Mateusz Gładysz FESSH prep

FESSH prep Congenital hand

Constriction ring sequence (constriction band syndrome)

Last reviewed v1.0

FESSH-103Congenital hand●●○ intermediate

Regarding constriction ring sequence (constriction band syndrome):

  1. 1.The central digits are most often affected, whereas the thumb and little finger tend to be spared.

  2. 2.Constriction band syndrome is associated with prematurity and low birth weight.

  3. 3.In a tertiary-centre series, most children with constriction band syndrome had only one limb involved.

  4. 4.A constriction ring that compromises distal circulation or lymphatic drainage should be released within the first few days of life.

  5. 5.Constriction band syndrome usually occurs as part of a recognised genetic syndrome.

FESSH marking: +1 per correct statement, 0 for blank, −1 for incorrect. Click the same TRUE/FALSE button twice to clear it.

Question ID
FESSH-103
First published
Last reviewed
Version
v1.0
Answer key and explanations — open after answering

  1. 1. The central digits are most often affected, whereas the thumb and little finger tend to be spared.

    True

    In Demetri and colleagues' tertiary-centre series of 128 children, involvement showed a strong predilection for the central digits of the hands and feet, with sparing of the thumb or great toe and of the small finger or toe. Constriction bands were present in 96% of the children, limb or digit amputations in 88% and syndactyly or acrosyndactyly in 69%.

    • Demetri LRF, Starcevich AG, Manske MCB, James MA. Clinical Manifestations of Constriction Band Syndrome. J Bone Joint Surg Am. 2022;104(14):1301-1310. PMID 35856931

  2. 2. Constriction band syndrome is associated with prematurity and low birth weight.

    True

    Demetri and colleagues found the syndrome to be associated with gestational trauma, prematurity, low birth weight and young or older maternal age. In a Finnish population register of 71 affected children, 31% were born preterm and 25% had a low birth weight (frequencies only, without a comparison group).

    • Demetri LRF, Starcevich AG, Manske MCB, James MA. Clinical Manifestations of Constriction Band Syndrome. J Bone Joint Surg Am. 2022;104(14):1301-1310. PMID 35856931
    • Koskimies E, Syvänen J, Nietosvaara Y, Mäkitie O, Pakkasjärvi N. Congenital constriction band syndrome with limb defects. J Pediatr Orthop. 2015;35(1):100-3. PMID 24787313

  3. 3. In a tertiary-centre series, most children with constriction band syndrome had only one limb involved.

    False

    In Demetri and colleagues' series, children had on average 2.6 involved limbs and only 23% had a single limb affected; those with more limbs involved needed more operations. The figures come from a tertiary referral population, which may over-represent extensive disease; a 2024 review of surgical management also notes that involvement of more than one limb is common.

    • Demetri LRF, Starcevich AG, Manske MCB, James MA. Clinical Manifestations of Constriction Band Syndrome. J Bone Joint Surg Am. 2022;104(14):1301-1310. PMID 35856931
    • Sabapathy SR, Mohan M. Surgical Considerations in the Management of Constriction Ring Syndrome. J Hand Surg Asian Pac Vol. 2024;29(6):537-546. PMID 39544036

  4. 4. A constriction ring that compromises distal circulation or lymphatic drainage should be released within the first few days of life.

    True

    A 2024 review of surgical management advises that a ring causing vascular or lymphatic compromise be excised and released in the first few days of life; rings are released with multiple large Z-plasties in one or two stages. Moran and colleagues similarly note that early repair carries a better prognosis when distal oedema accompanies acrosyndactyly.

    • Sabapathy SR, Mohan M. Surgical Considerations in the Management of Constriction Ring Syndrome. J Hand Surg Asian Pac Vol. 2024;29(6):537-546. PMID 39544036
    • Moran SL, Jensen M, Bravo C. Amniotic band syndrome of the upper extremity: diagnosis and management. J Am Acad Orthop Surg. 2007;15(7):397-407. PMID 17602029

  5. 5. Constriction band syndrome usually occurs as part of a recognised genetic syndrome.

    False

    In a Finnish population-based register study of 71 children, none of the cases was associated with a known syndrome, although 30% had other anomalies such as clubfoot, cleft palate or congenital heart disease. The authors suggested that these associated anomalies might point to a genetic contribution in some children.

    • Koskimies E, Syvänen J, Nietosvaara Y, Mäkitie O, Pakkasjärvi N. Congenital constriction band syndrome with limb defects. J Pediatr Orthop. 2015;35(1):100-3. PMID 24787313