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Bone pathology
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- 5. Myelomeningocele is abirth defect in which the backbone and spinal canal do not close before birth. The condition is a type of spina bifida.
- 6. Myelomeningocele is themost common type of spina bifida. neural tube defect in which the bones of the spine do not completely form, resulting in an incomplete spinal canal. Myelomeningocele may affect as many as 1 out of every 800 infants.
- 7. The restof spina bifida cases are most commonly: Spina bifida occulta, a condition in which the bones of the spine do not close but the spinal cord and meninges remain in place and skin usually covers the defect Meningoceles, a condition where the tissue covering the spinal cord sticks out of the spinal defect but the spinal cord remains in place.
- 8. symptoms A newbornmay have a sac sticking out of the mid to lower back. The doctor cannot see through the sac when shining a light behind it. Symptoms include: Loss of bladder or bowel control Partial or complete lack of sensation Partial or complete paralysis of the legs Weakness of the hips, legs, or feet of a newborn
- 9. Other symptoms : Abnormal feet or legs, such as clubfoot Build up of fluid inside the skull (hydrocephalus) Hair at the back part of the pelvis called the sacral area Dimpling of the sacral area
- 10. Serum AFP(alpha-fetoprotein) testing in conjunction with a second trimester ultrasound detects over 90% of infants affected with a neural tube defect.
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- 23. Discitis ordiskitis is an infection in the intervertebral disc space that affects different age groups, but usually spontaneously affects children under 8 years of age. Nonetheless, discitis occurs post surgically in approximately 1-2 percent of patients after spinal surgery.
- 24. Symptoms severe backpain, leading to lack of mobility. Some very young children may refuse to walk and arching of the back is possible. In post-operative situations, the symptoms occur within a week and result in severe low back pain or neck pain (depending on the surgical location). If untreated, the discitis may resolve on its own, cause a chronic low grade infection, or progress to osteomyelitis and possibly even an epidural abscess.
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- 28. Definition Acute jointinfection due to bacterial agents Medical emergency
- 29. Risk factors: Systemic: Old age (>80 Y) RA DM Immunosuppressive Hemodyalisis Malignancy Local: RA OA Prosthetic joint
- 30. Etiology (microbiology) Microbialagent: Staphylococcus aureus: most common (75-80%) Other organism in special patients: Sexually active woman: Neisseria gonorrheae Elderly, IV drug abuser, immunocompromised, UTI: Gram negative (p. aeruginosa and E.coli) SLE: Salmonella HIV: Pneumococci, Salmonella, H. influenzae Alcoholism, Humeral immunity abnormality, Hemoglobinopathies: Pneumococcal infections Primary immunoglobolin deficiency: Mycoplasma
- 31. Clinical manifestations Fever(toxic) Acute Sever pain Sever swelling of one joint Sever tenderness Warmth Sever effusion Sever limited ROM
- 34. Infections of prosthesis implants Many joint prostheses are used in orthopedic practice and, whereas hip and knee joint replacement is performed thousands of times per year throughout the world. shoulder, elbow, wrist, ankle, metacarpophalangeal or interphalangeal joint replacement is much more recent and experimental . In 2004, hip and knee replacement procedures accounted for 95% of the 1.07 million arthroplasty procedures performed in the USA.
- 35. Although prostheticjoint implantations improve patients' quality of life, these procedures are associated with complications, including aseptic failure (i.e., aseptic loosening) and prosthetic joint infection (PJI). More than 25% of all prostheses will eventually demonstrate evidence of loosening, often necessitating a revision arthroplasty. Infection, although uncommon, is the most serious complication following joint prosthesis implantation.
- 36. In patientswith primary joint replacement, the infection rate in the first 2 years is usually <1% in hip and shoulder prostheses, <2% in knee prostheses, and <9% in elbow prostheses. The reported infection rates are probably underestimated, since many cases of presumed aseptic failure may be due to unrecognized infection. In addition, infection rates after surgical revision are usually considerably higher (up to 40%) than after primary replacement.
- 37. Other factors predisposingto PJI older age, poor nutritional status, underlying joint disease (RA, psoriasis), obesity, diabetes mellitus, malignancy,
- 38. remote infection, prior native joint infection, bacteremia (Staphylococcus aureus), advanced HIV infection, a revision surgery, or preoperative use of low-molecular-weight heparin. The mortality rate attributed to PJI may range between 0.4% in 65-year-old patients to 7% in 80-year-old patients.
- 39. The mostcommonly isolated microorganisms are Gram- positive cocci with coagulase-negative staphylococci, S. aureus and enterococci accounting for 65% of cases. Aerobic Gram-negative bacilli, including Escherichia coli, Proteus mirabilis andPseudomonas aeruginosa, are far less frequent causes of infection (6%). Anaerobes, including Propionibacterium acnes, account for 4% of infections.
- 41. Erb's palsy Erb'spalsy or Erb–Duchenne palsy is a paralysis of the arm caused by injury to the upper group of the arm's main nerves, specifically the severing of the upper trunk C5–C6 nerves.The brachial plexus is a network of nerves near the neck that give rise to all the nerves of the arm.
- 42. These nervesprovide movement and feeling to the arm, hand, and fingers. Palsy means weakness, and brachial plexus birth palsy causes arm weakness and loss of motion.
- 43. One ortwo of every 1,000 babies have this condition. It is often caused when an infant's neck is stretched to the side during a difficult delivery.
- 44. Causes: difficult delivery large baby, a breech presentation, prolonged labor. Forceful delivery (If one side of the baby's neck is stretched, the nerves may also be stretched, and injury may result).
- 45. Symptoms Weakness inone arm Loss of feeling in the arm Partial or total paralysis of the arm The arm cannot be raised from the side; all power of flexion of the elbow is lost, as is also supination of the forearm". The resulting biceps damage is the main cause of this classic physical position commonly called "waiter's tip."
- 50. Down Syndrome Genetics Allpersons with Down syndrome have duplicated chromosome 21 material The origin of the duplicated genetic material can vary Nondisjunction (about 96% of cases) The extra chromosome may arise by abnormal sorting to produce an extra, free-standing chromosome (trisomy 21) Translocation (3 % of cases) a joining of chromosomes to produce extra chromosome 21 material that is attached to another chromosome Mosaicism (1-2%) Patients with mixtures of normal and trisomic cells (mosaic Down syndrome) often have milder phenotypes. Percentages of normal cells within the blood sample used for chromosome studies may differ from the percentages of normal cells in other tissues like brain or heart.
- 51. Genetics Trisomy 21(47, +21), - 94 %, The frequency of trisomy increases with increasing maternal age. Robertsonian translocation involving chromosome 21- Approx. 3-4 %, not related to maternal age. Trisomy 21 mosaicism – 2 to 3 % cases
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- 54. Clinical Features Headand neck Brachycephaly Up-slanting palpebral fissures Epicanthal folds Brushfield spots Flat nasal bridge Folded or dysplastic ears Open mouth Protruding tongue Short neck Excessive skin at the nape of neck Extremities Short broad hands Short fifth finger Incurved fifth finger Transverse palmer crease Space between first and second toe Hyper flexibility of joints
- 55. Related Conditions CongenitalHeart Disease Seizures Eye Anomalies Hearing Loss Genito-Urinary Tract Anomalies Respiratory Disease Obesity Sleep Apnea Developmental Delays Blood Disease Endocrine Disease Blood Disorders Alzheimer’s Disease
- 56. Mental Retardation Almostall DS babies have MR. Mildly to moderately retarded . Starts in the first year of life. Average age of sitting(11 mon), and walking (26 mon) is twice the typical age. First words at 18 months. IQ declines through the first 10 years of age, reaching a plateau in adolescence that continues into adulthood.
- 57. Growth BW, lengthand HC are less in DS Reduced growth rate Prevalence of obesity is greater in DS Weight is less than expected for length in infants with DS, and then increases disproportion ally so that they are obese by age 3-4 yrs
- 58. Diagnosis Prenatal screening If no screening – It is recognized from the characteristic phenotypic features. Confirmed by Karyotype.
- 59. Management 1. Growth –Measurements should be plotted on the appropriate growth chart for children with DS. This will help in prevention of obesity and early diagnosis of celiac disease and hypothyroidism. 2. Cardiac disease – All newborns should be evaluated by cardiac ECHO for CHD in consultation with pediatric cardiologist. 3. Hearing – Screening to be done in the newborn period, every 6 months until 3 yrs of age and then annually.
- 61. Arthrogryposis multiplex congenita rthrogryposismultiplex congenita (AMC) is a group of nonprogressive conditions that cause multiple joint contractures (stiff joints) and abnormal muscle development.
- 62. signs and symptoms AMC are present at birth but can vary greatly in severity. exact cause of AMC is not fully understood, but it is thought to be associated with decreased movement or limited space in utero, connective tissue disorders, or maternal illness. Sometimes AMC occurs as part of genetic syndrome.
- 64. Treatment focuseson the specific symptoms experienced by each individual and may include physical therapy, removable splints, exercise, or surgery.