Intestinal Malrotation LADD’S PROCEDURE AND LONG-TERM OUTCOME Ali Darehzereshki, MD UW General Surgery Seattle Children’s Hospital February 20, 2014

Intestinal Malrotation Shalaby M S et al. BMJ 2013 Intestinal Malrotation

Intestinal Malrotation Incidence: 1 in 500 live birth (up to 1% in autopsy studies) 80% present within first month of life: Cardinal symptom at this age is bilious vomiting (71-100%). Up to 82% have midgut volvulus at emergency laparotomy. 20% present after 1 year-old: Chronic intermittent abdominal pain and vomiting, as well as failure to thrive are the most common symptoms. Acute presentation with volvulus is less common. Any patient with malrotation is considered at a risk of midgut volvulus.

Ladd WE, NEJM, 1932

Ladd’s procedure for small bowel volvulus Shalaby M S et al. BMJ 2013 Ladd’s procedure for small bowel volvulus What are the long-term outcomes after Ladd’s procedure? Is open approach preferred over laparoscopic procedure?

AC 22 yo male with history of severe reflux in teen yrs, s/p Nissen in 2010. Continued with intermittent abdominal pain and bloating. 1/2011: Presented to ED with acute abdominal pain and diagnosed with cecal volvulus. Underwent ileocecectomy, dissection of mesenteric root and resection of Meckel’s diverticulum. The duodenum and ligament of Treitz was found in the expected anatomic position. 1/2014: Presented to the ED with acute epigastric pain and bilious vomiting.

Duodenal volvulus

AC Underwent an elective ex-lap, lysis of Ladd’s band and widening of mesenteric base on 2/4/14. Uncomplicated course. Tolerated LRD and discharged on POD 5.

HW 15 yo F with complex PMH including congenital anomalies associated with duplication of Ch. 1q, and GERD s/p Nissen/g-tube at age 4-month-old. Presented to OSH on 2/9/14 with one day h/o acute abdominal pain without n/v. CT A/P c/w complete SBO likely d/t cecal volvulus. Transferred to SCH. Emergent exploration confirmed cecal volvulus, non-fixation of right colon but normally positioned LOT and relatively wide mesentry. Underwent removal of Ladd’s band over duodenum and ileocecectomy with primary anastomosis. Hospital course complicated by UTI. Tolerated LRD and discharged on POD 8.

Stauffer et al, J Pediatr Surg, 1980 Comparison of late results in patients with corrected intestinal malrotation with and without fixation of the mesentery 76 with malrotation, s/p Ladd’s ± fixation, 1940-1975 6 required bowel resection, all died. 12 died from other reasons mainly in the early years of the study. 41 included in long-term follow-up (3-37 yrs).

RESULTS Complication rate: 46% 9% wound infection Murphy FL et al, Pediatr Surg Int, 2006 RESULTS Complication rate: 46% 9% wound infection 9% feeding difficulties 2% chronic abdominal pain/2% constipation 26% readmission 24% acute bowel obstruction 13% (6 patients) multiple readmission for SBO 13% reoperation for obstruction (4 adhesion-related and 2 internal hernia) No significant difference in the initial presentation, age and operative findings in those requiring surgery and those who did not. No short-bowel syndrome. Death: 9% (four patients): one from SBO, three from other medical conditions. Conclusion: Significant long-term morbidity is associated with intestinal malrotation even after corrective surgery. Other medical conditions: post liver transplant for biliary atresia, CCHD, sudden infant death

Murphy FL et al, Pediatr Surg Int, 2006 Long-term complications following intestinal malrotation and the Ladd's procedure: a 15 year review. 46 with malrotation, s/p Ladd’s, 1/1986-6/2000 Mean follow-up: 10 yrs (3-18 yrs). 78% emergency Ladd’s. 13% elective Ladd’s in asymptomatic patient. 6.5% intraoperative incidental findings of a malrotation during different procedures 2 patients had laparoscopic Ladd’s. None required bowel resection.

El-Gohary Y et al, Pediatr Surg Int, 2010 Long-term complications following operative intervention for intestinal malrotation: a 10-year review 161 with malrotation s/p Ladd’s, 1/1999-12/2008, Mean follow-up: 5 yrs (8 m-11.5 yrs). 76.4% emergency procedure. Volvulus in 31%. 23.6% elective Ladd’s. 23.6% intraoperative incidental findings of a malrotation during other procedures All open Ladd’s. Two required bowel resection.

RESULTS 74.7% follow up. Complication rate : 8.7% El-Gohary Y et al, Pediatr Surg Int, 2010 RESULTS 74.7% follow up. Complication rate : 8.7%

Other incidents (not included in outcome): El-Gohary Y et al, Pediatr Surg Int, 2010 RESULTS Other incidents (not included in outcome): 6 chronic constipation 5 GERD, 2 required Nissen 2 chronic abdominal pain 1 diarrhea Conclusion: Ladd’s procedure has a low postoperative morbidity and remains a vital treatment for malrotation in children.

Summary The long-term sequelae and late complications of Ladd’s procedure is poorly defined. Incidence of small bowel obstruction associated with operative intervention for malrotation ranges from 5-24%. A past history of Ladd’s procedure does not exclude recurrence of volvulus (2-7% recurrence rate). Whether asymptomatic patients with a documented malrotation require surgery is controversial.

Open vs. Laparoscopic Ladd’s The first report of laparoscopic Ladd’s procedure was in 1995 (Netherland), and shortly thereafter by Waldhausen (1996) and Gross (1996) in the US institutes. Since then it been reported in several studies as a safe, effective and feasible treatment modality for malrotation, even in the presence of volvulus. Advantages of laparoscopic approach include smaller incision, earlier feeding, shorter hospital stay and fewer complications. Open approach may facilitate adhesion development, which decreases the risk for volvulus. The laparoscopic approach, however, is felt to generate less postoperative adhesions, which may not be ideal for this condition.

Matzke GM et al, Surg Endosc, 2005 Surgical management of intestinal malrotation in adults: comparative results for open and laparoscopic Ladd procedures 21 adults included, 1984-2003 10 open vs. 11 laparoscopic Ladd’s Mean age: 36 yr (14-89 yo) Most common presenting symptoms: chronic abdominal pain, nausea and repeated vomiting Volvulus: 5 patients. All underwent open procedure. Upper GI/SBFT had 100% diagnostic accuracy compared to 75 and 60% for CT scan and barium enema, respectively. Conversion rate: 27.2% (3 patients)

RESULTS Mean follow-up: 42 months (2wk- 97m). 86% completed follow up. Matzke GM et al, Surg Endosc, 2005 RESULTS Mean follow-up: 42 months (2wk- 97m). 86% completed follow up. No repeat operation. No volvulus. No 30-day mortality.

Matzke GM et al, Surg Endosc, 2005 CONCLUSIONS The laparoscopic Ladd is feasible, safe, and as effective as the standard open procedure for adults with malrotation and without midgut volvulus. Laparoscopic approach is manifested by an earlier oral intake, a decreased need for intravenous narcotics, and an earlier discharge from the hospital.

The Role of Laparoscopy in the Management of Malrotation Fraser et al, J Surg Res, 2009 The Role of Laparoscopy in the Management of Malrotation Retrospective analysis over 13 years in a single institution. 284 included.

RESULTS Conversion rate: 33% (volvulus or bowel orientation). Fraser et al, J Surg Res, 2009 RESULTS Conversion rate: 33% (volvulus or bowel orientation). Volvulus after Ladd's procedure: 6 (all open, 2.4%). The mean time to volvulus: 378.67 d (range 24–1309)

Fraser et al, J Surg Res, 2009 CONCLUSIONS A laparoscopic Ladd's procedure should be the initial approach in patients with malrotation in the absence of volvulus. A low threshold for conversion to an open approach is encouraged, if there is any concern about volvulus/orientation.

Hagendoorn et al, Surg Endosc, 2011 Laparoscopic treatment of intestinal malrotation in neonates and infants: retrospective study 45 underwent diagnostic laparoscopy, 2/1995-9/2009. 37 diagnosed with malrotation ± volvulus. Average age (yrs): 1.3 ± 0.6 (4 days-13 years) 22/37 younger than 2 months old 9/37 concomitant congenital anomalies. 5/37 concurrent volvulus: four managed laparoscopically. Feeding was resumed on POD1.

RESULTS No 30-day mortality. Hagendoorn et al, Surg Endosc, 2011 RESULTS No 30-day mortality. 9/37 infectious complications (UTI, wound infection and line sepsis)

Hagendoorn et al, Surg Endosc, 2011 CONCLUSION Diagnostic laparoscopy is the procedure of choice when intestinal malrotation is suspected. Malrotation can be treated adequately with laparoscopic procedure in the majority of cases. A low threshold for conversion to an open procedure is mandated to prevent recurrence of malrotation or volvulus.

Stanfill et al, J of Laparoendosc Adv Surg Tech A, 2010 Laparoscopic Ladd’s Procedure: Treatment of Choice for Midgut Malrotation in Infants and Children Retrospective; 156 children included, 9/1998-6/2008. 120 open (OL) vs. 36 laparoscopic Ladd’s (LL). 75% symptomatic in each group. Most common symptoms: emesis and pain. Newborns with congenital diaphragmatic hernia, gastroschisis, or omphalocele were excluded. Follow-up range: 9m-10yrs. Median age: 3 m vs. 7 m in OL vs LL (P=0.004).

Stanfill et al, J of Laparoendosc Adv Surg Tech A, 2010 RESULTS No significant difference for intraoperative findings between the OL vs. LL group, including: Volvulus (16.7 vs 11.1%) Bowel resection (7.7 vs. 0%) Median duration of surgery (76 vs. 75 min). Conversion rate: 8.3% (d/t inadequate visualization) Appendectomy 97%

RESULTS Stanfill et al, J of Laparoendosc Adv Surg Tech A, 2010 Median follow-up: 5.7 vs. 2.4 yrs in the OL vs. LL group.

Stanfill et al, J of Laparoendosc Adv Surg Tech A, 2010 Conclusion Laparoscopic diagnosis and treatment of intestinal malrotation should be considered as the treatment of choice for rotational anomalies in infants and children. Short-term results are superior to OL and can be achieved without any increase in operative duration. Children’s Hospital of Illinois

Summary Laparoscopic Ladd’s procedure is safe, effective and feasible, even in the presence of volvulus Laparoscopic Ladd’s procedure may decrease the overall risk of bowel obstruction. Long-term outcomes for laparoscopic approach remain to be determined.

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