急性肾小球肾炎（AGN）

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1 发育性髋关节脱位 DEVELOPMENTAL DISPLASIA of the HIP (DDH) Department of Pediatrics Soochow University Affiliated Children s Hospital

2 Can you find any problem in those X-rays?

3 DEVELOPMENTAL DYSPLASIA OF THE HIP Developmental dysplasia of the hip generally includes subluxation (partial dislocation) of the femoral head, acetabular dysplasia, and complete dislocation of the femoral head from the true acetabulum. Instability Subluxation Dislocation

4 In a newborn, the femoral head can be dislocated and reduced into and out of the true acetabulum.

5 In an older child, the femoral head remains dislocated and secondary changes develop in the femoral head and acetabulum.

6 Incidence The incidence of DDH is approximately 1 in 1000 live births. The left hip is more commonly involved than the right. Bilateral involvement is more common than involvement of the right hip alone. The disorder is more common in females than in males, in many series as much as five times.

7 Several risk factors Several risk factors should arouse suspicion of DDH: 女孩 Female sex 早产 Prematurity 臀位产 Breech delivery 家族史 Family history 神经肌肉系统疾病 Neuromuscular disorders

8 A strong association also exists between DDH and other musculoskeletal, such as : metatarsus adductus congenital torticollis talipes calcaneovalgus

9 病因学 Etiology Shallow acetabulum Genetic factors Ligament laxity Tightly packaging in uterus Mechanical factors Breech delivery

10 病理学 Pathology Adductor and iliopsoas tendon contracture, ligamentum teres thickening D D H The femoral head reduced in size with posteromedial flattening Acetabulaum shallow Excessive anteversion Compensatory scoliosis and pelvis tilt

median adductor approach. Ferguson AB Jr.

11 髂腰肌阻碍复位, 关节囊呈葫芦状切断髂腰肌后复位肥大的圆韧带阻碍复位引自 :Primary open reduction of congenital dislocation of the hip using a median adductor approach. Ferguson AB Jr. Journal of Bone & Joint Surgery - American Volume. 55(4):671-89, 1973 Jun.

12 Physical Examination limitation of abduction 内收肌紧张髋关节外展受限

13 Ortolani test The hip is abducted slightly, then, with the index and long fingers over the greater trochanter, the thigh is raised to reduce the hip.

14 Barlow test The thumb is placed on the inner aspect of the thigh near the lesser trochanter. The hip is add-ucted and longitudinal pressure is exerted on the thigh with the thumb pushing it towards the table. This test detects whether the hip is subluxatable or dislocatable.

15 Allis(Galeazzi)sign The Allis sign is noted when the femoral head becomes displaced not only laterally but also proximally, causing an apparent shortening of the femur on the side of the dislocated hip.

16 臀纹不对称 Asymmetrical skin folds 该体征没有特异性, 正常的儿童也有臀纹不对称, 髋脱位的患儿也可能臀纹对称

17 6 月龄女孩, 因臀纹不对称就诊,X 线片显示无髋关节脱位

18 Ultrasound screening Professor. Graf used ultrasound to screen newborns for early diagnosis of DDH in 1980s, and devised an ultrasonographic classification for hip dysplasia. Ultrasound is noninvasive and simple to use. It is able to reveal the cartilaginous areas of the joints and dynamic instability during movement of the hip.

19 髂骨三角软骨股骨头股骨大转子

20 X-Ray Perkins line H line AI Normal Shenton line Break 1. Horizontal Y line (Hilgenreiner line) 2. Vertical line (Perkins line) 3. Quadrants (formed by lines 1 and 2) 4. Acetabular Index 5. Shenton line

21 CT scan of pelvis to confirm bilateral reduction of femoral head into true acetabulum

22 Diagnosis and Clinical presentation The clinical presentation of DDH varies according to the age of the child. In newborns, it is especially important to perform a careful clinical examination. Since roentgenograms are not absolutely reliable in making the diagnosis of DDH in this age group.

23 Neonatal period : High risk factor suspicion Ortolani and Barlow Test Ultrasound Early infantile age(1~6months): Ortolani test Ultrasound X-rays

24 Infantile and Toldder age (7~36months): Clinical presentation The limited abduction of the hip Asymmetrical skin folds Unstable waddling gait Positive Trendelenburg test Bilateral pelvic AP view

25 Treatment Treatment for DDH should be started as early as possible. The treatment of DDH is age related and tailored to the specific pathological condition.

26 Five treatment groups related to age have been designated : 1. birth to 6 months 2. 6 to 18 months to 36 month 4. 3 to 8 years 5. beyond 8 years

27 Pavlik Harness shoulder strap chest strap The Pavlik harness consists of a chest strap, two shoulder straps, and two stirrups. stirrup Each stirrup has an anteromedial flexion strap and a posterolateral abduction strap.

28 The harness is applied with the child supine and in a comfortable undershirt.

29 The chest strap is fastened first, allowing enough room for three fingers to be placed between the chest and the harness. The shoulder straps are buckled to maintain the chest strap at the nipple line. The feet are then placed in the stirrups one at a time. The hip is placed in flexion (90 to 110 degrees), and the anterior flexion strap is tightened to maintain this position. The lateral strap is loosely fastened to limit adduction, not to force abduction. Excessive abduction to ensure stability is not acceptable.

30 Once a child reaches 4 to 6 months old, success with the Pavlik harness decreases significantly. A child aged 6 to 18 months with a dislocated hip probably will require either closed manipulation or open reduction. In our hospital, we apply medial approach to correct the soft tissue structures and to reduce the femoral head concentrically in the acetabulum.

31 Ferguson Approach Incision( 手术切口 ) 在内收短肌和股薄肌之间剥离

32 Adductor longus 分离内收长肌腱, 切断其腱性部分

33 The branches of the anterior obturator nerve 显露闭孔神经前支

34 Iliopsoas tendon 显露髂腰肌腱, 切断其腱性部分

35 ligamentum teres 沿髋臼边缘进入关节囊, 见圆韧带肥厚, 脂肪组织增生

36 Iliopsoas tendon Adductor longus 通过内侧入路切断挛缩的内收长肌腱髂腰肌腱切断髋臼横韧带清除臼内脂肪组织圆韧带, 实现同心圆复位, 并减少股骨头坏死的发生

37 2 months later Human Spica

38 Case -1

39 6 months after operation 1 year later 2 years later

40 Case month-old

43 1 year later

44 1.5 years later

45 2 years later

46 内侧入路的优点 : 手术剥离少满足美观上可接受的疤痕不影响髂骨的生长不损伤外展肌内侧入路的缺点 : 股骨头缺血性坏死的发生率可能增加, 主要是易伤及旋股内侧血管无法显露髋关节假臼, 不能行关节囊修复术

47 When the children are beyond 18 months of age, abnormalities persisting are difficult to manage and may require open reduction with realigning derotation osteotomies, and possibly acetabular angulation.

48 Salter Innominate Osteotomy Salter 骨盆截骨术 F, 24 months

50 显露出髂骨内外板

51 插入修整好的截骨块, 在截骨近端平行钻入两枚螺纹克氏针, 增加截骨的稳定性

53 Salter 骨盆截骨注意事项 1. 全下肢皮肤无菌敷料铺巾, 允许在手术过程中可以自由活动 2. 常采用比基尼切口, 此切口有益于术后体表外观 3. 保护股外侧皮神经, 在髂前上棘以下纵行切开深筋膜 4. 保持在骨膜下操作, 以免损伤神经和血管 5. 填入截骨块过程中确保截骨端前侧张开而后侧闭合 6. 应确定克氏针未进入关节

54 Innominate Osteotomy and Femur Shortening varus osteotomy 骨盆截骨联合股骨短缩旋转截骨 subluxation dislocation A seven-year old girl has abnormal gait.

56 Flexion External rotation Internal rotation

57 Chiari medial displacement osteotomy

58 Steel triple innominate osteotomy A, Sixteen-year-old girl with painful right hip, subluxation, and acetabular dysplasia. B, After Steel osteotomy. C/D, One year after surgery.

59 髋关节脱位患者至成年后, 因为髋关节退行性骨关节炎难以避免, 需要手术治疗, 如全髋关节置换术

60 大龄儿童髋关节脱位手术目的 (Purpose) : 恢复关节稳定 (restore hip stability) 缓解关节疼痛 (relieve pain) 维持关节运动 (maintain mobility)

61 手术方法切断髂腰肌 (reliease the iliopsoas tendon from the lesser trochanter) 45 T 形切开关节囊, 显露髋关节和股骨近端部分 ( T shape incision in the hip capsule to expose the hip joint and the proximal part of the femur )

分别自小转子下处作斜形和横行截骨, 取下楔形截骨块, 角度约 45~60 ( make an oblique and transverse cut from the lateral cortex on the inferior margin of the lesser trochanter, and remove the wedge of 45~60 )

62 分别自小转子下处作斜形和横行截骨, 取下楔形截骨块, 角度约 45~60 ( make an oblique and transverse cut from the lateral cortex on the inferior margin of the lesser trochanter, and remove the wedge of 45~60 ) 采用合适的钢板系统进行固定, 并透视保证股骨近端外翻小转子和髋臼相关节 (Fixation of the osteotomy site with plate. Intraoperatively with the image intensifier, to ensure the lesser trochanter articulates with the acetabulum.)

63 病例1 患儿走路时存在跛行 2岁在外院诊断为髋脱位牵引治疗 8岁时行行截骨手术 17岁时来我院就诊时肢体不等长脊柱代偿性侧弯摄片发现左侧髋臼发育不良股骨头变形

65 参考书目及文献 : 1. 坎贝尔骨科手术学 ( 第十版 ) 山东科技出版社 2. 发育性髋关节脱位潘少川主译, 人民卫生出版社 3. Paediatrics ( 第二版, 英文原版 ) 北京医科大学出版社 4. Primary open reduction of congenital dislocation of the hip using a median adductor approach. Ferguson AB Jr. JBJS (Am):55(4):671-89, 1973 Jun.

66 Quiz 1. What s the meaning of the DDH? 2. How to identify the DDH? 3. What is the perfect treatment of the DDH?

67 Thank You!

68 大脑性瘫痪 Cerebral Palsy The Children s Hospital of Soochow University 苏州大学附属儿童医院

69 On average, children should sit at 6 months, stand at 8 months, and walk at 12 months. If child does not reach these milestones by 1.5 times, an investigation into the developmental delay is warranted.

70 Cerebral palsy is a non-progressive insult to the developing brain that usually occurs in the neonatal period.

73 The manifestations of cerebral palsy, though due to a central nervous system (CNS) lesion, are often first evident in the musculoskeletal system. As a result, the initial diagnosis of cerebral palsy is often made by the orthopaedist,especially in children with mild cerebral palsy.

74 Etiology There are many potential causes, such as : anoxia infection prematurity hemorrhage

75 There is a strong association with prematurity. In such cases there is often debate as to whether the neurological damage occurred during the delivery or whether the brain was naturally impaired. It is not solely the result of prematurity because 60% of children with CP are born at full term.

76 Incidence Cerebral palsy occurs in approximately 1 in 500 children, a rate that has not changed significantly despite medical advances of the past several decades.

77 Twin pregnancies result in a child with CP about 12 times more than do singleton pregnancies. This is largely related to low birth weight.

78 Lesions in the brain Lesions in the brain that cause abnormality in movement or posture occur primarily in the following four areas: 1. the cerebral cortex (spasticity); 2. the midbrain or base of the brain (athetosis); 3. the cerebellum (ataxia); 4. widespread brain involvement (mixed).

79 Presentation & Diagnosis Children with CP to the orthopedist for myriad reasons. Common reasons for referral include limping, foot problems, leg-length discrepancy, toe walking, tight muscles, and poor use of the hands.

80 The diagnosis of cerebral palsy is often easily made when the appropriate history has been elicited from the parents, even before the child is examined. A detailed birth history should include problems during the pregnancy and delivery, possible anoxic events, and duration of hospitalization.

81 Classification of CP CP is classified by the neuropathic type of motor abnormality and by the anatomic region of involvement.

82 Neurological type 痉挛型 (Spastic) 手足徐动型 (Athetoid) 共济失调型 (Ataxic) 松软型 (Atonic) Anatomical distribution 单侧瘫 (Hemiplegia) 半身瘫 (Diplegia) 全瘫 (Quadriplegia) 混合型 (Mixed)

83 Spastic The most common type of motor disturbance is spasticity, which affects the flexor muscle groups more than the extensors.

84 Severe spasticity is often referred to as rigidity. Joint contractures are common in spastic CP. Adductor Hamstring Achilles tendon

85 Athetoid Athetoid cerebral palsy is the next most common type, accounting for about 25% of cases. Joint contractures are uncommon and muscle tone may not be increased. This children usually characterize by distorted postures and positions that are induced by voluntary movements.

86 Ataxic Fewer than 3% of patients with cerebral palsy have the primary ataxia type. It is a disturbance of coordinated movement, most notable when walking, and is usually the result of cerebellar dysfunction. cerebellum

87 Mixed The mixed type has been found to account for an increasing number of patients, probably more than 10%. As the immature brain develops, occasionally the clinical manifestations of the type of movement or posture disorder may change.

88 Hemiplegia Diplegia Quadriplegia

89 Hemiplegia One side of the body is involved, the upper limb being more affected than the lower. The diagnosis is usually not made until after walking has begun.

90 Diplegia Both lower extremities are involved and to a greater extent than the uppers, which are always affected to some degree.

91 Quadriplegia Quadriplegia involve all four limbs. Many of these children have global involvement, with mental retardation. These children show delayed developmental milestones. The spectrum of severity is variable, from having no sitting ability or head control to being able to walk independently.

92 Treatment Although the brain damage in CP is static, the resulting musculoskeletal manifestations of the condition are progressive during the growing years. The dynamic contractures and mobile deformities deteriorate and may become fixed with growth. Orthopedic surgery can only have limited goals in cerebral palsy as it deals with the effects rather than the cause of the problem.

93 In ambulatory children who require surgery for gait abnormalities, surgery is most commonly undertaken when the child is between the ages of 5 and 10 years. One of the most important concepts in appropriate surgical treatment of these children is to postpone surgery until the child has fulfilled the following criteria:

94 When considering surgery it is important to distinguish a fixed joint contracture from a dynamic contracture. In the former, the muscultoendinous unit is truly short and even under anaesthetic the contracture will persist. In a dynamic contracture, the cause is muscle spasm and under anaesthetic the contracture relaxes.

95 Tendon lengthening is indicated only for fixed contractures.

96 The aim of surgery should be to improve function and facilitate the care of these patients by correcting or preventing deformity. Regular physiotherapy to stretch tight muscles and joints and supervise the use of orhtoses is very important. Parents must also be involved in the care of these children. Realistic goals must be set and it has to be explained that the results of surgery can be unpredictable and disappointing, especially in dystonic cases.

97 The orthopedic goals in total body involvement can be summarized as follows.

98 The gait disturbances are interfering with function. Nonsurgical interventions will no longer suffice to address the gait disturbances. The child has failed to make significant functional progress over the preceding 6 months.

99 Hip These are at risk of dislocation because of adductor and psoas spasm. The joints should be monitored clinically by the range of abduction and annual radiographs. If abduction is less than 40, the hip is at risk. Preventative measures include exercises, abduction splints and seating adaptations.

100 If the hip is displacing, surgical options include adductor and psoas release and,if severe, femoral osteotomy.

101 If the hip has dislocated, and open reduction and hip reconstruction is often indicated. Approximately 40% of patients with a dislocated hip will develop pain that is very difficult to treat. The emphasis is therefore on prevention. In a long-standing dislocation the femoral head becomes inflamed and deformed and hip reconstruction may not be appropriate as the symptoms would not be relieved.

102 Spine Scoliosis is common and should be anticipated. Patients also tend to collapse forwards into a kyphosis and may have poor head control. Early curves can be treated by postural exercises, seating and bracing. Even minor curves should be treated; they will progress and do not behave like idiopathic scoliosis. More severe curves need surgical stabilization to prevent progression to unmanageable deformities.

103 Foot Equinus will be exacerbated by weakness of the dorsiflexors. When the deformity is dynamic it can be treated by an ankle-foot orthosis (AFO).

104 Postoperative recovery is slow and there may be problems such as muscle spasm and pressure sores under casts.

105 Varus is usually due to overactivity in tibialis posterior or anterior. If the equinus is fixed then Achilles tendon lengthening may be required. Occasionally the varus deformity needs correction by tendon transfer.

106 参考书目及文献 : 1. 坎贝尔骨科手术学 ( 第十版 ) 山东科技出版社 2. Paediatrics ( 第二版, 英文原版 ) 北京医科大学出版社 3. 实用小儿骨科学 ( 第 2 版 ), 潘少川主编人民卫生出版社 4. 实用小儿骨科学 ( 第 2 版 ),Staheli LT 著, 潘少川主译, 人民卫生出版社

107 Quiz We should know the meaning of Cerebral Palsy. What s the classification of Cerebral Palsy? Please master the principle of the treatment of CP.

108 Thank You!

第一章　緒論

第一章　緒論 (informed consent) 6 . (Informed 23 Consent) 24 (Informed Consent) informed consent 1. 2. 7 3. 4. 5. 6.. 26 1. 8 2. 3. 4. 5. 6.. Braddock 1993 59 65 1057 (discussion of the patient s role in decision making)