Maria Hukkinen1, Antti Koivusalo2, Risto J Rintala2, Mikko P Pakarinen2. 1. Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Finland. Electronic address: maria.hukkinen@helsinki.fi. 2. Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Finland.
Abstract
BACKGROUND: No consensus exists on the optimal surgical management of total colonic aganglionosis (TCA). Outcomes after restorative proctocolectomy (RPC) as the initial reconstructive procedure among neonatal and infant TCA patients have not been evaluated previously. METHODS: Medical records of patients with Hirschsprung disease (HD) who underwent RPC during infancy between 1997 and 2012 (n=8) were reviewed. Bowel function and satisfaction with operative results were assessed in a follow-up interview. RESULTS: Median age at RPC was 1.1 months, and covering loop ileostomies were closed 3.7 months later. No operative complications occurred. Hospitalizations for enterocolitis and obstruction occurred each in 50% of patients postoperatively. Enterocolitis-associated outlet obstruction occurred in one third of patients, most of whom responded well to intersphincteric botulinum toxin (botox) injections. No pouchitis or elevated fecal calprotectin levels (median 51 μg/g) were observed. At last follow-up 3.2 years after ileostomy closure, the median 24-hour stooling frequency was 3.5. None had socially limiting fecal incontinence or problems in holding back defecation. Parent satisfaction with operative results was high. CONCLUSIONS: The rate of postoperative enterocolitis was similar to other procedures, but a better functional outcome was achieved. Botox injections were effective for postoperative functional outlet obstruction. Short-term results following RPC among neonates and infants are promising.
BACKGROUND: No consensus exists on the optimal surgical management of total colonic aganglionosis (TCA). Outcomes after restorative proctocolectomy (RPC) as the initial reconstructive procedure among neonatal and infant TCA patients have not been evaluated previously. METHODS: Medical records of patients with Hirschsprung disease (HD) who underwent RPC during infancy between 1997 and 2012 (n=8) were reviewed. Bowel function and satisfaction with operative results were assessed in a follow-up interview. RESULTS: Median age at RPC was 1.1 months, and covering loop ileostomies were closed 3.7 months later. No operative complications occurred. Hospitalizations for enterocolitis and obstruction occurred each in 50% of patients postoperatively. Enterocolitis-associated outlet obstruction occurred in one third of patients, most of whom responded well to intersphincteric botulinum toxin (botox) injections. No pouchitis or elevated fecal calprotectin levels (median 51 μg/g) were observed. At last follow-up 3.2 years after ileostomy closure, the median 24-hour stooling frequency was 3.5. None had socially limiting fecal incontinence or problems in holding back defecation. Parent satisfaction with operative results was high. CONCLUSIONS: The rate of postoperative enterocolitis was similar to other procedures, but a better functional outcome was achieved. Botox injections were effective for postoperative functional outlet obstruction. Short-term results following RPC among neonates and infants are promising.
Authors: Kristiina Kyrklund; Cornelius E J Sloots; Ivo de Blaauw; Kristin Bjørnland; Udo Rolle; Duccio Cavalieri; Paola Francalanci; Fabio Fusaro; Annette Lemli; Nicole Schwarzer; Francesco Fascetti-Leon; Nikhil Thapar; Lars Søndergaard Johansen; Dominique Berrebi; Jean-Pierre Hugot; Célia Crétolle; Alice S Brooks; Robert M Hofstra; Tomas Wester; Mikko P Pakarinen Journal: Orphanet J Rare Dis Date: 2020-06-25 Impact factor: 4.123
Authors: P Stenström; K Kyrklund; M Bräutigam; H Engstrand Lilja; K Juul Stensrud; A Löf Granström; N Qvist; L Söndergaard Johansson; E Arnbjörnsson; H Borg; T Wester; K Björnland; M P Pakarinen Journal: BJS Open Date: 2020-07-13