CASE DISCUSSION

CLEFT LIP
GENERAL DATA

• G.T.B
• 5 mos male
• Filipino
• Roman Catholic
• Born on July 09, 2019
• Residing in Valenzuela City
• Admitted in our institution on January 07,2020
CHIEF COMPLAINT

• Cleft lip
HISTORY OF PRESENT ILLNESS

Patient was born to a 38 year old G4P4 (4004) via CS delivery secondary to breech presentation at
Valenzuela General Hospital, full term, live baby, weighing 2.7 kg, with no fetomaternal complication.
Patient’s mother had a total of 8 prenatal checkups, 4 at local health center, 4 at Valenzuela General
Hospital, patient claimed intake of folic acid on the 1st trimester of pregnancy, (+) UTI, 6 months AOG,
completed 1 week of cefalexin, no history of GDM and hypertension, (+) breech presentation at 37 weeks
AOG, no history to exposure to radiation
HISTORY OF PRESENT ILLNESS

Patient was born with a right lip deformity, no associated symptoms. Patient sought consult at our
institution for repair of lip deformity hence admission for cheiloplasty with primary rhinoplasty
SALIENT FEATURES

• (+) Right lip deformity

PAST MEDICAL HISTORY:

• (-) Allergy
• (-) Measles
• (-) Chickenpox
• (-) Mumps
• (-) Seizure episodes
• (-) Bronchial Asthma
HEREDITARY/FAMILIAL DISEASES

• (+) Hypertension - Grandmother

• (+) Diabetes Mellitus - Grandmother
• (-) Bronchial Asthma
• (-) Pulmonary Tuberculosis
• (-) Seizure disorder
• (-) Psychiatric Disorder
• (-) Liver Disease
• (-) Thyroid Disease
IMMUNIZATION HISTORY

• BCG
• Hepatitis B x 3 doses
• DPT/OPV x 3 doses
• Hib x 3 doses
REVIEW OF SYSTEMS:
General:
(-) easy fatigability (-) weight change
Integumentary:
(-) pruritus (-) pigmentation (-) texture change (-) jaundice (-)skin rashes
Head and Neck:
(-) syncope (-) blurring (-) diplopia (-) photophobia (-)ear pain (-)discharge (-)tinnitus (-)vertigo (-)
taste disturbance (-) nasal obstruction (-) epistaxis (-) hoarseness (-) sore throat
Cardiovascular:
(-) palpitation (-)dyspnea (-) orthopnea (-) shortness of breath
Gastrointestinal:
(-)dysphagia (-) constipation (-) flatulence (-)hematochezia (-) steatorrhea
Genito – urinary:
(-) dribbling (-) incontinence (-) polyuria (-) oliguria (-) passage of stone (-) discharge
REVIEW OF SYSTEMS:
Musculoskeletal:
(-) swelling (-)bone deformity (-) seizures (-) weakness of extremities
Chest and lungs:
(-) difficulty of breathing
Neurologic:
(-) syncope (-)seizure (-) weakness or paralysis
Endocrine:
(-) polyuria (-) polydipsia (-) polyphagia (-) tremors (-) heat or cold intolerance
Hematologic:
(-) easy bruisability
PHYSICAL EXAMINATION:

General Survey:
• Conscious , Coherent, not in cardiorespiratory distress

HR: 101 bpm

RR: 29 cpm
T: 36.2 C
Weight: 2.7 kg
PHYSICAL EXAMINATION:
Skin:
Warm to touch, moist, good skin turgor, capillary refill time <2 seconds

HEENT:
Normocephalic , anicteric sclera, pinkish palpebral conjunctiva, no alar flaring, moist lips, buccal mucosa and
tongue, (+) right lip deformity, no tonsillo-pharyngeal congestion, no cervical lymphadenopathy

Chest and Lungs:

Symmetrical chest expansion, no retractions, clear breath sounds

Heart:
Adynamic precordium, normal rate , regular rhythm, no murmurs appreciated
PHYSICAL EXAMINATION:

Abdomen:
Globular, soft, nontender, normoactive bowel sounds

Extremities:
Grossly normal extremities, no cyanosis and no edema, full and equal pulses
INITIAL DIAGNOSIS:

• Cleft Lip, s/p cheiloplasty with primary rhinoplasty (January 08, 2020 JRRMMC)
COURSE IN THE WARD
 S O A P
Day1
COURSE IN THE WARD:
(-) fever
(-) cough
Awake, conscious Cleft lip  Monitor VS
 Ranitidine 12mg IV
(-) cold Anicteric sclera, pink palpebral conjunctiva once on NPO
(-) DOB (+) cleft lip, (-) cleft palate  NPO at 3am for
(-) N and V  NPO at 5am for clear
Symmetrical chest expansion, clear breath sounds liquid
Diagnostics:
Adynamic precordium, normal rate, regular rhythm, no • CBC c PC prior to OR
murmur

Abdomen soft, non tender

Full and equal pulses

HR: 107 bpm

RR: 25 cpm
T: 36.4 C
O2 sat: 98%
 S O A P
Day2 (+) fever Awake, conscious Cleft lip  Breast milk via
POST OP (-) cough dropper once awake
Day1 (-) cold Anicteric sclera, pink palpebral conjunctiva  s/p cheiloplasty with
(-) DOB (+) cleft lip, (-) cleft palate primary rhinoplasty
(-) N and V done
Symmetrical chest expansion, clear breath sounds  Referred to Pedia
 Medication:
Adynamic precordium, normal rate, regular rhythm, no • Paracetamol
murmur

Abdomen soft, non tender

Full and equal pulses

HR: 125 bpm

RR: 33 cpm
T: 37.2 C
O2 sat: 98%
 S O A P

Day3 (+) fever Awake, conscious Surgical site  Breast milk via
POST OP (+) cough infection, dropper once awake
Day2 (-) cold Anicteric sclera, pink palpebral conjunctiva subgaleal  s/p cheiloplasty with
(-) DOB (+) cleft lip, (-) cleft palate and primary rhinoplasty
(-) N and V epidural done
Symmetrical chest expansion, clear breath sounds abscess  Referred to Pedia
with  Medication:
Adynamic precordium, normal rate, regular rhythm, no osteomyeli • Paracetamol
murmur tis

Abdomen soft, non tender

Full and equal pulses

HR: 125 bpm

RR: 33 cpm
T: 37.2 C
O2 sat: 98%
ANCILLARIES

• CBC with platelet count

DIFFERNTIAL DIAGNOSIS
SALAMERO, JONNA
VAN DER WOUDE
VAN DER WOUDE
Rule In Rule Out
Gap in the lip (cleft lip) Small mounds of tissue on their lower lip.
Absent teeth (hypodontia) Lower lip pits alone

An opening in the roof of their mouth (cleft

palate)
ROBERT SYNDROME
ROBERT SYNDROME
Rule In Rule Out
Opening In The Lip With Or Without An Opening In The Roof Of The Mouth
Small Chin
Ear Abnormalities
Wide-set Eyes
Small Nostrils, And A Beaked Nose
ORAL – FACIAL – DIGITAL SYNDROME
ORAL – FACIAL – DIGITAL SYNDROME
Rule In Rule Out
Split in the lip Split in the tongue
Extra, missing, or defective teeth
Opening in the roof of the mouth
Wide nose with a broad, flat, nasal bridge
Widely spaced eyes
DIAGNOSIS

CLEFT LIP, RIGHT

CASE DISCUSSION
EVANGELISTA, EZEKIEL C.
ANATOMY
CLEFT LIP
MAXILLOFACIAL CLEFTS
CLEFT LIP AND CLEFT PALATE

• One of the most frequent serious congenital malformations

• Occurs one in every 700 births
• Family history / heredo – familial
• Cleft lip/palate (male patient) – 25%
• Cleft palate (female patient)
• Clefts (left side) – 70%
EMBRYONIC CONSIDERATIONS

• Normal: fusion of the medial nasal process with the MAXILLARY PROCESS from the:
• Upper lip
• Premaxilla
• Segments of the alveolar process
• Premaxilla/alveolus – PRIMARY PALATE
• Fusion of palatal process – SECONDARY PALATE
• Incisive foramen -> divides the primary and secondary foramen
CLEFT LIP
• Simplest kind of cleft which does not have the underlying cleft of the Premaxilla
• Notch may be present in the corresponding Alveolar Process
• INCOMPLETE CLEFT
• Extend from the Vermillon Border to just short of the floor of the Nasal Vestibule
• COMPLETE CLEFT
• Extend into the floor of the Nares
• Basic Deformities
• Depression of the Lateral Crus
• Deflection of the tip toward the cleft
• Shorter collumelar border
• Wider vestibular floor
• Diminished size of the Ala
UNILATERAL CLEFT LIP AND PALATE
COMPLETE

• Total lack of continuity of structure across the midline of the Palate on the side of the cleft and
Premaxilla separated from the Alveolar Process
• Palatal Process of the opposite side in fused with the nasal septum
• Vomer is horizontally inclined and attached to the palatal process on the normal side so that the oral
cavity communicates only with the nasal chamber of the cleft side
BILATERAL CLEFT LIP AND PALATE

• Anterior segment of the palate thus remains suspended from the anterior portion of the nasal septum
• Inferior margin of the vomer is Free and appears to be thickened but is vertical and in the midline
• Cleft throught the secondary palate is in the midline and divides at the incisive foramen to separate the
premaxilla from the end of the palate
• Termed as INCOMPLETE when the vomer is only partially attached to one of the palatine shleves
CLASSIFICATION OF CLEFT
PALATE

• The division between primary palate (prolabium, premaxilla, and

anterior septum) and secondary is the INCISIVE FORAMEN
• A – incomplete cleft of the secondary palate
• B – complete cleft of the secondary palate (extending as far as the
incisive foramen)
• C – incomplete cleft of the primary and secondary palates
• D – Unilateral complete cleft of the primary and secondary palate
• E – Bilateral complete cleft of the primary and secondary palate
MALFORMATIONS OF THE LIPS AND ORAL
CAVITY
Malformations of the lips, cheeks, and oral cavity (especially those involving the palate and oral floor) are of
EPITHELIAL ORIGIN. Although they are highly variable in their extent and clinical appearance, they are ALL based
on a common teratogenic mechanism

Epidemiology:
Cleft of the lip, palate, and alveolar ridge can occur in various combinations. They are among the most common
malformations with an incidence of 1 in 500

Classification:
• Cleft lip and alveolar ridge
• Cleft lip, alveolar ridge, and palate and isolated cleft palate. The bifid uvula
is a very mild variant of cleft palate
SYMPTOMS

• Different clefts have a spectrum of clinical manifestations, depending on the their morphology of extent:
• Hypernasal speech (rhinophonia asperta) due to incomplete closure of the nasopharynx
• Recurrent middle ear effusions and inflammations resulting from Eustachian tube dysfunction
• Variable abnormalities of the nasal septum (septal deviation) or in the shape of the nose
INCIDENCE

Of all of the maxillofacial clefts:

• Cleft lips with cleft palate – 50% (common to have bilateral cleft lip)
• Isolated cleft lip – 25% (more common on left side around 70% of cases)
• Isolated cleft palate - 25%
• About 85% of bilateral cleft lips and 70% of inilateral cleft lips are associated with cleft palate
DEMOGRAPHIC

Population
• White – 0.6 to 1.3 in 1000 births
• Orientals – 1.7 per 1000 births
• Black – 1 per 2500 births

• Cleft lip – Cleft palate : more common in MALES

PATHOPHYSIOLOGY
DAVID, ROSSMELL G
 In facial morphogenesis, neural crest cells migrate into
the facial region

They form the skeletal and connective tissue and all

dental tissue except the enamel
PATHOPHYSIOL
OGY
Cleft lip usually occurs at the junction between the
central and lateral parts of the upper lip on either side.
It may affect only the upper lip or may extend more
deeply into the maxilla and primary palate.

If the fusion of the palatal shelves is also impaired,

cleft lip is accompanied by cleft palate
• The upper lip is derived from Medial
nasal and maxillary processes.
• Failure of merging between the
medial nasal and maxillary processes
at 5 weeks gestation, on one or both
sides results in cleft lip.
RECOMMENDATIONS
AND DIAGNOSTIC
PROCEDURES
JOHN DARYLL L. UY
EARLY SECOND TRIMESTER DETECTION OF CLAP DEFORMITY THROUGH
ULTRASONOGRAPHY IS RECOMMENDED.
FOLIC ACID SUPPLEMENTATION IS RECOMMENDED PRIOR
TO CONCEPTION.
OTOACOUSTIC EMISSION (OAE) WITH OR WITHOUT
AUDITORY BRAINSTEM RESPONSE (ABR)
CEPHALOMETRIC RADIOGRAPHS FOR PATIENTS AGES 6 AND ABOVE (START OF MIXED DENTITION) AND
CANDIDATES FOR ALVEOLAR BONE GRAFTING IS RECOMMENDED.
PRESURGICAL APPLICATION OF NASOALVEOLAR MOLDING (NAM) FOR
CLEFT PALATE IS RECOMMENDED.
MANAGEMENT
MEDICAL AND SURGICAL
ERMITA, MYCA
MEMBERS OF A CLEFT LIP AND PALATE TEAM
USUALLY INCLUDE
• Plastic surgeon
• Orthodontist
• Dentist
• Prosthodontist
• Speech pathologist
• Otolaryngologist
• Audiologist
• Nurse coordinator
• Social worker/psychologist
• Geneticist
HOW ARE CLEFT LIP AND PALATE
TREATED?

• A cleft lip may require 1 or 2 surgeries, depending on the extent (complete or incomplete) and width
(narrow or wide) of the cleft. The first surgery is usually performed by the time a baby is 3 months old.
NON-INVASIVE

• A lip-taping regimen can narrow the gap in the child’s cleft lip.
• A nasal elevator is used to help form the correct shape of the baby’s nose.
• A nasal-alveolar molding (NAM) device may be used to help mold the lip tissues into a more favorable
position in preparation for the lip repair.
GUIDELINES BASED PRACTICE
RECOMMENDATIONS ON DIAGNOSTICS
AND PRE-SURGERY

1. Early second trimester detection of CLAP deformity through ultrasonography is recommended.

2. Folic acid supplementation is recommended prior to conception.
3. While Otoacoustic Emission (OAE) with or without Auditory Brainstem Response (ABR) is already done for
newborn hearing screening, Tympanometry is recommended to be added for patients with cleft palate.
4. Pre- and post-operative photodocumentation of patients with cleft lip and palate deformity may aid the
clinician in surgical planning and assessing surgical outcomes.
RECOMMENDATIONS ON DIAGNOSTICS
AND PRE-SURGERY

5. Cephalometric radiographs for patients ages 6 and above (start of mixed dentition) and candidates for
alveolar bone grafting is recommended.
6. Pediatric evaluation and clearance prior to surgical intervention to assess for other co-morbid
conditions is recommended.
7. Presurgical application of Nasoalveolar Molding (NAM) for cleft palate is recommended
SURGICAL MANAGEMENT OF THE
UNILATERAL CLEFT LIP-ALVEOLUS- PALATE
DEFORMITY

• 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.
RECOMMENDATIONS FOR PRIMARY
SUGICAL PROCEDURES

1. Cheiloplasty is done as early as three months

2. Alveoloplasty (soft tissue only) can be done with primary cheiloplasty or until the ideal age for bone
grafting is reached
3. Primary rhinoplasty can be done with primary cheiloplasty or until as early as 14 years old for females
and 16 years old for males which is the ideal age for definitive rhinoplasty.
RECOMMENDATIONS FOR PRIMARY
SUGICAL PROCEDURES

4. Palatoplasty can be done at 12 to 18 months.

5. Ventilation tube insertion can be done as indicated.
RECOMMENDATIONS FOR SECONDARY
SURGICAL PROCEDURES

• The secondary surgical procedures aim to improve on the aesthetic and other functional problems.
RECOMMENDATIONS FOR SECONDARY
SURGICAL PROCEDURES

1. Alveolar bone grafting can be done as indicated at 7 to 9 years old in consultation with the Orthodontist.
2. Palate re-repair/velopharyngoplasty can be done as indicated or whenever recommended by a speech
therapist.
3. Definitive rhinoplasty can be done as indicated, as early as 14 years old for females and 16 years old for
males.
RECOMMENDATIONS FOR SECONDARY
SURGICAL PROCEDURES

4. Lip revision can be done as indicated but not earlier than 3 months from previous lip surgery.
5. Orthognathic surgery can be done as indicated as early as 14-16 years old for females and 16-18 years
old for males
ADDITIONAL (CLEFT LIP REPAIR –
CHEILOPLASTY)
• 3 main deformities:
• Maxillary deformity
• Lip deformity
• Nasal deformity
• Basic Objectives of Repair
• Preserve
• Lengthen lip
• Reconstruct of the Orbicularis Oris muscle
• Correct distortion of alar base and columella
• Require Orthodontic Correction for deformed alveolus
• Precautions
• In general do not perform the following
• Perform any nasal tip modification concomitantly with cleft palate repair
• Damage or excise any nasal cartilage
• Perform simultaneous cleft palate repair
• Excise any normal landmarks
OPTIMAL AGE FOR OPERATION (RULE OF
OVER 10)

• Over 10 lbs
• Over 10 weeks
• Over 10 grams Hemoglobin

• 3 months (most opportune time)

• Surgeon’s “Primum non nocere” – when not sure seek HELP
CLEFT LIP REPAIR TECHNIQUES

• Rotation Advancement Flap (MILLARD technique)

• Rotation (downward) of cupids bow
• Advancement medially or upper rotation of lateral cleft
• Elongation of cleft (side columella)
• Independent correction of cleft (side alar base)
• Reconstruction of Orbicularis Oris muscle
CLEFT LIP REPAIR TECHNIQUES

• Rose Thompson (Straight line Closure)

• Tennison-Randall (Triangular Flap)

 CLEFT PALATE REPAIR (PALATORRHAPY)
• Reconstructive goals:
• Closure of oronasal
• Production of normal speech
• Ensurance of normal occlusion and facial growth
• Production of normal Eustachian tube Function

• High Points
• Adequate mobilization of lateral flare
• Preserve post palatine vessels
• Layer closure
• Reconstruction of Levator veli Palatini
• Palatal Lengthening with V-Y advancement

18 – 24 months optimal age for operations

CLEFT PALATE REPAIR TECHNIQUES
• Von Langenbeck technique (Secondary Cleft
Palate)

• Two – Flap Pataoplasty (V-Y Pushback)

SOURCES:

• Basic Otorhinolayngology A step-By-Step Learning Guide by Rudolf Probst, Gerhard Grevers, Heinrich Iro
(pages 69 to 96)
• Boies Fundametnals of Otolaryngology A textbook of Ear, Nose, Throat Diseases by Adams, Boies, Hilger
6th Edition (pages 273 to 370)
• Schwart’s Principle of Surgery 11th Edition