Cabato, Marlon Vincent

Mabalod, Nicole Recah

Sinon, Danica Alexis
Sta. Elena, Kervin Dondi
Solaiman, Amina
Tabbu, Honey Faye
Patient Profile
Name: S.P.
Age: 12 years old
Sex: Male
Address: Sta. Maria, Zamboanga City
Civil Status: Child
Occupation: Student
Religion: Islam
Nationality: Filipino
Chief Complaint

LIP AND PALATE DICONTINUITY,

RIGHT
 HISTORY OF PRESENT ILLNESS
 Patient is born with lip and palate discontinuity.
 No consults done.
 Patient tolerated his condition.
 As he would begin the school year, he opted for repair
of his condition thus this consult.
 BIRTH HISTORY
 Planned pregnancy.
 Delivered to a 29 year old G2P2 mother via NSVD at
the local health center.
 Uneventful delivery.
 Noted to have lip and palate discontinuity.
 No consult done.
 MATERNAL HISTORY
 Regular prenatal check up at local health center.
 With FeSO4 intake daily.
 No folic acid supplement.
 Denies maternal illness (such as hypertension and
diabetes mellitus)
 Denies intake of medications.
 Denies smoking and drinking alcoholic beverages
 PAST MEDICAL HISTORY
 No allergies to food or medications.
 No previous medical conditions.
 No previous surgeries.
 FAMILY HISTORY
 No history of cleft lip and palate in family.
 No HPN, DM, CA, BA.
 PERSONAL AND SOCIAL HISTORY
 Grade 4 student at a public school with average grades.
 Denies vices such as smoking, drinking alcoholic
beverages, and illicit drug use.
 Diet is mainly composed of rice, vegetables and fish
 REVIEW OF SYSTEMS
General
(-) easy fatigability (-) weakness (-) fever (-) weight gain

Skin
(-) rashes (-) itching (-) slow healing lesion

Head, Eyes, Ears, Nose, Throat (HEENT)

Head: (-) headache (-) dizziness (-) lightheadedness
Eyes: (-) edema (-) redness (-) blurring of vision, right eye (-) loss
of vision
Ears:(-) ear pain (-) tinnitus (-) hearing difficulties
Nose:(-) nasal stuffiness/obstruction/discharges (-) sinus pain (-)
colds
Throat/mouth:(-) dry mouth (-) bleeding gums (-) hoarseness (-)
non-healing sores
Neck
(-) tightness (-) pain (-) stiffness
Respiratory
(+) cough (-) hemoptysis (-) DOB
Cardiovascular
(-) chest pain (-) chest tightness (-) palpitations
Gastrointestinal
(-) nausea (-) dysphagia (-) change in bowel habits (-)
loss of appetite (-) vomiting
Genitourinary
(-) dysuria (-) change in urinary frequency (-) hematuria
(-) itchiness
Musculoskeletal
(-) weakness (-) stiffness (-) muscle/joint pain (-)
numbness in hands/feet
Neurologic
(-) history of fainting (-) loss of sensation (-) involuntary
movements (-) tingling/pain in hands/feet
Hematologic
(-) easy bruising (-) history of bleeding with unknown
cause
Endocrine
(-) excessive sweating (-) flushing (-) heat/cold
intolerance (-) excessive thirst/hunger
 PHYSICAL EXAMINATION
General:
Awake, ambulatory, cooperative, not in respiratory
distress.

Vital Signs:
Temp: 36.5 C Wt.: 34kg
R/R: 24 bpm Ht.: 135 cm
P/R: 72 bpm O2 sat: 98%
BP: 90/60 mmHg
 PHYSICAL EXAMINATION
 Skin:
No mass or lesions noted. Warm and dry to touch
with good skin turgor.
 Head:
Atraumatic head, symmetrical, no scars, no lesions, no
mass.
Eyes:
Anicteric sclerae, pink palpebral conjunctiva, PERRLA,
EOM’s intact.
 PHYSICAL EXAMINATION

 Ears: • TM
Inspection: Symmetrical, no gross Near total perforation AD, 10% central
ear deformities, no discharges, no perforation AS
swelling, no erythema, patent
EAC.
 PHYSICAL EXAMINATION
 Nose:
Widened alar base,
shortened columella,
nasal septum deviated to
non cleft side, absent
nasal floor
 PHYSICAL EXAMINATION
 Mouth:
Complete lip and palate
discontinuity, right
Absent uvula, (+) purulent
discharge on oropharynx
 PHYSICAL EXAMINATION
 Neck:
No tracheal deviation lymphadenopathies, no mass.

 Chest and Lungs:

Equal chest expansion, clear breath sounds, no
intercostal retractions.
 Heart:
Adynamic precordium, normal rate, regular rhythm,
no murmurs.

 Abdomen:
Flat, normoactive bowel sounds, soft, nontender.

 Extremities:
Good pulses, no deformity, no edema.
ADMITTING IMPRESSION

CLEFT LIP AND PALATE,

UNILATERAL, COMPLETE, RIGHT
(L3 A3 H3 S3 H0 A0 L0)
 BASIS
HISTORY
Patient is born with lip and palate
discontinuity.
No folic acid supplement.
PHYSICAL EXAMINATION
Complete lip and palate discontinuity,
right
Absent uvula, (+) purulent discharge on
oropharynx
Complete lip and palate discontinuity,
right
Absent uvula, (+) purulent discharge on
oropharynx
 PARACLINICALS
COMPLETE BLOOD COUNT
Hemoglobin 122 g/L
Hematocrit 0.35 %
RBC Count 4.3 x10^12/L
WBC Count 6.8 x10^12/L
Neutrophil 41 %
Lymphocytes 45 %
Eosinophils 4%
Monocytes 10 %
Basophils 0%
Platelet Count 321 x10^3/uL
Syndromes associated with CL/P
 Van der Woude syndrome
 Mutations in the IRF6 gene
 30%-79% of affected
individuals have CL/P
and/or Lower lip pit
 5%-29% have hypodontia,
hairlip and/or abnormal
salivary gland morphology
Syndromes associated with CL/P
 Velocardiofacial Syndrome
(Shprintzen’s Syndrome)
 caused by a deletion
(microdeletion) at the
q11.2 band, located on the
long arm of chromosome
22
 Congenital heart disease
 Defective Thymic
development
 Median Facial Dysplasia
 PHARYNGEAL OR BRANCHIAL
ARCHES
 The most distinctive feature in development of the
head and neck is the presence of pharyngeal
arches.

 appear in the 4th and 5th weeks of development and

contribute to the characteristic external appearance
of the embryo
 PHARYNGEAL ARCHES
 Bars of mesenchymal tissue separated by deep clefts -
pharyngeal clefts and,
a number of outpocketings - pharyngeal pouches

 The pouches penetrate the surrounding mesenchyme but

do not establish open communication with external clefts
PHARYNGEAL ARCHES
 FIRST
PHARYNGEAL
POUCH
• TUBOTYMPANIC
RECESS – EXTERNAL
AUDITORY MEATUS

• PRIMITIVE
TYMPANIC/MIDDLE
EAR CAVITY –
AUDITORY
(EUSTACHIAN) TUBE

• TYMPANIC MEMBRANE
/ EARDRUM
 SECOND
PHARYNGEAL
POUCH

• PALATINE TONSILS

• TONSILLAR FOSSA
 THIRD
PHARYNGEAL
POUCH

• INFERIOR
PARATHYROID GLAND
(DORSAL WING) –
DORSAL SURFACE OF
THE THYROID GLAND

• THYMUS (VENTRAL
WING) – MIGRATES
CAUDAL AND MEDIAL
AND ATROPHIES, LIES
BEHIND STERNUM
 FOURTH
PHARYNGEAL
POUCH

SUPERIOR PARATHYROID
GLAND
 FIFTH
PHARYNGEAL
POUCH
CONSIDERED TO BE PART
OF THE 4TH PHARYNGEAL
POUCH

ULTIMOBRANCHIAL
BODY – PARAFOLLICULAR
/ C CELLS OF THE
THYROID GLAND –
SECRETE CALCITONIN
Derivatives of the Pharyngeal Pouches
Pharyngeal
Derivatives
Pouch
1 Tympanic (middle ear) cavity

Auditory (eustachian) tube

2 Palatine tonsils

Tonsillar fossa

3 Inferior parathyroid gland

Thymus

Superior parathyroid gland ultimobranchial body

4
(parafollicular [C] cells of the thyroid gland)
 Prominences of the facial region:

FACE  Maxillary prominence

 Mandibular prominence
 Frontonasal prominence
 Formation of medial and lateral prominence
(nose)
UPPER LIP
 Fusion of two
maxillary
prominences
with the two
medial
prominences
NOSE
formed from five facial prominences:
 frontal prominence (bridge)
 merged medial nasal prominences (crest and tip)
 lateral nasal prominences (alae)
INTERMAXILLARY SEGMENT
 Formation: merger of two medial nasal prominences at the
midline
 Composition:
 Philtrum
 Upper jaw component (four incisor teeth)
 Palatal component (triangular primary palate)
PALATE
 Palatal shelves
fuse (from the
maxillary
prominences) to
form hard and
soft palate
 Structures Contributing to Formation of the
Face
Prominence Structures Formed
Forehead, bridge of nose, and medial
Frontonasal
and lateral nasal prominences
Maxillary Cheeks, lateral portion of upper lip
Philtrum of upper lip, crest, and tip of
Medial nasal
nose
Lateral nasal Alae of nose

Mandibular Lower lip

Facial / Palatal Clefts
EMBRYOLOGY
 The central upper lip is formed by growth of the
nasomedian process in a downward medial and
forward direction.

 Paired lateral maxillary processes grow medially

toward the descending nasomedian process. These
processes provide the lateral upper lip elements.
 Failure of the structure to unite on one side, or both,
provides clefts of the lip, extending into the nose and
through the alveolus between the lateral incisor and
cuspid teeth.

 Paired processes or shelves arise from the maxillary

processes. They converge upon the primary palate, on
each other, and the septum, to fuse (from front to back)
to provide the secondary palate.

 The lip and hard palate formation is completed by the

eight week of embryonic life and

 The soft palate and uvula are completed by the twelfth

week of embryonic life.
CLEFT LIP AND PALATE
Cleft lip and palate is a congenital anomaly with a wide
range of presenting variety of forms and combinations.
 CLEFT LIP
 hypoplasia of the mesenchymal layer resulting to failure
of the medial nasal and maxillary processes to join.
 CLEFT PALATE
 appears to represent failure of the palatal shelves to
approximate or fuse.
Cleft Lip
Partial or complete lack of
fusion of the maxillary
prominences with the medial
nasal prominence in one or
both.

Anterior
Lateral cleft lip
Cleft upper jaw
Cleft between the primary &
secondary palate.

Incisive foramen

Posterior
Cleft palate & cleft uvula
Cleft palate

• Lack of fusion of the

palatine shelves
• smallness of the shelves
• failure of the shelves to
elevate
• inhibition of the fusion
process
• failure of the tongue to
drop between the shelves
because of micrognathia
Oblique facial cleft

Failure of the
maxillary
prominences to
merge with its
corresponding
lateral nasal
prominence.

Lacrimal duct is
usually exposed.
Median cleft lip
 Rare abnormality
 Incomplete merging of the two nasal
prominences in the midline
 Usually accompanied by a deep
groove between the right & left sides
of the nose
 Associated with MR
 Holoprosencephaly – fused lateral
ventricles (3rd wk – neurolation)
ANATOMICAL ANOMALIES IN CLEFT
LIP DEFECT
 Orbicularis oris muscle with abnormal direction and
insertion
 Prolabial skin deficient
 Shortened columella, which deviates to the noncleft side
 Floor of the nose may be absent
 Central portion of the alveolar arch may be deficient
 Widened nasal tip is deflected to the noncleft side
 Septum and anterior nasal spine are displaced to the
noncleft side
DIAGNOSIS
 Thallwitz
Classification

 Descriptive
classification since
site, size, extent
and type of cleft are
considered.
 Each area is divided
into thirds, and
cleft defects are
graded as to extent
of affected areas.
 Veau System
 classifies cleft lip and palate deformities into four
classes, depending on whether the primary and/or
secondary palates are affected and by laterality

Veau Class I Incomplete cleft, soft palate only (no

unilateral/bilateral designation)
Veau Class II Hard and soft palate, secondary
palate only (no unilateral/bilateral
designation)
Veau Class III Complete unilateral cleft including
lip
Veau Class IV (primary and secondary palates)
Complete bilateral cleft
The ICD-10 system is an
international standard
of coding
COMPLICATIONS
 Feeding difficulties.
 Ear infections and hearing loss.
 Otitis media with effusion was found in 92-100% of
patients with cleft palate to have otitis media with
effusion.
 Speech and language delay.
 Dental problems.
 Displacement of the maxillary arches and malposition of
the teeth
 Velopharyngeal dysfunction
CLEFT LIP AND PALATE
 2nd most frequently occurring congenital
deformity
CLEFT LIP
 2:1000 live births (1997)
 0.46:1000 live birth (2008)
CLEFT LIP AND PALATE
 3rd most common birth defect in the Philippines
(1999-2001)
 110 cases
 5.6:1000 live births
 Clefts of the lip are more common in
males. (Nelson’s)
 the incidence is highest among Asians (~1 in 500)
(Nelson’s)
 When both parents are normal: the
probability of cleft first baby is one in
600.
 When both parents are normal and one
child has a cleft: the probability of a
second cleft child is one in 20.
 When both parents are normal and
both the first and second child have
clefts: the probability of a third cleft
child is one in four.
 When one parent has a cleft lip: the
probability of a cleft lip child is one in 20.
 When one parent and one child is cleft:
the probability of another cleft lip child is
one in four.
 When both parents have a cleft lip: the
probability of cleft in the first born is one
in four.
MANAGEMENT
 The aim of cleft surgery is to restore the entire
cleft defect to as near a normal anatomy as
possible. It is divided into primary and secondary
surgical procedures.
Cleft Lip
 Surgery (3 mo):
 Millard
 Tennison-Randall
 Fisher subunit repair corrections usually required later
on (especially for nasal deformity)
Millard
 The Millard procedure is known as the rotation-
advancement cheiloplasty technique. It is a more
flexible technique – cut as you go – but needs more
experience and artistry.
 It is the single most popular operative procedure in the
world for repairing the unilateral cleft lip.
 Favorable scar position. The technique ensures
preservation of the cupid's bow complex with
construction of a symmetric, unviolated philtral
column and retention of the central dimple hollow.
 Easy access to repair accompanying nasal
deformities. The classic cleft nasal deformity can be
addressed at primary cheiloplasty. The technique
provides direct access to the nasal cartilages
through the lateral advancement incision, and the
basilar columella through which the alar cartilages
can be completely mobilized for repositioning.
 Flexibility. The design of the operation can be
altered, and the surgeon is not forced to accept a
predetermined, inflexible plan, dependent on
accurate inviolate measurements.
 The disadvantage however is that one can easily get a
vertical scar contracture with vermilion notching of
the lip or lowering of the alar base.
 Horizontal scar contracture provokes a tendency
towards a small nostril. Excessive narrowing of the
nostril is never far from reality and the surgeon should
simply aim for a slightly larger nostril on the cleft side.
Tennison-Randall
 It is known as a geometrical design requiring exact
pre-surgical measurements. .
 The operation is done strictly on mathematical
principles and measurements and should lead to
pleasing surgical results.
 There is little room for surgical flexibility and artistry.
This is most of the time and for most of us an
advantage since errors in surgical artistic licence are
common and never far away.
 An important advantage of the Tennison-Randall
procedure is the lip length- ening effect between the
alar base and the Cupid’s bow on the affected side
(distance 8-10).
 The disadvantage however is the disturbance of the
aesthetic unit of the CS philtrum column in the lower
third. This is a violation of a known anatomic subunit,
but not always that obvious. The philtrum dimple has
a tendency to be more at in the classical Tennison.
This is no longer the case if the basket- weave method
of interlacing the orbicular muscle is used.
Cleft palate
 Special bottles for feeding
 Speech pathologist
 Surgery (6-9 mo):
 Von Langenbeck or Furlow Z-Plasty
 ENT consult – often recurrent otitis media, requiring
myringotomy tubes
 The primary reason for repairing a cleft palate is to
provide a mechanism for normal speech, and this is
believed by many surgeons to be the most critical
operation for children with clefts.
 There are three general approaches for handling the
mucoperiosteum in cleft palate repair, with many
variations of each:
 bipedicle mucoperiosteal flaps
 bilateral peninsular flaps with skeletonization of the
greater palatine neurovascular bundles to optimize
tissue mobilization
 double-opposing Z-plasty repair, which does not
include lateral relaxing incisions, but rather depends
on the medial mobilization of the mucoperiosteum.
Von Langenbeck
 It is the most common and popular method for the
repair of cleft palate.
 It involves the development of flaps based on greater
palatine neurovascular bundle bilaterally followed
by closing the defect in a layered manner i.e nasal
layer followed by oral layer.
Furlow Z-Plasty
 The furlows double opposing Z-plasty repair of cleft
palate was first described by Leonard Furlow at the
southeastern society of plastic & reconstructive
surgery meeting
 It is based on the hard palate closure in one procedure
without push back and closure of soft palate with
mirror image Z- plasty which allows a repositioning
and an overlap of the palatal muscles to form a palatal
muscle sling.
 Advantages include improved speech results with less
hypernasality and lower fistula rates.
 Disadvantage is related to the surgical aspect of Z
plasty with resulting increased operating time initially
for young surgeons.