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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