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Congenital Defects of Spine and Limbs
1. SAHAJA VYADHI :- KHANDA
OUSTHA,KHANDATALU,SANNIRUDHA
GUDA,PADA VIKRUTI, SPINA BIFIDA,
MENIGOCELE, MENINGOMYOCELE
PRESENTED BY:-
PAVAN J. SORATHIYA
2. KHANDA OUSHTHA(OSHTABHEDA)
• INTRODUCTION:-
• This Is congenital condition illustrating the non fusion of lips at birth.
• तत्र खण्डौष्ठ इत्युक्तो वातेनौष्ठौ द्ववधा कृ तः। (A.H.U.21/3)
- Ayurveda attributes vaatakopaas the main causitive factor.
3. • CHIKITSA:-
~ Ashtangahridaya explains its managementas lekhana and then seevana.
- खण्डौष्ठस्य ववलिख्यान्तो स्यूत्वा व्रणवदाचरेत । (A.H.U.22/1)
• Lekhana
• Seevana
• Managementsimilar to vrana
• Abhyanjanawith taila prepared from madhuka,jyotishmati, lodhra, sraavani,
saariba,utpala,patolaand kaakamaachi.
• Nasyam with taila medicated with vatahara and madhura gana drugs.
• Sushruta, the father of surgery has described the surgery of lips being
similar to nasoplasty.
4. CLEFT LIP
• The developmental error in the formation of upper
lip will lead to the formation of cleft lip.
• The cleft lip(CL) is classified in several ways:-
5. • TREATMENT:-
• The operative treatment is the only choice for this
condition.
• When the baby attains 5-6Kgm of the body weight
or at the age of 3months or before the
commencement of primary dentitionoperation can
be performed.
• Cosmetic problems, difficulty in sucking, defective
dentition,defective speech and deformed nostrils
are the chief problem encounteredwith the cleft lip.
6. KHANDATAALU(CLEFT PALATE)
• INTRODUCTION:-
• Cleft palate is the condition due to the failure of
the fusion of the three basic components which
together from the palate.
• These are:- (i) the premaxilla, developing from
the medial nasal process, (ii) the two palatine
processes one from each maxillary process.
7. • TYPES:-
• It is of two types:- complete and incomplete.
• 1.Complete:- The two halves of the palate fail
to fuse in the entire length so that the nose
and mouth become interconnected.
• 2.Incomplete:- The fusions of the two halves of
palate proceed from before backwards. Here,
the anterior part of palate shows no
abnormality.
8. • COMPLICATIONS:-
• Difficulty in sucking
• Difficulty in eating
• Difficulty in speech
• Difficulty in hearing
• Dental problems
• Defect in smelling
• Repeatedrespiratory tract infection
• Chance of aspiration bronchopneumonia
9. • TREATMENT:-
• Surgery must be carried out at the age of one to one and half years of
age.
• Operations for repair of cleft palate:-
• 1.Langenbeck‘s operation:- • 2.Wardill’s operation:-
10. SANNIRUDDHA GUDA(ANAL STRICTURE)
• INTRODUCTION:-
• This is a deformity of the anal canal
characterized by narrowing of anus resulting
in difficulty on defecation.
• The predominant dosha is vata.
11. • FEATURES:-
• Sushruta explains the features of this disease in kshudrarogaadhyaaya.
• वेगसन्धारणाद्वायुवविहतोगुदमाश्रितः ।
ननरूणद्श्रध महत्स्त्रोतः सूक्ष्मद्वारं करोनत च ।।
मागिस्य सौक्ष््यात्कृ च्छ्रेण पुरीषं तस्य गच्छ्छनत ।
सन्न्नरूद्धगुदं व्याश्रधमेनं ववद्यात्सुदुस्तरम्।। (S.S.Ni.13/55-56)
• Here the apaanavayugetting vitiated at gudamarma resulted to the
stricture of anal canal, clinically expressed as difficulty for the defecation.
12. • CHIKITSA:-
• It is asaadhya roga.
• After pratyaakhyaaya,managementof niruddhaprakasahas to be
adopted.
• The features of Sanniruddhaguda are similar to those of stricture of
rectum; and dilatation by surgical means is the treatment of choice
13. IMPERFORATE ANUS(SANNIRUDDHA GUDA)
• INTRODUCTION:-
• This is a common congenital anomaly wherein the
post-allantoic gut(hindgut) and the
proctodeum(lower part of anal canal) are
imperfectly fused.
14. • TYPES:-
• Dependingon the level of termination of the bowel, it may broadly be
divided into two:-
• 1) Low anomaly:- In this bowel terminates below the pelvic floor. This type
includes only minor development errors and the anal sphincter mechanism
is not affected.
• 2) High anomaly:- Here, the bowel terminates above the pelvic floor.
15. LOW ANOMALY HIGH ANOMALY
i. Membranous stenosis
ii. Covered anus
iii. Stenosedanus
iv. Ectopic anus
v. Anal agenesis
i. Rectal atresia
ii. Anorectal agenesis
iii. Cloaca
16. 1) LOW ANOMALY:-
• Membranousstenosis:-Narrowed anal canal,coveredwith a thin
membrane.
• Coveredanus:- Anal openingis abnormally placed due to a covering skin
layer.
• Stenosedanus:- Narrowed anal canal with microscopic anus having
minute openings.
• Ectopicanus:- Normally developedanal canal with a thick skin covering
the anal opening.
• Anal agenesis
17. 2) HIGH ANOMALY:-
• Rectalatresia:-Noraml anal canal but ends proximally below the pelvic
floor
• Anorectalagenesis:-Anal canal not developed
• Cloaca:- Condition present only in females. Here hind gut, urinary bladder
and genital tract open into a common cavity.
18. • CLINICAL FEATURES:-
• Non passage of meconium is the major clinical feature present
• TREATMENT:-
• Surgical managementis indicated in all these conditions
19. PADA VIKRUTI (CONGENITAL TALIPES
EQUINOVARUS)
• INTRODUCTION:-
• Talipes:- Club foot
• Equinus:- Full dorsi flexion of foot is
preventeddue to fixation in planter
flexion
• Varus:- Foot is inverted and adducted
at midtarsal joint
20. • CAUSES:-
• Congenital and acquired causes.
• Congenital causes are of more significance in paediatrics and include:-
1. Environmentallfactor like intra-uterine compression
2. Genetic factor
3. Dysplasia
4. Drugs like Thalidomide
21. • PATHOLOGY:-
• Involves four major deformities:-
1. Equinus:- Flexion of the ankle joint
2. Varus:- Inversion of foot at subtalar joint
3. Adduction of forefoot
4. Medial rotation of tibia
22. • Degreesof deformity:-
• Three degrees of deformity are involved.
1.First degree:- Only addiction in forefoot
2.Second degree:- Adduction and equinus of foot
3.Third degree:- Adduction,equinus and inversion of foot.
• Changesin bone:-
• These are involved secondarily only.
• In general, displacementand rotation of the bones- talus, calcaneum,
navicular and cuboid takes place from their side.
23. • Changesin soft tissues:-
~ There are primary changes involved which are followed by bone changes.
~ It generally involves:-
• Shorteningand decreased growth of the muscles and tendons- involves
flexors of ankle, inverters of foot; tendo-achillis
• Shorteningand contracture of ligaments and capsules- involves planter
calcaeonavicular ligament, deltoid ligament of ankle.
• Shorteningof vessels and nerves on inferior and medial aspect of foot
• Skin- Callosities appear and later shorteningof skin is present.
24. • CLINICAL FEATURES:-
• More common in boys (male:female = 2:1)
• Generally, it is a unilateral condition
• Deformity itself is diagnostic.
25. • TREATMENT:-
• Planning of treatment is based on patient’s age. Accordingly, patients
can be grouped into three:- a) below 5 years, b) 5-10years, and c) above
10years.
• Below 5 years:-Correction of the deformity by manipulation.This is easy
in age group below 2months and can be done without anaesthesia.
Above 2months correction is resisted and early soft tissue operations eg.
Perkin’s operation,Brockman’s operation etc. are suggested
26. • 5-10years:- Bone deformity becomes prominent by this time and bone
operations are required, the most common being Evan’s operation.
• Above 10years:- Cuneiform tarsectomy is the conventionalmanagement.
27. SPINA BIFIDA
• INTRODUCTION:-
• It is the congenital defect of the spinal
cord involving the laminas. The
common anomaly involved here is the
midline defect of the vertebral bodies.
28. • TYPES:-
• Dependingon the severity of defect and
the protrusion of the contents of the canal
it is of following types:-
1.Spina bifida occulta
2.Meningocele
3.Meningomyelocele
4.Syringomyelocele
5.Myelocele
29. • SPINA BIFIDA OCCULTA:-
• It involves non fusion of the neural
arches,yet there is no protrusion of the
spinal cord or the meninges.
• The condition is mostly asymptomatic.
• In some cases patches of hair, lipoma,
discoloration of skin and dermal sinus in
middle low back may indicate the pathology.
• In some others, neurological defects like
foot drop, nocturnal enuresis or backache
may be presented.
30. • MENINGOCELE:-
• In this there occurs protrusion of the meninges
through the defect in neural arch fusion.
• The protrusion contains only the cerebrospinal fluid.
• It is most common in the lumbo-sacral region, may
occur in skull also.
• Most of these are well covered by skin and are
asymptomatic.
• Though, constipationand bladder dysfunction may be
presentedas late symptoms with increasing size of
the lesion.
31. • MENINGOMYOCELE:-
• Here, the spinal cord protrusion is
accompaniedby the meninges also.
• Neurological manifestationsare almost
always present.
• This may range from sensory disturbances
in initial stages, which may later advance
to extensive paralysis of the legs.
• Bilateral talipes and urinary incontinence
are common here.