cleft lip, cleft palate & spina bifida
TRANSCRIPT
Cleft Lip, Cleft Palate and Spina Bifida
SarathRoll No. 41
Development of face Starts from 4th Week Finishes by 10th Week
Development of face- formation of frontonasal process
Development of face- formation of mandibular & maxillary processes
Development of face- formation of lens placode, nasal placode and nasal pit
Development of face- naso-optic furrow
Development of face- Cheek
Development of face
Cleft Lip
Developmental error in the formation of lips due to defect In the fusion of PROCESSES of face.
Incidence 1:2000 live births
Etiology & Risk factors Increased Parental Age Infections During Pregnancy Smoking During Pregnancy First Child with Cleft lip Drugs- Steroids, Phenytoin, Diazepam, Alcohol, Smoking Maternal Diabetes Mellitus Nutritional Deficiency- Folic Acid Associated Syndromes- Pierre Robin’s Syndrome, Stickler’s Syndrome,
Shprintzen’s Syndrome, Down’s Syndrome, Apert’s Syndrome
Classification- based on position
Cleft Lip
Central Lateral
Unilateral Bilateral
Classification- based on extent
Cleft lip
Complete
Complicated Uncomplicated
Incomplete
Problems associated with Cleft Lip
Cosmetic problem Difficulty in sucking Defective dentition Defective speech particularly with labial letters B, F,
M, P, V
Muscle Rings of Delaire Nasolabial MusclesTransverse LabialisLevator Labii Superioris Alaeques nasiLevator Labii Superioris Bilabial MusclesOrbicularis Oris(Oblique Head)Orbicularis Oris(Horizontal Head)Orbicularis Oris(Lower Lip) Labiomental MusclesDepressor Anguli orisDepressor Labii InferiosisMentalis
Optimum time for repair Rule of 1010 lbs of weight10 weeks old10g% hemoglobin Advantages are:Avoids Defective DentitionFacilitates SuckingDefective Speech is avoidedWhen associated with cleft palate, it will reduce the gap of palate.
Surgical Techniques
Millard Blair Operartion
Cleft Palate
Complete Incomplete
Time of Surgery
Problems associated with Cleft palate Difficulties in sucking Difficulty in eating Difficulty in speech Difficulty in hearing Dental problems Defect in smelling
Treatment
Langenbeck’s operation Wardill’s operation
Feeding Difficulties
Cleft lip= makes it more difficult for an infant to suck on a nipple
Cleft Palate= may cause formula or breast milk to be accidently taken up into the nasal cavity
Inability to create negative pressure inside oral cavity
Frequent regurgitations Upper respiratory tract infections
Dental Problems Congenitally Missing teeth, Hypodontia,
Hyperdontia, Oligodontia Presence of natal and neonatal teeth Anamalies of tooth morphology like microdontia,
macrodontia etc Fused teeth Enamel Hypoplasia Poor periodontal support, early loss of teeth Spacing and crowding
Nasal Deformity and Esthetic Problems Facial Disfigurements Poor nasal shape Scar marks of surgeries Poor lip function during speech Poor dental alignment and smile
Ear Problems
Middle ear disease - 22% to 88% Conductive hearing loss and chronic suppurative otitis media may result Repeated tympanostomy tube placement
Classification Systems
Kernahan “Striped Y”
Millard Modification of Kernahan “Striped Y”
Kriens “LAHSHAL”
Lip
Alveolus
Primary PalateHard Palate (Maxillary)
Hard Palate (Palatine)
Soft Palate
Karnahan’s Classification Millard’s Modification of Karnahan’s Classifcation
L = Lip (right)A = Alveolus (right)H = Hard Palate (right)S = Soft Palate (median)H = Hard Palate (left)A = Alveolus (left)L = Lip (left)
Capital letter = complete cleftLowercase letter = incomplete cleft“.” or “-” = normalExamplesLA….l = complete right cleft lip and alveolus, incomplete left cleft lipLAHS = complete right unilateral cleft lip, alveolus, hard, and soft palate
Kriens LAHSHAL
Prenatal Diagnosis Cleft lip can be easily diagnosed
by performing ultrasonography in the second trimester
Diagnosing a cleft palate with ultrasonography is very difficult
Three-dimensional imaging has been introduced to prenatal ultrasonography diagnostics of cleft anomalies
Diagnosis Advantages of Prenatal Diagnosis:
1. Time for parental education2. Time for parental psychological
preparation3. Opportunity to investigate other
associated anomalies4. Gives parents the choice of continuing
the pregnancy5. Opportunity for fetal surgery
Spina Bifida
Failure of fusion of vertebral arches Incidence – 0.1% TypesSpina Bifida OccultaMeningoceleMeningomyeloceleSyringomyeloceleMyelocele
Spina Bifida Occulta
Failure of arches to unite, yet there is no protrusion of cord or membranes
Local patch of hair, depression of skin or naevolipoma
Membrana reuniens connects skin to spinal theca Usually symptomless Sometimes present with neurological deficiencies-
foot drop, nocturnal enuresis or backache
Membrana reuniens
Treatment
Excision of local lesion If there are neurological symptoms, membrana
reuniens is excised in its whole length
Meningocele
Protrusion of meninges occurs through the defect in neural arch
Content is CSF Common in lumbosacral region but also occurs in
occipital region or root of nose
Clinical features
Cystic swelling, so fluctuation +ve Highly translucent Compressible Expansile cough impulse +ve On palpation, edge of bony defect is palpable Neurological manifestations are usually absent
Investigations
X-Ray CT Scan to exclude hydrocephalus MRI to understand the relation of swelling with
neural structures
Treatment
Operate as early as possible Excise the cyst Redundant part is excised Margins of excised are sutured together in midline Erector spinae and overlying fasciae are
approximated over the gap Skin is closed
Menigomyelocele
Most common cause of spina bifida in living children Normally developed spinal cord or cauda equina
protrudes along with meninges and these structures are adherent to posterior aspect of sac.
Bony defect usually extends over 3 or more segments
Rupture is more common
Clinical Features
In trans illumination test, nerves may be seen in the sac
Neurological manifestations are always present-Sensory & motor deficits, urinary incontinence, hydronephrosis
Treatment
Operate as early as possible Excise the cyst Carefully separate the nerves Redundant part is excised Margins of excised are sutured together in midline Erector spinae and overlying fasciae are
approximated over the gap Skin is closed
Syringomyelocele
Central canal of spinal cord is dilated Rarest variety of spina bifida Gross neurological deficits and paralytic
manifestations are present
Myelocele
Most common type of spina bifida Gravest form of spina bifida Besides bony defect there is also defect in
development of spinal cord Majority cases are still born
Meningocele Meningomyelocele Syringomyelocele Myelocele
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