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111 Cards in this Set
- Front
- Back
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hand development patterns is determined by which gene?
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Hox A-D genes
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hand development patterns is mediated by which proteins?
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fibroblast growth factors, sonic hedgehog, and Wnt-7a
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What governed proximal to distal axis growth in limb developement?
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apical ectodermal ridge (AER)
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The apical ectodermal ridge arises from what strucure?
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Wolffian ridge, which protrudes from the main trunk of the embryo
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What molecule does the apical ectodermal ridge (AER)secrete?
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fibroblast growth factors (FGF)
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What governed anterior-posterior axis growth in limb developement?
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zone of polarizing activity (ZPA)
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What molecule does the zone of polarizing activity (ZPA) secrete?
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a protein called sonic hedgehog (Shh).
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What governed dorsal-ventral axis growth in limb developement?
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factor called wingless-type mouse mammary tumor virus integration site family member 7a (Wnt-7a).
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What happens on the 4th week of hand development?
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limb buds appear
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What happens on the 5th week of hand development?
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Apical ectodermal ridge induced development longitudinally (hand plate forms)
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What happens on the 6th week of hand development?
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Apoptosis resulting in digit separation
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What happens on the 7th week of hand development?
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Mesenchymal differentiation; chondrgenesis and osteogenesis
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fingernails can be identified by which week?
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17 week gestation
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Regarding hand development, what function is present at birth?
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Grasp reflex present
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Regarding hand development, what function is present at 3 months?
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power grip with ulnar digits
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Regarding hand development, what function is present at 5 months?
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Finger grip with adducted thumb
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Regarding hand development, what function is present at 7 months?
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thumb opposition
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Regarding hand development, what function is present at 9 months?
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small object pinch
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Regarding hand development, what function is present at 10 months?
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fine pinch
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Regarding hand development, 3-4 years?
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hand preference established
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How are hand deformities classified under failure of formation of parts?
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Transverse absence, longitudinal absence, Radial deficiency, thumb hyperplasia,Ulnar club hand, Cleft hand
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A child is born with an amputation at the elbow. How is this best treated?
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Prosthesis
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A child presents with his hand attached directly to his trunk. What is the diagnosis and what drug. was most likely ingested by his mother during her pregnancy?
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phocomelia, thalidomide
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A child presents with radial deviation of her right wrist , thumb hypoplasia, a short curved Ulna and hypoplasia of the radius. what is the diagnosis?
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radial clubhand
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radial club hand is associated with which type of inheritance?
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Autosomal dominant
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A child presents with radial deviation of her right wrist , thumb hypoplasia, a short curved Ulna and hypoplasia of the radiussand elbow stiffness. What syndromes are associated with this condition?
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Vaterr, Holt-Oran syndrome, fanconi'sanemia FAR syndromes Nagel's syndrome, Goldenhar syndrome ,mobius syndrome, cleft lip / palate, treachery-Collins syndrome, and kilippel-Feil syndrome
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what is the most serious condition associated with radial club hand
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Fanconi aplastic anemia
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what test is ordered in neonates with radial club hand and why?
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Mitomycin testing should to detect Fanconi anemia
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A child presents with radial deviation of her right wrist , thumb hypoplasia, a short curved ulna and absence of the radius, and elbow stiffness. What is the treatment?
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centralization of the ulna into the carpus followed by pollicization of the index finger
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a child presents with a thumb remnant withoutbony stability. How is this treated?
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thumb amputation with index politicization
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A neonate presents with partial absenceof the ulna. How is this treated?
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Splinting at birth, soft tissue release,radial and ulnar fusion and ilizarov distraction
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A child has a cleft hand and syndactyly. What is the inheritance pattern?
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Autosomal dominant
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A child has a cleft hand and syndactyly. How is this treated?
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1st web space syndactyly release, 2-plasty,and thumb osteotomies or tendon transfer
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ectrodactyly is classified as a...
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central hand deficiency,a partial or total absence of the fingers
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is ectrodactyly typically uni or bilateral?
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bilateral
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What are assoc. findings of ectrodactyly?
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lip and palate, congenital heart disease, and significant deformities of the upper and lower extremities
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The inheritance pattern of ectrodactyly is...
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familial
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cleft hand deformity is most commonly inherited as ...
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autosomal dominant, but also can occur as a spontaneous mutation
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cleft hand deformity are classified as...
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a longitudinal deficiency
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clinical manifestations of cleft hand deformity are...
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V-shaped cleft, suppression of the central digit, and sometimes minor syndactyly of the ulnar border digits
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what is the inheritance pattern of atypical cleft hand?
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sporadic
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clinical manifestations of atypical cleft hand deformity are...
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broad, flat cleft hand with finger nubbins containing nail remnants
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A child presents with short, webbed fingers secondary to short middle phalanges with partial syndactyly. What is the most likely diagnosis?
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symbrachydactyly
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A child presents with short, webbed fingers secondary to missing middle phalanges with partial syndactyly. What is the inheritance pattern?
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sporatic, nonfamilial
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A child presents with short, webbed fingers secondary to short middle phalanges with partial syndactyly. What is the treatment?
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surgical manipulation of the web space or osteotomies
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A child presents with short, webbed fingers secondary to missing phalanges with partial syndactyly. what is the most closely associated condition?
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Polands syndrome
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What is the incidence of Syndactyly?
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one of every 2500 neonates
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Describe simple syndactyly
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fusion of the skin of adjacent digits
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Describe complex syndactyly
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skin and bones are fused
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Describe complete syndactyly
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involves the entire web space
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Describe incomplete syndactyly
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only part of the web space is fused
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In syndactyly, which gender is affected more frequently?
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boys > girls
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In syndactyly, which webspace is affected most frequently?
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third
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A child presents with syndactyly of 3rd web space. What is the treatement?
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Ziz zag incisions, dorsal skin flap to cover he web space, and FTSG
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A child presents with syndactyly of 3rd web space. By which age should repair be completed?
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before 18 months of age
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A child presents with apert syndrome and multiple syndactylies and involving the thumb. What is the appropriate treatment?
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Release thumb at 1st stage, at concurrent stages avoid operating on both radial and ulnar side of digit at same operation
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What is the postoperative dressing should be used after syndactyly correction?
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a long arm (above-elbow)cast
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a 6 month old presents with a flexion deformity of IP joint and a palpable nodule in the FPL. what is the most likely diagnosis?
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Congenital trigger thumb
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a 6 month old presents with a flexion deformity of IP joint and a palpable nodule in the FPL.. what is the treatment?
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observation
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a 3 year old presents with a flexion deformity of IP joint and a palpable nodule in the FPL.. what is the treatment?
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A1 pulley release
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a patient presents with congenitalflexion contracture of the PIP joint of the small finger. what is the most likely diagnosis?
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Camptodactyly
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how do you tx camptodactyly with 20 degrees of extension of the (PIP) joint?
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Splinting, no surgery needed
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in camptodactylyI, how is extension deficit of 30 degrees or more treated?
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surgical release of the abnormal lumbrical and superficialis tendons &Release of the attachment of the accessory collateral ligaments to the volar plate
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Camptodactyly usually involves which finger?
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the small finger
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What are possible causes of camptodactyly?
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skin deficiencies, shortening of the volar plate, abnormalities of the palmar fascia and Landsmeer's ligament, deficiencies of the central slip, and shortening of the superficialis tendon
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a child has radial angulation of the small finger, at the DIP. what is the most likely diagnosis?
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Clinodactyly
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a child has radial angulation of the small finger, at the DIP. what is the most likely radiographic finding?
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delta phalanx
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describe a delta phalanx
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angulated, short, tubular base, trapezoidal or triangular in shape
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a child has radial angulation of the small finger, at the DIP. how is it treated?
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Excision and wedge osteotomy
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What is the must common type of thumb duplication?
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type IV
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A patient presents with a triphalangealthumb. what is the diagnosis?
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Type lV thumb duplication
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How do you treat types I/II thumbduplication?
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resection of central duplication halveswith primary closure
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How do you treat types III/IV thumbduplication?
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retain ulnar side
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How do you treat typesV/VI thumbduplication?
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retain radial side
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A child has overgrowth of one digit . what is the most likely diagnosis?
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Macrodactyly
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what cell mediates overgrowth in macrodactyly?
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neurophils
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correction of macrodactyly is...
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reduction or ablation
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Decribe Macrodactyly
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overgrowth of one digit or hand segment
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what cell mediates overgrowth in macrodactyly?
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neurophils
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A child has overgrowth of one digit . what is the most likely diagnosis?
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reduction or ablation
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describe brachydactyly
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short, fused digits
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How does amniotic constriction band syndrome manifests?
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intrauterine amputation of the digits with no known genetic transmission and is considered to be an intrauterine accident
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What causes amniotic constriction band syndrome?
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rupture of the amniotic membrane caused by oligohydramnios
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How common is amniotic constriction band syndrome ?
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fourth most common congenital anomaly of the hand
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amniotic constriction band syndrome incidence is..
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1 in 5000 to 15,000 births
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Anomalies frequently associated with amniotic constriction band syndrome include ...
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club foot, cleft lip and/or palate, craniofacial defects, hemangioma, and meningocele
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What type of repair is recommended for Amniotic constriction bands?
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Z-plasy
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Klippel-Tranaunay syndrome is characterized by ..
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musculoskeletal limb-length discrepancies involving one extremity, typically the leg, with associated venous and lymphatic malformations
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How does maffucci syndrome present?
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multiple enchondromas , venous and lymphatic anomalies,limbs foreshortened. Exostoses
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Poland syndrome typically involves ...
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hypoplasia of the chest wall and upper extremity unilaterally, absence of the pectoralis major and minor muscles and limb hypoplasia with syndactyly and brachydactyly.
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Poland's syndrome is associated with ....
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unilateral findings, including absence of the sternocostal portion of the pectoralis major muscle, hypoplasia of the upper extremity, simple syndactyly, and shortened fingers.
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Patients with Sturge-Weber syndrome have ....
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port-wine stains in the distribution of the first and second divisions of the trigeminal nerve (V1and V2) associated with seizures and ipsilateral leptomeningeal calcifications
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what is the house classification?
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stratify the thu found in mb-in-palm deformity patients with cerebral palsy
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Define the House classification
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Treatment of the thumb-in-palm deformity involves ...
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of the spastic muscles and stabilization of the joint.
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What is the treatment for a child who has partial or complete absence of function of the thumb or one or more finger?
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who has partial or complete absence of function of the thumb or one or more fingers
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Arthrogryposis is a congenital disorder characterized by...
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nonprogressive joint contractures present at birth
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Arthrogryposis is thought to be caused by?
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neurogenic or myopathic weakness in utero
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what is Madelung's deformity?
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congenital disorder of the wrist
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what are the clinical manifestations of Madelung's deformity?
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shortening of the radius, apparent palmar subluxation of the carpus, and prominence of the ulnar head. Finger abnormalities are not associated
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Madelung's deformity is characterized by...
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arrested growth of the ulnar aspect of the distal radius resulting in a patterned wrist deformity
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what are the extremity findings in Apert syndrome?
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symmetric syndactyly of the hands and feet
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Patients with Apert's syndrome have ...
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bilateral, symmetric, complex syndactyly with shortened fingers Craniofacial deformities are characteristic
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How does apert's syndrome present?
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bilateral, symmetric, complex syndactyly with shortened fingers. Craniofacial deformities are characteristic
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what are the extremity findings in Carpenter syndrome?
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partial digital syndactyly and preaxial polysyndactyly of the feet
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How does carpenter's syndrome present?
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simple syndactyly, shortened fingers, preaxial polysyndactyly, broad thumbs, and craniosynostosis
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what are the extremity findings in Nager syndrome?
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hypoplasia to agenesis of the radius, thumbs, and metacarpals
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what are the extremity findings in Pfeiffer syndrome?
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broad thumbs and halluces.artial syndactyly of the second and third digits has also been identified
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Pfeiffer's syndrome is characterized by..
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mild partial syndactyly, broadened, deviated thumbs and severe midface hypoplasia
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In the Zancolli-lasso procedure, slips of the ___ tendon are looped through the ____
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flexor digitorum superficialis,A2 pulley
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Zancolli-lasso procedure appropriate for correction of ....
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digital clawing associated with ulnar palsy
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