Curly Toe Syndrome

Figure 1.126. Symmetrical syndactyly of the toes in an infant with Apert's syndrome (acrocephalosyndactyly).

In symphalangism, no joint movement whatever is possible at the sites of the affected interphalangeal joints because the bony fusion has taken place. The absence of flexion creases is an excellent clue to the presence of this anomaly.

Symphalangism SyndromeCongenital Curly Toes

Figure 1.127. Another example of symmetrical syndactyly of the toes in Apert's syndrome.

Figure 1.127. Another example of symmetrical syndactyly of the toes in Apert's syndrome.

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Figure 1.128. Bilateral symmetrical

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'■.'.■'■■'' " polysyndactyly giving the appearance of .'■ ' ■'■ ■ :.;■■;webbing between the toes in an infantwith Carpenter's syndrome (acrocephalopolysyndactyly).

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Broad Toes

Figure 1.129. Broad toes in a normal infant. This may be familial. Broad toes are seen in certain syndromes such as Rubenstein-Taybi syndrome and Larsen's syndrome.

Figure 1.130. Preaxial Polydactyly with bifid big toes in an otherwise normal infant.

Figure 1.130. Preaxial Polydactyly with bifid big toes in an otherwise normal infant.

Bifid Middle Finger

Figure 1.131. Bifid big toes with Polydactyly in an infant who also has digitalization of the thumbs.

Figure 1.132. Duplication of the big toe. Radiograph showed two separate digits. This may result from an early insult to the limb bud in the 5 th to 6th week of gestation.

What Congenital Curly Toe

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Toes Separate From Big Toe

Figure 1.134. Hypertrophy of the third toe of the right foot. This may occur as an isolated finding or may be seen in neurofibromatosis or in infants with vascular malformation of a digit.

Figure 1.133. Congenital curly toes ("overlapping" toes). These are very common and are often familial. The abnormality becomes less obvious as the infant grows.

Figure 1.134. Hypertrophy of the third toe of the right foot. This may occur as an isolated finding or may be seen in neurofibromatosis or in infants with vascular malformation of a digit.

Figure 1.135. Dorsal view of macrosyn-dactyly of die second and diird toes of the right foot.

Figure 1.136. Plantar view of the toes of the same infant.

Figure 1.136. Plantar view of the toes of the same infant.

Infant Plantar Crease

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Figure 1.137. Single palmar crease and clinodactyly in the left hand of an otherwise normal infant. Single palmar creases are noted bilaterally in 1 to 2% of normal infants and unilaterally in 6% of normal infants. It is present in about 50% of patients with Down syndrome. It is twice as common in males as in females and it is associated with many syndromes. Palm creases form in response to flexion at the metacarpophalangeal joints and opposition of die thumb. Three deep creases are usually seen but there are many normal variants.

Figure 1.138. Single palmar crease and clinodactyly of the right hand. Clinodactyly is the incurving of die finger to one side, usually toward the midline, due to an absent or hypoplastic middle phalanx. Involvement of die fifth finger is most common. With an absent phalanx only two creases are present as in diis infant. With a hypoplastic middle phalanx, the number of creases is normal but creases are closer togedier and will slope toward each other rather than being parallel. It is noted in otherwise normal infants but also occurs in many syndromes.

Curly Toes Syndrome

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Extra Crease Finger Lines
Figure 1.139. An extra crease on the fifth finger.

Figure 1.140. Increased number of finger creases in an otherwise normal infant. Increased finger creases may be seen in normal infants and in infants that have increased laxity of the joints such as in Larsen's syndrome and Ehlers-Danlos syndrome. They often signify increased fetal activity at 11 to 12 weeks of fetal life when the creases normally become evident. Hence, gross alteration in crease patterning is usually indicative of an abnormality in form and/or function of the hand prior to the 11th fetal week. If there is a lack of fetal movement before this period of gestation, the number of finger creases is decreased.

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Disorder Crease Palm
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Figure 1.141. The father of die same infant also had increased finger creases. He was otherwise normal and had no problems. The thenar crease normally circles the base of the thenar eminence, extending distally to between the thumb and index fingers. The distal palmar crease traverses the palm beneadi the last three fingers, beginning at the ulnar edge of the palm and curving distally to exit between the middle and index fingers. The proximal palmar crease may be less well defined. It begins over the hypothenar eminence and normally extends parallel to the distal crease to exit near or fuse with the distal portion of die thenar crease.

Bridged Palmar Crease

Figure 1.143. This infant has decreased creases in both the fingers and the palm due to lack of fetal movement. Absence of normal flexion creases invariably signifies inadequate movement of the underlying joints. Changes in die palmar crease include the single palmar crease (simian crease) and the bridged palmar crease (Sydney line) in which there is an extension of the proximal transverse crease which reaches the ulnar border of die hand and the medial edge of the palm between the index and middle fingers.

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Werdnig Hoffmann Syndrom

Figure 1.144. Lack of fetal movement is seen in acute infantile spinal atrophy (Werdnig-Hoffmann disease). Lack of normal development of the finger creases is due to lack of fetal movement early in gestation. On the left, note the position of comfort of the fingers with deep depression in the palm shown on the right.

Figure 1.144. Lack of fetal movement is seen in acute infantile spinal atrophy (Werdnig-Hoffmann disease). Lack of normal development of the finger creases is due to lack of fetal movement early in gestation. On the left, note the position of comfort of the fingers with deep depression in the palm shown on the right.

Amyotonia CongenitaCurly Toes Syndrome

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Figure 1.145. Lack of creases of the palm and fingers in an infant with amyotonia congenita.

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Hypoplastic Dermal Ridges

Figure 1.146. Hypoplastic (absent or sparse) dermal ridges and absence of flexion creases on the fingers and palms are seen in this infant with the fetal akinesia sequence (Pena-Shokeir phenotype).

Pena Shokeir Phenotype

Figure 1.147. This infant with arthrogryposis multiplex congenita shows the lack of palmar and finger creases due to lack of fetal movement before the 10th to 12th weeks of gestation.

Figure 1.148. Arthrogryposis multiplex congenita in this infant shows the contractures which occur in this condition. They are usually symmetrical and involve all four extremities but may involve only the upper or lower limbs. There is muscular hypotonia, generalized thickening of the skin with dimpling, and hip subluxation; and bilateral talipes equinovarus, opisthotonos and scoliosis of die spine are common.

Figure 1.148. Arthrogryposis multiplex congenita in this infant shows the contractures which occur in this condition. They are usually symmetrical and involve all four extremities but may involve only the upper or lower limbs. There is muscular hypotonia, generalized thickening of the skin with dimpling, and hip subluxation; and bilateral talipes equinovarus, opisthotonos and scoliosis of die spine are common.

Talipes Calcaneovalgus

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Figure 1.149. Contracture of the hand in an infant with arthrogryposis multiplex congenita.

Figure 1.150. Contracture of the lower extremity in the same infant. These infants commonly have bilateral talipes equinovarus.

Figure 1.149. Contracture of the hand in an infant with arthrogryposis multiplex congenita.

Arthrogryposis Multiplex Congenita

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Joint Dimpling
Figure 1.151. Dimples at the knee in an infant with arthrogryposis multiplex congenita. Normally dimples at a joint are of no significance, but they may occur with contractures and lack of movement as in this infant.

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Responses

  • Christin
    What is curly toe syndrome?
    11 months ago
  • Barry
    Is wiggling toe syndrome curable?
    9 months ago
  • ahmad
    What is palmar and plantar creases?
    4 months ago

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