Currarino Syndrome: lower urinary tract function, treatment plan and outcomes

Currarino syndrome (CS) is a rare congenital disorder characterized by anorectal malformation, sacral agenesis and presacral mass. We performed video-urodynamics (VUDS) assessment of patients with CS to characterize the lower urinary tract function, individualize management plans and to follow outcomes. We conducted a cross-sectional study on 11 patients diagnosed with CS at the spina bifida multidisciplinary clinic. Lower urinary tract function was assessed by VUDS from three months to 12 months after neurosurgery. All patients had sacral agenesis; nine patients had anorectal malformation (9/11, 81.8%) and five patients had a presacral mass (5/11, 45.5%). The average age at neurosurgery was 7.9 months (range, 2–19). One patient had bilateral vesicoureteral reflux (VUR) with increasing detrusor pressure at the end of filling. In two patients, the detrusor activity showed weakening during urination, while no other lower urinary tract abnormality was identified on urodynamic evaluation. Six patients underwent VUDS before and after surgery; of these, two patients showed improved bladder function after surgery, while the remaining four patients showed no change in urodynamics. The average duration of follow-up was 27.5 months (range, 9–51). Renal function was normal in all patients. We can conclude that patients with CS often exhibit associated neurological abnormalities. VUDS assessment may help detect lower urinary tract dysfunction at an early stage and facilitate timely urological intervention to avoid kidney damage.

Sacral Agenesis: A Neglected Deformity That Increases the Incidence of Postoperative Coronal Imbalance in Congenital Lumbosacral Deformities

Study Design: A retrospective study. Objectives: To identify if there is a link between sacral agenesis (SA) and post-operative coronal imbalance in patients with congenital lumbosacral deformities. Methods: This study reviewed a consecutive series of patients with congenital lumbosacral deformities. They had a minimum follow-up of 2 years. According to different diagnosis, they were divided into SA and non-SA group. Comparison analysis was performed between patients with and without post-operative coronal imbalance and risk factors were identified. Results: A total of 45 patients (18 in SA group and 27 in non-SA group) were recruited into this study, among whom 33 patients maintained coronal balance while 12 demonstrated postoperative coronal imbalance at last follow-up (14.32 ± 7.67 mm vs 35.53 ± 3.91 mm, P < 0.001). Univariate analysis showed that preoperative lumbar Cobb angle, immediate postoperative coronal balance distance and diagnosis of SA were significantly different between patients with and without post-operative coronal imbalance (P < 0.05). Binary logistic regression analysis showed that SA was an independent risk factor for postoperative coronal imbalance. Conclusions: As an independent risk factor for postoperative coronal imbalance, high level of suspicion of SA should be aware in children with congenital lumbosacral deformities. Sufficient bone grafts at sacroiliac joint are recommended for SA patients to prevent postoperative coronal imbalance.

A STUDY OF DORSAL SACRAL AGENESIS IN THE REGION OF EASTERN UTTAR PRADESH

Background: Sacrum is a triangular shaped irregular bone formed by fusion of five sacral vertebrae. Sacrum is also called ‘Hieron osteon’ meaning the holy or sacred bone. Methods: The present study has been carried out on 98 completely ossified undamaged dry human sacra of undetermined age and sex in the department of Anatomy in the population of Eastern Uttar Pradesh. Each sacrum is examined for the agenesis of the dorsal surface. Representative photographs of different sacrum having partial or complete agenesis on the dorsal surface are taken using a digital camera Result: Elongated sacral hiatus i.e. length more than 40 mm was seen in 5 (5.1%) bones. Maximum sacral hiatal length of 52.6 mm was noted. Elongated sacral hiatus were of two types i.e. inverted U and inverted V shaped. 4 (4.08%) bones showed partial dorsal agenesis of sacrum. Complete dorsal agenesis was seen in 1 sacrum. One bones with absent sacral hiatus was seen. Conclusion: Various anomalies on dorsal surface of sacrum were seen in Indian population. Knowledge of these variations will be of help to the Anaesthetists. We conclude that, variant anatomy on dorsal surface of sacrum make administration of caudal epidural anaesthesia difficult. So ultrasound or fluroscopic guidance leads to better patient outcome while giving caudal blocks. Keywords: Dorsal sacral agenesis, sacral hiatus, caudal epidural block