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Ischiopagus and pygopagus conjoined twins: neurosurgical considerations

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Abstract

Background

Neurosurgeons are familiar with the challenges presented by craniopagus twins, but other types of conjoined twins may also have neurosurgical implications. We report our experience in the management of ischiopagus and pygopagus conjoined twins.

Methods

This is a retrospective review of the management of conjoined twins at Red Cross Children’s Hospital in Cape Town, South Africa.

Results

Twenty-three pairs of symmetrical conjoined twins were managed over a 40-year period (1964–2003), of which 16 (70%) were separated. Of these cases, 6 are the focus of this study, namely 4 pairs of ischiopagus twins and 2 pairs of pygopagus twins seen between 1993 and 2003. In 2 cases, there was direct involvement of the nervous system at the site of union, with 1 pair of ischiopagi manifesting end-to-end union of their spinal cords, while a pair of pygopagi had back-to-back fusion of the conus. Another pair of ischiopagi had a fused dural sac without joined neural elements, but one of these children developed syringomyelia 2 years after separation. Neuroimaging was invaluable in detecting these abnormalities. The one pair of ischiopagi who died before separation were HIV positive and had severe brain atrophy and cystic encephalmalacia at autopsy. Nine of the 12 children (75%) had bony abnormalities of the spine remote from the area of conjunction. The most common finding was the presence of hemivertebrae, usually in the thoracic spine. Six children manifested scoliosis, which has already progressed in the oldest two. Technical aspects such as timing and sequence of separation, the division of neural tissues and reconstruction are discussed, as are the long-term complications of their spinal abnormalities.

Conclusions

Ischiopagus and pygopagus conjoined twins manifest an interesting array of spinal abnormalities, which present challenges, not only at the time of separation, but also in their long-term management.

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Acknowledgements

We thank Emeritus Professor Sid Cywes and Professor Teddy Hoffman for their valuable comments. Dr Jenny Thomas and Ms J Raad are thanked for providing some of the clinical data on the children in this study, as is Dr Alp Numanoglu, who provided the photograph used as Fig. 3a. Mrs J Swanepoel provided expert secretarial assistance.

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Authors and Affiliations

  1. Division of Paediatric Neuroscience (Neurosurgery), Red Cross War Memorial Children’s Hospital and School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch 7700, South Africa

    A. Graham Fieggen & Jonathan C. Peter

  2. Division of Orthopaedics, Red Cross War Memorial Children’s Hospital and School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch 7700, South Africa

    Robert N. Dunn

  3. Division of Paediatric Radiology, Red Cross War Memorial Children’s Hospital and School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch 7700, South Africa

    R. Dick Pitcher

  4. Division of Paediatric Surgery, Red Cross War Memorial Children’s Hospital and School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch 7700, South Africa

    Alastair J. W. Millar & Heinz Rode

Authors
  1. A. Graham Fieggen
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  2. Robert N. Dunn
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  3. R. Dick Pitcher
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  4. Alastair J. W. Millar
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  5. Heinz Rode
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  6. Jonathan C. Peter
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Corresponding author

Correspondence to A. Graham Fieggen.

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Fieggen, A.G., Dunn, R.N., Pitcher, R.D. et al. Ischiopagus and pygopagus conjoined twins: neurosurgical considerations. Childs Nerv Syst 20, 640–651 (2004). https://doi.org/10.1007/s00381-004-0987-2

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  • DOI: https://doi.org/10.1007/s00381-004-0987-2

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