Images In Clinical Practice Indian Pediatrics 2003; 40:268 |
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Disorganization -like Syndrome |
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A two-month-old, female child presented with a lumbosacral mass, a partial foot was arising from this mass, which consisted of three digits and a rudimentary appendage (Fig. 1). The mass was cystic with no bony prominences were felt. Other features included an umbilical hernia and a ventricular septal detect. MRI revealed the presence of meningomyelocele. The attachment of foot was superficial and consisted of well-formed metacarpals and cartilage.
Disorganization-like syndrome is a rare occurrence of multiple congenital anomalies, the spectrum of which ranges in different studies. The occurrence of these anomalies are difficult to explain by amniotic bands. It was suggested that these might result from a mutual gene, the homologue of mouse mutant "disorganization" (Ds). There is high fre-quency of limb duplication (usually involving a single limb), polydactyly, and limb originating from abnormal sites. Anoop Verma, | ||
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