Group35SpinaBifida final.pptx

SPINA BIFIDA FARAH LOGDAY - TZM/10698 McDONALD KALIYANGILE - TZM/10293 CLINICAL CORRELATIONS 1 MODERATOR: DR MWAANGA Table of contents ❏ Introduction ❏ Embryology ❏ Etiology ❏ Epidemiology ❏ Pathophysiology ❏ Types ❏ Clinical Picture ❏ Investigations ❏ Differential diagnos...

SPINA BIFIDA FARAH LOGDAY - TZM/10698 McDONALD KALIYANGILE - TZM/10293 CLINICAL CORRELATIONS 1 MODERATOR: DR MWAANGA Table of contents ❏ Introduction ❏ Embryology ❏ Etiology ❏ Epidemiology ❏ Pathophysiology ❏ Types ❏ Clinical Picture ❏ Investigations ❏ Differential diagnosis ❏ Management ❏ Surgical options ❏ Complications ❏ References  Introduction ❏ Neural tube defect. ❏ Incomplete closing of the neural arches and meningeal layers around the spinal cord. ❏ Developmental congenital anomaly. Neural tube embryology Etiology ❏ Idiopathic ❏ Family history ❏ Risk factors: ❏ Folic acid deficiency ❏ Uncontrolled diabetes ❏ Obesity ❏ Hyperthermia during pregnancy ❏ Medications that interfere with folate metabolism ❏ Anticonvulsants Epidemiology ❏ Most common neural tube defect. ❏ 1-2 per 1000 births. ❏ More common in females than males - idiopathic. Pathophysiology Types Spina Bifida Occulta ❏ Most common, least severe.. ❏ No meninges are protruded. ❏ Asymptomatic. Meningocele ❏ Protrusion of meninges and sac containing CSF. ❏ Covered by normal skin ❏ Spinal cord NOT involved ❏ Lumbosacral region is common but may be seen in thoracic region and skull. Myelomeningocele ❏ Cystic sac of meninges with spinal tissue and CSF, which herniates through a defect in the vertebral arch. ❏ Type types: ❏ Open-myelocele ❏ Closed-Myelomeningocele Clinical Picture-Occulta ❏ Lesion over defect ❏ Tuft of hairs ❏ Dimple ❏ Birthmark ❏ Lipoma ❏ Deformity of foot ❏ Change in micturition pattern ❏ Gait alteration ❏ Trophic ulcers over foot and toes Clinical Picture-Meningocele ❏ Hydrocephalus- macrocephaly ❏ Spastic weakness ❏ Uncoordinated muscle movement ❏ Delayed developmental milestones ❏ Vision problems ❏ Seizures Clinical Picture-Myelomeningocele ❏ Flaccid paralysis ❏ Absence of sensation ❏ Drop reflex ❏ Postural abnormalities ❏ Hydrocephalus ❏ Musculoskeletal deformity ❏ Contracture of joints, scoliosis and kyphosis ❏ Rupture of sac and infection of CNS Clinical Picture-Myelomeningocele continued ❏ Chiari malformation type II ❏ Brain abnormality common in myelomeningocele spina bifida. ❏ Brainstem is elongated and positioned lower than usual. ❏ Dyspnea and dysphagia Investigations ❏ X-ray of spine and skull. ❏ MRI of spine and skull. ❏ Myelogram. ❏ CT scan. ❏ Prenatal screening tests ❏ Routine blood and urine exam ❏ Ultrasound ❏ Amniocentesis The Lemon sign The Banana Sign The banana sign The cerebellum as it herniates through the foramen magnum takes up the shape of a banana. When both the lemon and banana signs are seen together, an open spinal defect is present. Differential Diagnosis ❏ Sacrococcygeal teratoma ❏ Tethered cord syndrome ❏ Diastematomyelia ❏ Chiari malformation ❏ Syringomyelia ❏ Congenital dermal sinus ❏ Spinal cord tumors  MANAGEMENT Treatment Spina bifida treatment depends on how serious the condition is. Spina bifida occulta often doesn't need any treatment at all, but other types of spina bifida do. Surgery before birth Nerve function in babies with spina bifida can get worse if it's not treated. Prenatal surgery/fetal surgery, before 26th week of pregnancy. Repair the unborn baby's spinal cord. Sometimes Instruments are inserted into the uterus through tiny ports to perform surgery on the unborn baby. Cesarean birth C-Section birth is a safer way to deliver the baby in breech or has a large cyst or sac. Surgery after birth Myelomeningocele requires surgery to close the opening within 72 hours of birth. Early surgery lower the risk of infection. It also may help protect the spinal cord from more trauma. Spinal cord and exposed tissue are placed inside the baby's body and covered with muscle and skin. Shunt in the baby's brain to control hydrocephalus. Treatment for complications In babies with myelomeningocele, nerve damage that can't be repaired has likely already occurred in the uterus. Ongoing care from a multispecialty team of is needed. Treatment options may include:  Walking and mobility aids  Bowel and bladder management.  Surgery for hydrocephalus. Special equipment such as bath chairs, commode chairs and standing frames may help with daily functioning. Most spina bifida complications can be treated or managed to improve quality of life ONGOING CARE Children with spina bifida often need treatment and ongoing care from healthcare professionals with expertise in:  Physical medicine and rehabilitation.  Neurology.  Neurosurgery.  Urology.  Orthopedics.  Physical therapy.  Occupational therapy.  Special education.  Social work.  Nutrition. Parents and other caregivers are a key part of the team.  COMPLICATIONS The severity of the condition is affected by: The size and location of the neural tube defect, whether skin covers the affected area, which spinal nerves come out of the affected area of the spinal cord  COMPLICATIONS 1. Neurological Complications Hydrocephalus: Accumulation of cerebrospinal fluid in the brain, which can cause increased pressure and potentially lead to brain damage. Tethered Cord Syndrome: The spinal cord is abnormally attached to the spine, limiting its movement and causing neurological symptoms. 2. Physical Complications Mobility Issues: Muscle weakness or paralysis, particularly in the legs, which can affect walking and movement. Orthopedic Problems: Issues such as scoliosis (curvature of the spine), hip dislocation, and foot deformities. 3. Bladder and Bowel Complications Incontinence: Loss of bladder and bowel control is common due to nerve damage. Frequent Infections: Increased risk of urinary tract infections due to bladder dysfunction. Other Complications Meningitis: Infection of the tissues surrounding the brain and spinal cord, which can be life-threatening. Skin Problems: Pressure sores and other skin issues due to reduced mobility and sensation. References 1. Hockenberry,M.J & Winkelstein, W. (2005). Wong’s essential of pediatric nursing (7th ED).philadelphia,USA: Elsevier Mosby 2. Sandler, A., 2004. Living with Spina Bifida: A Guide for Families and Professionals. Chapel Hill: University of North Carolina Press 3. ICON Health Publications, 2018. Spina Bifida: A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References. San Diego: ICON Group International. 4. Grogan, K.D., 2010. Managing Secondary Conditions of Spina Bifida. Cincinnati: University Affiliated Cincinnati Center for Developmental Disorders. 5. Lutkenhoff, M. and Oppenheimer, S.G., 2017. Spinabilities: A Young Person’s Guide to Spina Bifida. Bethesda: Woodbine House. 6. Spina bifida: A neural tube defect Neupsy Key https://neupsykey.com/spina-bifida-a- neural tube-defect/ 7. https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/spina-bifida Thank you!