fuse the lateral nasal process 
 D Maxillary process to fuse the medial nasal process
Q. 2 A newborn boy has isolated cleft lip and palate. What is the most likely form of inheritance of this defect?
 A Autosomal recessive    
 B Autosomal dominant
 C X-Iinked                       
 D Multifactorial
Q. 3 A infant with cleft lip, cleft palate, polydactyly, microcephaly with holoprosencephaly, ectodermal scalp defect is suffering from:
 A Trisomy 21
 B

Trisomy 18
 C

Trisomy 13
 D

Turner syndrome
Q. 4

Repair of cleft lip should be undertaken at:

 A

4 weeks
 B

6 weeks
 C

8 weeks
 D

10 weeks
Q. 5

Millard repair is used for treatment of:

 A

Cleft lip
 B

Cleft palate
 C

Meningocele
 D

Saddle nose
Q. 6

A midline cleft lip is present when there is failure of fusion between the following structure?

 A

Mandibular processes
 B

Medial nasal processes
 C

Medial and lateral nasal process
 D

Medial nasal and maxillary process.
Q. 7

Which of the following disease cannot be diagnosed by amino centesis :

 A

Trisomy 21
 B

Duchenne’s
 C

Lesch Nyhan
 D

Cleft lip
Q. 8

Multifactorial inheritance is most likely to play a role in –

 A

Cleft lip
 B

Marfan’s syndrome
 C

Down’s syndrome
 D

Erythroblastosis fetalis
Q. 9

A new born developed cleft lip and cleft palate and atrial septal defect. Which of the following drug is likely to be consumed by the mother so as to cause such type of congenital anomalies –

 A

Digoxin
 B

Methanol
 C

ACE Inhibitors
 D

Isotretinoin
Q. 10

Commonest type of cleft lip is ‑

 A

Bilateral
 B

Midline
 C

Combined with cleft palate
 D

Unilateral
Q. 11

In cleft lip operation all the stitches are removed on ‑

 A

2nd day
 B

4th day
 C

10th day
 D

14th day
Q. 12

Unilateral clefts are most common on –

 A

Left side
 B

Right side
 C

Median
 D

None of the above
Q. 13

The following is the method for operating cleft lip All/Except-

 A

Le Mesurier’s method
 B

Tennison’s method
 C

Millard’s method
 D

Wardill’s method
Q. 14

Unilateral cleft lip is associated with –

 A

Posterior displacement of alar cartilage
 B

Columella elongated
 C

Always cleft palate
 D

Defective sucking
Q. 15

Most common congenital anomaly of the face is:

 A

Cleft lip alone
 B

Isolated cleft palate
 C

Cleft lip and cleft palate
 D

All have equal incidence
Q. 16

Amphetamine causes which of the following ‑

 A

IUGR
 B

Cardiac anamoly
 C

Cleft lip
 D

All the above
Q. 1 You assist to deliver a full term baby boy who is otherwise normal but has a left cleft of the upper lip that extends upward toward the left nostril and left anterior cleft of the primary palate just deep to the cleft lip. These defects are most likely due to a failure of which of the following?
 A Mandibular process to fuse with the lateral nasal process
 B Mandibular process to fuse with the medial nasal process
 C Maxillary process to fuse the lateral nasal process 
 D Maxillary process to fuse the medial nasal process
Ans. D
Explanation:

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

Q. 2 A newborn boy has isolated cleft lip and palate. What is the most likely form of inheritance of this defect?
 A Autosomal recessive    
 B Autosomal dominant
 C X-Iinked                       
 D Multifactorial
Ans.
D
Explanation:

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.

Q. 3

A infant with cleft lip, cleft palate, polydactyly, microcephaly with holoprosencephaly, ectodermal scalp defect is suffering from:

 A

Trisomy 21
 B

Trisomy 18
 C

Trisomy 13
 D

Turner syndrome
Ans.
C
Explanation:

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. 

 

The incidence of trisomy 18 syndrome is about 1:4000 live births, and the ratio of affected males to females is approximately 1:3. Trisomy 18 is characterized by prenatal and postnatal growth retardation that is often severe, and hypertonicity. Complications are related to associated birth defects. Death is often caused by heart failure or pneumonia and usually occurs in infancy or early childhood, although a small percentage of patients reach adulthood.

 

Characteristic features of Trisomy 21 include upslanting palpebral fissures, epicanthal folds, midface hypoplasia, and small, dysplastic pinnae.

 

Characteristic features of Turner syndrome (Monosomy X) include webbed neck, triangular facies, short stature, wide-set nipples, amenorrhea, and absence of secondary sex characteristics.

 

Ref: Tsaic A.C., Manchester D.K., Elias E.R. (2012). Chapter 37. Genetics & Dysmorphology. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

 

Q. 4

Repair of cleft lip should be undertaken at:

 A

4 weeks
 B

6 weeks
 C

8 weeks
 D

10 weeks
Ans.
D
Explanation:
Timing of Lip Repair: The classic “rule of tens” is still a reasonable guideline for lip repair:

10 weeks of age, a weight of 10 pounds, and hemoglobin of 10 have been considered prerequisites for lip repair.

 
This is partly based on anesthetic safety, which is probably a little better with increased age. The timing of the lip repair must be individualized for the patient.
 
 
Q. 5

Millard repair is used for treatment of:

 A

Cleft lip
 B

Cleft palate
 C

Meningocele
 D

Saddle nose
Ans.
A
Explanation:
The rotation advancement cleft lip repair, also referred to as a Millard repair, is probably the most commonly performed repair today.

Almost no tissue is discarded; the medial lip element is rotated downward, even with a back cut, if necessary, and the lateral lip element is advanced into the defect under the nasal sill.

Mucosal flaps are used to line the nose and the vestibule of the lip            
 
Q. 6

A midline cleft lip is present when there is failure of fusion between the following structure?

 A

Mandibular processes
 B

Medial nasal processes
 C

Medial and lateral nasal process
 D

Medial nasal and maxillary process.
Ans.
B
Explanation:

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. 

The cause of orofacial clefting is felt to be multifactorial. Factors that likely increase the incidence of clefting include,
  • Increased parental age
  • Drug use
  • Infections during pregnancy
  • Smoking during pregnancy
  • Family history of orofacial clefting.
Q. 7 Which of the following disease cannot be diagnosed by amino centesis :
 A

Trisomy 21
 B

Duchenne’s
 C

Lesch Nyhan
 D

Cleft lip
Ans.
D
Explanation:

Cleft lip

Q. 8

Multifactorial inheritance is most likely to play a role in –

 A

Cleft lip
 B

Marfan’s syndrome
 C

Down’s syndrome
 D

Erythroblastosis fetalis
Ans.
A
Explanation:

Ans. is ‘a’ i.e., Cleft lip

Q. 9

A new born developed cleft lip and cleft palate and atrial septal defect. Which of the following drug is likely to be consumed by the mother so as to cause such type of congenital anomalies –

 A

Digoxin
 B

Methanol
 C

ACE Inhibitors
 D

Isotretinoin
Ans.
D
Explanation:

Ans. is ‘d’ i.e., Isotretinoin

Isotretinon causes

  • Craniofacial abnormality o CVS anomaly o CNS anomaly
  • Cleft, lip & palate     
  • ASD                     
  • Neural tube defect
  • Microcephaly        
  • Microcephaly
Q. 10

Commonest type of cleft lip is ‑

 A Bilateral
 B Midline
 C

Combined with cleft palate
 D

Unilateral
Ans.
C
Explanation:

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%

Q. 11 In cleft lip operation all the stitches are removed on ‑
 A

2nd day
 B

4th day
 C

10th day
 D

14th day
Ans.
B
Explanation:

Ans. is ‘b’ i.e., 4th day 

Stiches are removed on 5 post -op day

Q. 12

Unilateral clefts are most common on –

 A

Left side
 B

Right side
 C

Median
 D

None of the above
Ans.
A
Explanation:

Ans. is ‘a’ i.e., Left side 

Q. 13

The following is the method for operating cleft lip All/Except-

 A

Le Mesurier’s method
 B

Tennison’s method
 C

Millard’s method
 D

Wardill’s method
Ans.
D
Explanation:

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

Q. 14 Unilateral cleft lip is associated with –
 A Posterior displacement of alar cartilage
 B

Columella elongated
 C

Always cleft palate
 D

Defective sucking
Ans.
A
Explanation:

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

Q. 15

Most common congenital anomaly of the face is:

 A

Cleft lip alone
 B

Isolated cleft palate
 C

Cleft lip and cleft palate
 D

All have equal incidence
Ans.
C
Explanation:

Ans. C: Cleft lip and cleft palate

  • Clefts of the lip, alveolus and hard and soft palate are the most common congenital abnormalities of the orofacial structures and are known to occur in 1 in 500 live white births.
  • Clefting of the lip and/or palate is felt to occur around the eighth week of embryogenesis, either by the failure of a fusion of the medial nasal process and the maxillary prominence or by the failure of mesodermal migration and penetration between the epithelial bilayer of the face.
  • Factors that likely increase the incidence of clefting include increased parental age, drug use, and infections during pregnancy, smoking during pregnancy, and a family history of orofacial clefting.
Q. 16 Amphetamine causes which of the following ‑
 A

IUGR
 B

Cardiac anamoly
 C

Cleft lip
 D

All the above
Ans.
D
Explanation:

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