Cleft lip and cleft palate are birth defects where the tissues of the lip and roof of the mouth do not fully fuse during development in the womb. They occur in approximately 1 in 700 births. Cleft lip involves a split in the upper lip while cleft palate involves an opening in the roof of the mouth. Treatment involves surgery to repair the cleft, with lip surgery typically within the first few months and palate surgery between 6-12 months. Ongoing care from a multidisciplinary team is often required and may include management of feeding issues, speech therapy, dental care and psychological support.
cleft lip and palate are the most common type of congenital anomalies. the worldwide prevalence of cleft lip and cleft palate ranges from 0.8 to 2.7 cases per 1000 live births. cleft lip is called cheiloschisis and cleft plate is called palatoschisis. Cleft lip is a gap or indentation in the lip or split continued up to the nostril due to the failure of fusion of the maxillary and medial nasal process.
Cleft palate is the condition in which the two plates of the skull that forms hard palate are not completely joined due to the failure of fusion of the lateral palatine processes, nasal septum and medial palatine process. EMBRYOLOGYPrimary palate forms during the 4-7th week of gestation when two maxillary processes and two medial nasal processes fuse.
Secondary palate forms in 6-9th weeks of gestation when palatal shelves change from vertical to horizontal position and fuse. Tongue migrates Antero-inferiorly.
Cleft lip occurs when an epithelial bridge fails.
Clefts of primary palate occur anterior to incisive foramen and clefts of secondary palate occur posterior to the incisive foramen.
ETIOLOGY Genetic: Non-syndromic inheritance (risk increases with parents or siblings or both affected); chromosome aberrations, associated with other syndromes like Van der Woude syndrome.
Environmental teratogens: Intrauterine exposure to the anticonvulsant phenytoin, alcohol, retinoic acid, maternal smoking, Rubella virus, thalidomide, aminopterin.
Maternal/intrauterine condition: Maternal diabetes mellitus and amniotic band syndrome.
Advanced paternal age
Unknown
CLASSIFICATION Prof. Balakrishnan (1975) classified cleft lip and palate according to the Indian context and divided them into three groups.
Group 1: Only cleft lip, which may be unilateral (right/ left), bilateral, or midline.
Group 2: Only cleft palate, which may be which also can be unilateral (right/left), bilateral, or submucosa.
Group 3: Includes cleft lip, alveolus, and cleft palate, which can be unilateral, bilateral, or midline. LAHSAL system for the classification of cleft lip and/or palate (2005) modified by Royal College of Surgeons Britain: LAHSAL system is a diagrammatic classification of cleft lip and palate. According to this classification, the mouth is divided into six parts. LAHSAL code indicates a complete cleft with a capital letter and an incomplete cleft with a small letter.
CLINICAL FEATURES Cleft lip: Notched vermilion border and may involve alveolar ridge.
Cleft palate: Nasal distortion, exposed nasal cavities.
Misaligned teeth.
Passage of milk through nasal passages during feeding.
Recurrent ear infection.
Speech difficulties.
Poor weight gain and failure to thrive.
DIAGNOSIS Newborn examination at birth
Palpate with a gloved finger or visual examination flashlight
In-utero ultrasonography
PROBLEMS OR COMPLICATIONS OF A CHILD WITH CLEFT LIP AND CLEFT PALATE Immediate Problems:
Feeding difficulty:
Infant with an unrepaired cleft palate will have ...
cleft lip and palate are the most common type of congenital anomalies. the worldwide prevalence of cleft lip and cleft palate ranges from 0.8 to 2.7 cases per 1000 live births. cleft lip is called cheiloschisis and cleft plate is called palatoschisis. Cleft lip is a gap or indentation in the lip or split continued up to the nostril due to the failure of fusion of the maxillary and medial nasal process.
Cleft palate is the condition in which the two plates of the skull that forms hard palate are not completely joined due to the failure of fusion of the lateral palatine processes, nasal septum and medial palatine process. EMBRYOLOGYPrimary palate forms during the 4-7th week of gestation when two maxillary processes and two medial nasal processes fuse.
Secondary palate forms in 6-9th weeks of gestation when palatal shelves change from vertical to horizontal position and fuse. Tongue migrates Antero-inferiorly.
Cleft lip occurs when an epithelial bridge fails.
Clefts of primary palate occur anterior to incisive foramen and clefts of secondary palate occur posterior to the incisive foramen.
ETIOLOGY Genetic: Non-syndromic inheritance (risk increases with parents or siblings or both affected); chromosome aberrations, associated with other syndromes like Van der Woude syndrome.
Environmental teratogens: Intrauterine exposure to the anticonvulsant phenytoin, alcohol, retinoic acid, maternal smoking, Rubella virus, thalidomide, aminopterin.
Maternal/intrauterine condition: Maternal diabetes mellitus and amniotic band syndrome.
Advanced paternal age
Unknown
CLASSIFICATION Prof. Balakrishnan (1975) classified cleft lip and palate according to the Indian context and divided them into three groups.
Group 1: Only cleft lip, which may be unilateral (right/ left), bilateral, or midline.
Group 2: Only cleft palate, which may be which also can be unilateral (right/left), bilateral, or submucosa.
Group 3: Includes cleft lip, alveolus, and cleft palate, which can be unilateral, bilateral, or midline. LAHSAL system for the classification of cleft lip and/or palate (2005) modified by Royal College of Surgeons Britain: LAHSAL system is a diagrammatic classification of cleft lip and palate. According to this classification, the mouth is divided into six parts. LAHSAL code indicates a complete cleft with a capital letter and an incomplete cleft with a small letter.
CLINICAL FEATURES Cleft lip: Notched vermilion border and may involve alveolar ridge.
Cleft palate: Nasal distortion, exposed nasal cavities.
Misaligned teeth.
Passage of milk through nasal passages during feeding.
Recurrent ear infection.
Speech difficulties.
Poor weight gain and failure to thrive.
DIAGNOSIS Newborn examination at birth
Palpate with a gloved finger or visual examination flashlight
In-utero ultrasonography
PROBLEMS OR COMPLICATIONS OF A CHILD WITH CLEFT LIP AND CLEFT PALATE Immediate Problems:
Feeding difficulty:
Infant with an unrepaired cleft palate will have ...
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2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
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Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
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Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
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2.
Cleft lip and cleft palate refer to facial malformations that evolve
during embryonic development resulting in nonunion of the bones
and tissues of the lip and palate.
• Cleft lip and cleft palate may occur independently or together.
• There may be a combination of defects and degrees of
involvement.
• Facial defects are noticeable at birth.
Cleft lip (cheiloschisis) and cleft palate (palatoschisis), which can also
occur together as cleft lip and palate, are variations of a type of clefting
congenital deformity caused by abnormal facial development
during gestation. A cleft is a fissure or opening—a gap. It is the non-
fusion of the body's natural structures that form before birth.
Approximately 1 in 700 children born have a cleft lip or a cleft palate or
both. In decades past, the condition was sometimes referred to
as harelip, based on the similarity to the cleft in the lip of a hare, but
that term is now generally considered to be offensive.
Introduction
3.
Approximately 1 in 700 children born have a cleft lip
or a cleft palate or both. In decades past, the
condition was sometimes referred to as harelip,
based on the similarity to the cleft in the lip of a hare,
but that term is now generally considered to be
offensive.
Cont..
4.
Cleft lip is a birth defect that occurs when the tissues
of the upper jaw and nose do not join as expected
during fetal development, resulting in a split (cleft)
in the lip.
Cleft palate is a condition in which the two plates of
the skull that form the hard palate (roof of the
mouth) are not completely joined. The soft palate is
in these cases cleft as well.
Definition
5.
Cleft lip is classified according to its location and
severity:
Unilateral - (on one side of the lip). The unilateral cleft
may cause the nostril on that side to be tilted and lower
than the other nostril.
Bilateral - (on two sides of the lip). The bilateral cleft may
cause the nose to be broader and shorter than normal.
Complete - extends to the nose.
Incomplete -does not extend to the nose.
A cleft palate is first categorized according to whether it
affects the hard palate, the soft palate, or both.
The hard palate is the front part of the roof of the mouth.
The soft palate is the back part of the roof of the mouth.
In cleft palate , the roof of the mouth (Palate) has an
opening (cleft)that may go through to the nasal cavity .
Types of cleft lip AND
CLEFT PALATE
6.
Combination terms
Complete classification of a cleft palate combines all of the
appropriate terms. For example:
A cleft of the hard palate with an incomplete unilateral
cleft lip is a cleft in the front of the mouth and a cleft in
one side of the upper lip that doesn't extend to the nose.
A cleft of both the hard and soft palate with bilateral
complete cleft lip describes a cleft that extends from the
soft palate to the hard palate, and includes the alveolar
ridge. Both sides of the lip have clefts extending to the
nose.
Cont..
9.
There are many causes of cleft lip and palate.
Problems with genes passed down from one or both
parents, drugs, viruses, or other toxins can all cause
these birth defects. Cleft lip and palate may occur
along with other syndromes or birth defects.
A cleft lip and palate can:
Affect the appearance of the face
Lead to problems with feeding and speech
Lead to ear infections
Causes
10.
Combination of genetic and environmental factors; cleft
palate is found mostly in girls and cleft lip alone or
together with cleft palate is found mostly in boys
family history of cleft lip or palate and other birth defects.
Anti-convulsant drugs.
Maternal tobacco use.
Genetic predisposition.
Maternal alcohol consumption
Folic acid deficiency.
Maternal smoking.
Risk factors
12.
• Incompletely formed lip
• Opening in roof of the mouth can be palpated
• Unilateral or bilateral cleft lip
• With cleft palate, milk or formula escapes through
the nose
• Abdominal distention due to swallowing air
• Slow weight gain
CLINICAL
MANIFESTATION
13.
Traditionally, the diagnosis is made at the time of birth by
physical examination. Recent advances in prenatal
diagnosis have allowed obstetricians to diagnose facial
clefts in utero.
Visual assessment of defects at birth and by palpation.
ultrasound to identify cleft lip or cleft palate in utero.
MRI to detect the extent of the abnormality; evaluation of
the infant’s ability to suck, swallow and breathe.
A genetic evaluation to determine reoccurrence.
Diagnostic evaluation
14.
CLEFT LIP -
Within the first 2–3 months after birth, surgery is performed to
close the cleft lip. While surgery to repair a cleft lip can be
performed soon after birth, often the preferred age is at
approximately 10 weeks of age, following the "rule of 10s"
coined by surgeons Wilhelmmesen and Musgrave in 1969 (the
child is at least 10 weeks of age; weighs at least 10 pounds, and
has at least 10g hemoglobin). If the cleft is bilateral and
extensive, two surgeries may be required to close the cleft, one
side first, and the second side a few weeks later. The most
common procedure to repair a cleft lip is the Millard
procedure pioneered by Ralph Millard. Millard performed the
first procedure at a Mobile Army Surgical Hospital (MASH)
unit in Korea.
Management
15.
Often an incomplete cleft lip requires the same surgery as
complete cleft. This is done for two reasons. Firstly the
group of muscles required to purse the lips run through
the upper lip. In order to restore the complete group a full
incision must be made. Secondly, to create a less obvious
scar the surgeon tries to line up the scar with the natural
lines in the upper lip (such as the edges of the philtrum)
and tuck away stitches as far up the nose as possible.
Incomplete cleft gives the surgeon more tissue to work
with, creating a more supple and natural-looking upper
lip.
18.
A blue line indicate incision.
Movement of the flaps; flap A is moved between B
and C. C is rotated slightly while B is pushed down.
Post-operation, the lip is swollen from surgery and
will get a more natural look within a couple of
weeks.
Cont..
19.
Often a cleft palate is temporarily covered by a palatal
obturator (a prosthetic device made to fit the roof of the
mouth covering the gap).
Cleft palate can also be corrected by surgery, usually
performed between 6 and 12 months. Approximately 20–
25% only require one palatal surgery to achieve a
competent velopharyngeal valve capable of producing
normal, non-hypernasal speech. However, combinations
of surgical methods and repeated surgeries are often
necessary as the child grows. One of the new innovations
of cleft lip and cleft palate repair is the Latham appliance.
The Latham is surgically inserted by use of pins during
the child's 4th or 5th month. After it is in place, the doctor,
or parents, turn a screw daily to bring the cleft together to
assist with future lip and/or palate repair.
CLEFT PALATE
21.
If the cleft extends into the maxillary alveolar ridge, the
gap is usually corrected by filling the gap with bone
tissue. The bone tissue can be acquired from the patients
own chin, rib or hip.
Lantham appliance
when the infant is without any infections
(respiratory, oral, or systemic), surgical treatments begin
early and continue into adolescence; scheduling depends
on the severity of the cleft.Chiloplasty used for correction
of a cleft lip the involvement of a multidisciplinary team
is needed: pediatrician, plastic surgeon, orthodontist,
audiologist, speech therapist, prosthodontist, and
psychiatrist.
Speech and hearing
A tympanostomy tube is often inserted into
the eardrum to aerate the middle ear. This is often
beneficial for the hearing ability of the child.
Cont..
22.
Children with cleft palate typically have a variety of
speech problems. Some speech problems result
directly from anatomical differences such as
velopharyngeal inadequacy. Velopharyngeal
inadequacy refers to the inability of the soft palate to
close the opening from the throat to the nasal cavity,
which is necessary for many speech sounds, such as
/p/, /b/, /t/, /d/, /s/, /z/, etc. This type of errors
typically resolve after palate repair.
Cont..
23.
Speech-language pathology can be very beneficial to help
resolve speech problems associated with cleft palate. In
addition, research has indicated that children who receive
early language intervention are less likely to develop
compensatory error patterns later.
Hearing loss
Hearing impairment is particularly prevalent in children with
cleft palate. The tensor muscle fibres that open the eustachian
tubes lack an anchor to function effectively. In this situation,
when the air in the middle ear is absorbed by the mucous
membrane, the negative pressure is not compensated, which
results in the secretion of fluid into the middle ear space from
the mucous membrane. Children with this problem typically
have a conductive hearing loss primarily caused by this
middle ear effusion.
24.
Hearing loss
Hearing impairment is particularly prevalent in children
with cleft palate. The tensor muscle fibres that open
the eustachian tubes lack an anchor to function effectively.
In this situation, when the air in the middle ear is
absorbed by the mucous membrane, the negative pressure
is not compensated, which results in the secretion of fluid
into the middle ear space from the mucous
membrane. Children with this problem typically have a
conductive hearing loss primarily caused by this middle
ear effusion.
25.
Six-month-old girl before going into surgery to have
her unilateral complete cleft lip repaired.
The same girl, 1 month after the surgery.
The same girl, age 8, the scar almost gone.
Cont..
26.
Assessment –
Assess respiratory status.
Monitor vital signs.
Check weight periodically to evaluate adequate
nutritional intake.
Clean suture line to prevent infection.
Use a gentle restraint to prevent the infant from placing
hands on its mouth.
Position infant so that rubbing of lip is prevented.
Use wrist restraints after cleft-lip repair and elbow
restraints following cleft-palate repair to prevent the
child from putting anything into the mouth.
NURSING CARE
PLAN
27.
intervention rationale
Assess respiratory status and monitor vital
signs atleast every 2 hours.
Position on side after feedings.
Feed slowly and use adaptive equipment as
needed.
Burp frequently.
Position upright for feedings.
Keep suction equipment and bulb syringe
at bedside.
Allows for early identification of problems.
Prevent aspiration of feedings.
Facilitates intake while minimizing risk of
inspiration.
Helps to prevent regurgitation and aspiration.
Minimizes passage of feedings through cleft.
Suctioning may be necessary to remove milk or
mucus.
Pre-operative :
NURSING DIAGNOSIS -High risk for aspiration(breast milk ,
formula ,or mucus) related to anatomic defect.
GOAL- Infant has no episodes of gagging or aspiration.
Expected outcome –Infants exhibits no signs of respiratory distress.
32.
COMPLICATIONS
Feeding problems.
Ear disease.
Speech problem
Problem in socialization.
Prognosis
A child born with a cleft lip with or without cleft palate
will require a multidisciplinary approach to management
throughout his or her development. In the absence of an
associated syndromic condition; however, children with
cleft lip and/or palate can have excellent esthetic and
functional outcomes.
Cont..
33.
HEALTH EDUCATION
Parents can be directed to various internet resources
for further information and advice
Parents should be advised on appropriate feeding
methods.
Feeding the infant in an upright position avoids
nasal regurgitation.
Bottle feeding can be optimized using specialized
fissured nipples and bottles controlling the flow rate
of pumped breast milk or formula.
34.
Soft-bodied bottles are squeezed in synchronization with
infant sucking to reduce the effort of feeding and
maximize the amount of feed entering the mouth.
The infant should be burped during pauses in feeding.
Feeding should not be continued for more than 30
minutes, to avoid the infant becoming tired.
Parents should be warned that the neonate will lose
weight (up to 10% of birth weight) after birth, but
reassured that the birth weight will be regained within 2
postnatal weeks, and increase by at least 1 ounce per day
thereafter. Infants should have their weight monitored at
weekly intervals until it has stabilized.
Parents should be provided with instruction on
appropriate postoperative care to protect the wound and
promote healing, with maintenance of good wound care
and nutrition as well as careful bathing and feeding.
35.
Prevention
Although the etiologies of cleft lip and palate are
multifactorial, several recommendations are suggested-
Avoidance of teratogenic medications during pregnancy
is mandatory.
seizure medications should be adjusted by a neurologist
before conception, to prevent teratogen exposure.
Prenatal multivitamins (including folic acid
supplementation),
Antismoking counseling.
Advice on the importance of minimizing perinatal alcohol
consumption are strongly encouraged.
Cont..
36.
Clefts of the lip and palate are generally divided into two
groups, isolated cleft palate and cleft lip with or without
cleft palate, representing a heterogeneous group of
disorders affecting the lips and oral cavity. These defects
arise in about 1.7 per 1000 liveborn babies, with ethnic
and geographic variation Typically, children with these
disorders need multidisciplinary care from birth to
adulthood and have higher morbidity and mortality
throughout life than do unaffected individuals. .
Prevention is the ultimate objective for clefts of the lip and
palate, and a prerequisite of this aim is to elucidate causes
of the disorders.
CONCLUSION
37.
BIBLIOGRAPHY
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002046/
http://www.nlm.nih.gov/medlineplus/ency/article/001051.h
tm
https://online.epocrates.com/noFrame/showPage.do?method
=diseases&MonographId=675&ActiveSectionId=21
http://www.healthlinkbc.ca/kb/content/special/aa112054.ht
ml
Peadiatric nursing.caring for children.Jane ball and Ruth
binder.Page no 486 -490.
Peadiatric nursing care plan.Jaypee.Page no 465.
Essential peadiatric nursing.Piyush gupta.2nd edition.Page no
466 -477.
Cont..