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Congenital pseudoarthrosis of the tibia with localised gigantism in a case of congenital constriction band syndrome.

Agashe MV, Rathod CM, Dhamele JA - Indian J Plast Surg (2011)

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatric Orthopaedics, Bai Jerbai Wadia Hospital for Children, Mumbai, India.

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He had progressively increasing volume of the left lower limb and no movements of the ankle and toes... The child was born normally and there was no adverse perinatal history... The right lower limb showed superficial constriction bands over the foot and calf, with minimal distal swelling... Radiograph of the left lower limb showed a cyst-like lesion in the distal tibia with a pathological fracture similar to type III of Boyd's classification for congenital pseudoarthrosis of the tibia... The patient had an uneventful post-operative recovery and all surgical wounds healed well [Figure 4]... A decision regarding ambulation with a prosthesis would be taken later... Amniotic constriction band syndrome is a group of disorders characterised by a wide range of congenital anomalies, including annular constrictions over the extremities, acrosyndactyly, talipes equinovarus, cleft lip and palate and hemangiomas... According to Paterson's classification of constriction band syndromes, our patient seems to be in type II, with distal lymphedema and sensory deficits... Closed pseudoarthrosis of the tibia, which has been noted earlier in congenital amniotic band syndrome, usually requires no treatment other than adequate splintage... However, this was not possible here due to the associated severe anomalies... Neurocirculatory disturbance along with lymphatic obstruction of such magnitude has not been described before, although a similar case of neurodeficit without circulatory disturbances has been described earlier by Nambi et al... In conclusion, this case is presented to discuss the end result of untreated congenital constriction band syndrome as well as to present this unique combination of severe lymphedema leading to localised gigantism and congenital pseudoarthrosis of the tibia in the setting of Streeter's dysplasia.

No MeSH data available.


Related in: MedlinePlus

Final clinical photograph of the child with well-healed amputation stump and healing Z-plasties of the right leg
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Figure 5: Final clinical photograph of the child with well-healed amputation stump and healing Z-plasties of the right leg

Mentions: Although there was no actual neurovascular compromise in the left lower limb, the enlargement of the limb was so much and so grotesque that plastic surgical reconstruction would have entailed an extensive reconstructive process, with a minimal possibility of getting a functional and sensate limb. Considering this, a decision to amputate the left lower limb was taken after informed consent of the parents. The constriction bands of the other limbs were treated with multiple staged Z-plasties. The patient had an uneventful post-operative recovery and all surgical wounds healed well [Figure 4]. A decision regarding ambulation with a prosthesis would be taken later.


Congenital pseudoarthrosis of the tibia with localised gigantism in a case of congenital constriction band syndrome.

Agashe MV, Rathod CM, Dhamele JA - Indian J Plast Surg (2011)

Final clinical photograph of the child with well-healed amputation stump and healing Z-plasties of the right leg
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3111106&req=5

Figure 5: Final clinical photograph of the child with well-healed amputation stump and healing Z-plasties of the right leg
Mentions: Although there was no actual neurovascular compromise in the left lower limb, the enlargement of the limb was so much and so grotesque that plastic surgical reconstruction would have entailed an extensive reconstructive process, with a minimal possibility of getting a functional and sensate limb. Considering this, a decision to amputate the left lower limb was taken after informed consent of the parents. The constriction bands of the other limbs were treated with multiple staged Z-plasties. The patient had an uneventful post-operative recovery and all surgical wounds healed well [Figure 4]. A decision regarding ambulation with a prosthesis would be taken later.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatric Orthopaedics, Bai Jerbai Wadia Hospital for Children, Mumbai, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

He had progressively increasing volume of the left lower limb and no movements of the ankle and toes... The child was born normally and there was no adverse perinatal history... The right lower limb showed superficial constriction bands over the foot and calf, with minimal distal swelling... Radiograph of the left lower limb showed a cyst-like lesion in the distal tibia with a pathological fracture similar to type III of Boyd's classification for congenital pseudoarthrosis of the tibia... The patient had an uneventful post-operative recovery and all surgical wounds healed well [Figure 4]... A decision regarding ambulation with a prosthesis would be taken later... Amniotic constriction band syndrome is a group of disorders characterised by a wide range of congenital anomalies, including annular constrictions over the extremities, acrosyndactyly, talipes equinovarus, cleft lip and palate and hemangiomas... According to Paterson's classification of constriction band syndromes, our patient seems to be in type II, with distal lymphedema and sensory deficits... Closed pseudoarthrosis of the tibia, which has been noted earlier in congenital amniotic band syndrome, usually requires no treatment other than adequate splintage... However, this was not possible here due to the associated severe anomalies... Neurocirculatory disturbance along with lymphatic obstruction of such magnitude has not been described before, although a similar case of neurodeficit without circulatory disturbances has been described earlier by Nambi et al... In conclusion, this case is presented to discuss the end result of untreated congenital constriction band syndrome as well as to present this unique combination of severe lymphedema leading to localised gigantism and congenital pseudoarthrosis of the tibia in the setting of Streeter's dysplasia.

No MeSH data available.


Related in: MedlinePlus