The outcome of Jarcho-Levin syndrome treated with a functional latissimus dorsi flap - A series of three cases.

INTRODUCTION

Jarcho-Levin syndrome is manifested by vertebral body and rib malformations. Large rib defects with paradoxical chest motion lead to early deathdue to progressive respiratory insufficiency, hence it is a lethal syndrome. The only means of improving survival is early stabilisation of the chest wall defect by containing the thoracic herniation. Nitcher et al. and Thatte et al. showed that reconstruction of the chest wall was life saving. Thatte et al. had postulated that early coverage of the lungs and thoracic contents with functional latissimus dorsi may prevent the visceral overgrowth and secondary pleural changes.

MATERIALS AND METHODS

Our three cases which had medium- and long-term follow-up help to support this postulation. Three patients were assessed retrospectively. Their ages at surgery were 6 months, 8 months and 1 year, respectively. All had laboured breathing and paradoxical respiration. All of them were operated with ipsilateral latissimus dorsi flap.

RESULTS

The results were evaluated clinically. The patients had reduced or no respiratory infections. The lung compliance improved and they had no tachypnoea on walking, running or playing.

CONCLUSION

Hence, this can be used as a life-saving procedure for Jarcho-Levin syndrome on a long-term basis.