MANAGEMENT OF FIVE HUNDRED PATIENTS WITH MALROTATED GUT: THIRTY YEARS OF EXPERIENCE WITH THE INTRODUCTION OF NOVEL SURGICAL TECHNIQUES
Kareem Abu-Elmagd1, George Mazariegos2, *Neha Parekh1, *Ajai Khanna2, *Marissa Scalish1, *Sherif Armanyous1, *Mohammed Osman1, *Masato Fujiki1, *Giuseppe D'Amico1, *Guilherme Costa1, *Erick Remer1, Matthew Walsh1
1Cleveland Clinic, Cleveland, OH;2University of Pittsburgh Medical Center, Pittsburgh, PA
OBJECTIVE(S): Define the clinicopathologic spectrum and long-term outcome of gut-malrotation in both adults and children. With new insights, a novel surgical procedure was introduced and assessed in reference to the gold standard Ladd’s procedure.
METHODS: Over 30-years, 500 gut-malrotation patients were managed at two institutions. With 274(55%) adults and 226(45%) children, the congenital disorder was non-rotation in 290(58%), and typical/atypical mal-rotation in 210(42%). Surgery was performed in 361(72%) patients with 139(28%) treated conservatively. Urgent intervention was required for mid-gut-volvulus in 121(34%) with ultimate need for gut-transplantation in 113(93%). Elective Ladd’s was performed in 149(41%) patients for symptomatic(n=110) and incidental(n=39) diagnosis. Progression of gastrointestinal-symptoms, despite prior Ladd’s(n=44), dictated the need for definitive surgery in 91(25%) patients. The new enteromesenteric correction (EMC) procedure entailed completion of the 2700 embryonic rotation with correction of vascular-inversion and fixation of mesenteric-attachments. Partial colectomy(n=42) was added for concomitant colonic-dysmotility.
RESULTS: With 18% overall mortality, cumulative 10-year survival was 51% post-transplant, 85% post-Ladd’s, and 100% post-EMC. Despite risk-reduction of mid-gut-volvulus, Ladd’s was associated with cumulative worsening of preoperative gastrointestinal-symptoms-scales. Conversely, EMC significantly(p=0.001) improved NIH-PROMIS-gastrointestinal frequency and severity indices with 3% postoperative Clavien-Dindo-grade-III-IVa complications. EMC also improved(p<0.05) quality-of-life domains and was cost-effective compared to Ladd’s and transplantation. Age, sex, malrotation-type, presentation, treatment-modality, and associated-anomalies were significant predictors of outcome.
CONCLUSIONS: Congenital malrotation is a syndrome of all ages with a spectrum of mesenteric and neuroenteric defects. The described-herein procedure is safe and effective in management of the underlying pathophysiologic disorders. A novel model was established to predict outcomes.
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