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111 Cards in this Set

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