WOFAPS 2025 8th World Congress of Pediatric Surgery

Chieko Hisamatsu, Wataru Sasaki, Ayano Uematsu, Shohei Yoshimura, Yuichiro Tomioka, Keisuke Fukui, Taichi Nakatani, Shizu Murakami, Akiko Yokoi, Tadashi Hatakeyama

Kobe Children's Hospital, Department of Pediatric Surgery, Kobe, Japan

Purpose

Currarino syndrome is a rare genetic disorder characterized by the triad of anorectal malformations, presacral masses, and sacral malformations. We reviewed the diagnosis and management of this syndrome.

Method

The medical records of 10 children with Currarino syndrome treated at our hospital between 1993 and 2024 were retrospectively reviewed.

Results

Seven patients were boys. There were 4 sets of family histories.

Diagnosis: The initial symptom was constipation in all patients. Findings of anorectal malformations included anal stenosis (n=7), imperforate anus (n=2), and anal duplication (n=1). Cases of anal stenosis require differentiation from Hirschsprung’s disease or imperforate anus. Presacral masses and sacral malformations were confirmed by imaging.

Treatment: All patients with anal stenosis were managed with bougies, laxatives, and enemas. One underwent anoplasty after colostomy. Patients with an imperforate anus and anal duplication underwent radical surgery. Presacral masses were removed in 7 of the 10 cases. In 5 cases, the mass also occupied the spinal canal and was removed with neurosurgery. One patient underwent simultaneous presacral mass excision and anoplasty for an imperforate anus. Histopathological examination of the masses revealed mature teratoma (n=5) and lipomeningocele (n=2). In 6 patients, the spinal cord was untethered by neurosurgery. Perioperative complications included rectal injury (n=2), urethral injury (n=1), transient neurogenic bladder (n=2), and wound dehiscence of anoplasty (n=2).

Conclusion

The diagnosis of constipation requires exclusion of Currarino syndrome. The treatment plan for this syndrome is determined by the abdominal and neurological symptoms and the location of the presacral mass. Some presacral masses are adherent to the rectum. In a recent case of mass excision (simultaneous with anoplasty), intraoperative placement of a biologically transparent illumination device in the rectum was useful in preventing rectal injury.