Congenital Spine Deformity

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    20-Sep-2015

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Congenital Spine Deformity

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<ul><li><p>Congenital Spine DeformityNURYANI SIDARTA</p></li><li><p>Spina BifidaMENJELANG AKHIR MINGGU KE 4, SSP BERUJUD SEBAGAI TABUNG TERTUTUP YANG TERPISAH DARI EKTODERM YANG MENUTUPINYAKADANG-KADANG ALUR SARAF TIDAK MENUTUP SEMPURNABILA TIDAK TERJADI PENUTUPAN YANG SEMPURNA DI MEDULLA SPINALIS MAKA DISEBUT SPINA BIFIDA</p></li><li><p>Spina BifidaDefinisiAbnormalitas tulang belakang dimana terjadi penutupan tulang yang tidak sempurna dari 1 atau lebih lengkung neural (neural tube defect)Paling sering: daerah lumbosakral</p><p>Etiologi Genetikkurangnya intake asam folatTerdiri dari beberapa tipe</p></li><li><p>Spina Bifida2 PER ( 1000 BIRTHS (US)0.3 PER 1000 BIRTH (JAPAN)4.5 PER 1000 BIRTH (BRITISH)SOME EVIDENCE OF SEASONAL VARIATIONCONCEPTION IN MARCH AND MAY</p></li><li><p>Spina BifidaMORE COMMON IN FEMALES THAN MALESHIGHER IN CELTIC ORIGINRARE IN BLACK AND ASIANSLOWER SOCIAL CLASSHYPERTERMIA IN FIRST SEMESTERNUTRITIONAL DEFICIECIES : FOLIC ACID AND VITAMIN AGENETIC CONSIDERATION</p></li><li><p>SPINA BIFIDA</p></li><li><p>TYPE OF SPINA BIFIDASPINA BIFIDA OCCULTASPINA BIFIDA CYSTICA</p></li><li><p>SPINA BIFIDA OCCULTACHARACTERIZED BY A FAILURA OF ONE OR MORE OF THE VERTEBRAL ARCHES TO MEET AND FUSE IN THE THIRD MONTHS OF DEVELOPMENTTHE SPINAL CORD AND MENINGES ARE UNHARMED AND REMAIN WITHIN THE VERTEBRA CANAL</p></li><li><p>SPINA BIFIDA OCCULTATHE BONY DEFECT IS COVERED WITH SKIN THAT MAY BE MARKED WITH A DIMPLE, PIGMENTATION, OR PATCH OF HAIRTHE COMMON SITE : LUMBOSACRAL AREAUSUALLY ASSOCIATED WITH NO DISTURBANCE OF NEUROLOGICAL OR MUSCULOSKELETAL FUNCTIONING</p></li><li><p>SPINA BIFIDA CYSTICARESULTS WHEN THE NEURAL AND OVERLYING VERTEBRA ARCHES FAIL TO CLOSE APPROPRIATELYTHERE IS A CYSTIC PROTRUSION OF THE MENINGES OR OF THE SPINAL CORD AND MENINGES THROUH THE DEFECTIVE VERTEBRAL ARCHES</p></li><li><p>TYPE OF S.B. CYSTICAMENINGOCELEMYELOCELE OR MYELOCYSTOCELEMYELOMENINGOCELE</p></li><li><p>MENINGOCELEINVOLVES PROTRUSION OF THE MENINGES AND CEREBROSPINAL FLUID ONLY INTO THE CYSTIC SACTHE SPINAL CORD REMAIN WITHIN THE VERTEBRAL CANALRELATIVELY UNCOMMON</p></li><li><p>MYELOCELEPRESENT WHEN THE CENTRAL CANAL OF THE SPINAL CORD IS DILATED PRODUCING A LARGE SKIN COVERED CYSTTHE NEURAL TUBE APPEARS TO CLOSE NORMALLY BUT IS DISTENDED FROM THE CYSTIC SWELLINGRARE</p></li><li><p>MENINGOMYELOCELEMOST COMMON (94%) AND SEVERE FORMBOTH SPINAL CORD AND MENINGES ARE CONTAINED IN THE CYSTIC SACSENSORY AND MOTOR IMPAIRMENT AT THE LEVEL OF THE LESSION AND BELOWMOST COMMON IN THORACIS AND LUMBOSACRAL REGION50% : HIDROCEPHALUS</p></li><li><p>OTHER FORM OF S.BDISTEMATOMYELIALIPOMENINGOCELESACRAL AGENESIS</p></li><li><p>DIASTEMATOMYELIA30-40%IS SECONDARY TO PARTIAL OR COMPLETE CLEFTING OF THE SPINAL CORD</p></li><li><p>LIPOMENINGOCELEUSUALLY DUE TO A VERTEBRAL DEFECT ASSOCIATED WITH A SUPERFICIAL FATTY MASS THAT MERGES WITH THE LOWER LEVEL OF SPINAL CORDPROBLEM WITH URINARY CONTROL AND MOTOR CONTROL OF THE LOWER EXTREMITIES</p></li><li><p>LUMBOSACRAL AGENESISTHE ABSENT OF THE CAUDAL PART OF THE SPINE AND SACRUMNARROW FLATTENED BUTTOCOKS, WEAK GLUTEAL MUSCLES AND SHORTENED INTERGLUTEAL CLEFTNORMAL LUMBAR LORDOSIS IS ABSENTCHILDREN MAY HAVE SCOLIOSIS, MOTOR AND SENSORY LOSS</p></li><li><p>VISCERAL ABNORMALITIES, INCLUDING ANAL ATRESIA, FUSED KIDNEY, AND CONGENITAL HEART MALFORMATIONS</p></li><li><p>SACRAL AGENESIS</p></li><li><p>DIAGNOSISAnamnesis:Gangguan berkemih/BABGangguan gerak/ berjalan </p><p>Pemeriksaan Fisik:Benjolan pada tulang belakangKulit: dimple, hairy patch, pigmentasi, hemangiomaGangguan motorik ekstremitas bawah (LMN/UMN/Mix)Gangguan sensorikHidrosefalus </p></li><li><p>Pemeriksaan Penunjang:X-ray: defek pada tulang belakangAlfa feto protein serum meningkat saat hamil</p><p>Tata Laksana:Waspadai: infeksi CSS operasi penutupan defek hidrosefalus operasi pasang VP shuntGangguan sensorik: edukasi pencegahan ulcus decubitusmika, mikiGangguan motorik: edukasi pencegahan kontrakturInkontinensia urin: analisis urin </p></li><li><p>DETECTIONAMNIOCENTESISPRESENT OF SIGNIFICANT LEVELS OF ALPHA FETOPROTEIN IN THE AMNIOTIC FLUID80%PRENATAL SCREENING WITH USG</p></li><li><p>MALALIGNMENT OF VERTEBRA</p></li><li><p>WHAT IS PATHOLOGICAL CURVE?THE DEFORMITY OF THE SPINE IN ONE OR MORE OF ITS PLANES OF MOVEMENT</p></li><li><p>KYPHOSISABNORMAL CURVE OF THE SPINE IN THE SAGITAL CURVES WITH A POSTERIOR VERTEX (CONCAVE ANTERIORLY)</p></li><li><p>KYPHOTIC THORACAL</p></li><li><p>KYPHOSISNORMAL RANGE : 20-50 DEGREE (SRS)FON ET ALL : KYPHOSIS INCREASES ABOUT 4 TO 5 DEGREEE OVER CHILDHOOD TO THE END OF ADOLESCENCE</p></li><li><p>SCHEUERMANN DISEASEPAINLESS EXAGGERATED THORACIS KYPHOSISWITH COMPENSATORY LUMBER HYPERLORDOSISETIOLOGY : UNCERTAINAPPEAR TO BE A FAMILIAL LINKDUE TO REPETITIVE LOADING OF THE IMMATURE SPINE</p></li><li><p>RADIOGRAPHIC CRITERIAANTERIOR WEDGING OF AT LEAST THREE ADJACENT VERTEBRAEND PLATE IRREGULARITIESSCHMORLS NODESDISC SPACE NARROWING</p></li><li><p>LORDOSISAN ABNORMAL CURVE OF THE SPINE IN SAGITAL PLANE WITH EXCESSIVE POSTERIOR BENDINGLUMBAR LORDOSIS : 31-79 DEGREE (SRS)</p></li><li><p>HYPERLORDOTIC LUMBAL</p></li><li><p>HYPERLORDOTIC LUMBAL</p></li><li><p>SCOLIOSISGALEN DERIVED FROM THE GREEK WORD : SKOLIOS MEANING BENT, TWISTED OR CURVEDGALEN : ABNORMAL CURVE OF THE SPINE IN THE CORONAL PLATESIDIOPATHIC SCOLIOSIS : 0.3-2%</p></li><li><p>SCOLIOSISSCOLIOSIS RESEARCH SOCIETYANY CURVE THAT IS GREATER THAN OR EQUAL TO 10 DEGREES, WITH OR WITHOUT A ROTATORY COMPONENT</p></li><li><p>CONGENITAL SCOLIOSISDISORDER OF BONY FUNCTIONDISORDER OF ELASTIC TISSUE DYSFUNCTIONDISORDER OF NEUROMUSCULAR DYSFUNCTION</p></li><li><p>DISORDER OF BONY DYSFUNCTIONCONGENITALTHE ABNORMALIES ARE PRESENT AT BIRTHCLINICAL EVIDENCE OCCURS WHEN THE CHILD GROWS ONE PART OF THE VERTEBRAL COLUMN GROWS FASTER THAN ANOTHERFAILURE IN FORMATION OR IN SEGEMENTATION OR BOT</p></li><li><p>HEMIVERTEBRADEFECT IN FORMATION20-40% ALSO ASSOCIATED WITH GENETIC DISORDER SUCH AS GENITURINARY ANOMALIES, CARDIAC ANOMALIES, KLIPPEL FEIL SYNDROME AND SPONDYLOTHORACIS DYSPLASIA47% SHOW MILD TO MODERATE PROGRESSIONONLY 38% SHOWED SEVERE</p></li><li><p>DISORDER OF SEGMENTATION</p></li><li><p>DISORDER OF FORMATION (HEMIVERTEBRA)</p></li><li><p>SCOLIOS CAUSED BY ELASTIC TISSUE DYSFUNCTIONOSTEOGENESIS IMPERFECTAACHONDROPLASIAMARFAN SYNDROMEEHLERS-DANLOS SYNDROME</p></li><li><p>MARFAN SYNDROMEAUTOSOMAL DOMINANT SYNDROMRESULTS FROM A DEFECT IN THE FIBRILLAR GENE AT CHROMOSOM 15q21DIFFUSE JOINT LAXITY AND LONG LIMBSCOLIOSIS : 52%, IN THORACIC AND LUMBAR REGIONRISK FOR SEVERE CURVES</p></li><li><p>MARFAN SYNDROME</p></li><li><p>SCOLIOSIS RESULTING FROM NEUROMUSCULAR DYSFUNCTIONSPINAL MUSCULAR ATROPHYCHARCOT MARIE TOOTHCEREBRAL PALSYMULTISYSTEM INVOLVEMENTPRESENCE OF CONTRACTION HIP, HIP DISLOCATIONS, SENSORY ABNORMALITIES, AND MENTAL RETARDATION AND PRESSURE ULCERS</p></li><li><p>IDIOPATHIC SCOLIOSIS1.5-3%ASYMETRIC ABNORMALITIES IN DELTOID MUSCLESDIFFERENCES LATERALIZATION BALANCE AND VESTIBULAR FUNCTIONLLD 1.2-5.2 CM</p></li><li>IDIOPATHIC SCOLIOSISINFANTILE :</li><li><p>INFANTILE&lt; 3 YEAR1%MALES &gt;FEMALESCOMMON CURVE PATTERN : T OR T-LMAY SPONTANEOUSLY RESOLVE</p></li><li><p>JUVENILLE3-10 YEAR12-215EQUAL UNTIL 6 YEARS THEN 1:8COMMON CURVE : T, T-LUSUALLY WILL NOT SPONTANEOUSLY RESOLVE</p></li><li><p>JUVENILE IDIOPATHIC SCOLIOSISTO BE ASSOCIATED WITH ADULT COR PULMONALE AND EATHTREATMENT SHOULD BEGIN WHEN CURVES REACH APPROXIMATELY 25 DEGREE</p></li><li><p>ADOLESCENT IDIOPATHIC SCOLIOSIS10 YEAR SKELETAL MATURITY80-90%MALE : FEMALE : 1:5COMMON CURVE : T,T-LDEPENDENT ON AGE OF ONSET AND SIZE OF CURVE</p></li><li><p>MANAGEMENTEXERCISEPHYSICAL MODALITIESORTHOTIC MANAGEMENTSURGERY</p></li><li><p>SELAMAT BELAJAR</p></li></ul>