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Updated: Jul 27, 2026
Intraoperative Ultrasound in Spinal Surgery
Published on: August 17, 2022
A case report of caudal regression syndrome associated with an intraspinal arachnoid cyst
H Tsugu1, T Fukushima, S Oshiro
1Department of Neurosurgery, School of Medicine, Fukuoka University, Japan.
Insights
This study details a rare case of caudal regression syndrome combined with an intraspinal arachnoid cyst in an infant. Surgical repair led to a positive outcome with normal development.
Area of Science:
- Pediatric Surgery
- Congenital Abnormalities
- Neuroscience
Background:
- Caudal regression syndrome (CRS) is a rare congenital disorder affecting the lower spine and limbs.
- Intraspinal arachnoid cysts are uncommon lesions that can cause neurological deficits.
Observation:
- A 6-month-old female presented with a complex constellation of congenital anomalies, including CRS and an intraspinal arachnoid cyst.
- The patient exhibited sacrococcygeal dysgenesis, myelocystocele, imperforate anus, omphalocele, and other malformations.
Findings:
- Surgical intervention involved plastic repair for CRS and partial cyst wall removal for the intraspinal arachnoid cyst.
- The patient demonstrated successful postoperative recovery and normal mental development at 3-year follow-up.
Implications:
- This case highlights the successful surgical management of a rare combination of CRS and intraspinal arachnoid cyst.
- Early surgical intervention may lead to favorable outcomes in complex pediatric congenital anomalies.
- Further research into the etiology and management of co-occurring spinal malformations is warranted.
Abstract:
We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.
