Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
110 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
|
|
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
|