Surgical management of intestinal malrotation in adults: comparative results for open and laparoscopic Ladd procedures

Background This study aimed to characterize the clinical^; features of intestinal malrotation in adults, and to compare the results for the open and laparoscopic Ladd procedures. Methods Between 1984 and 2003, 21 adult patients with a mean age of 36 years (range, 14–89 years) were surgically treated for intestinal malrotation. The clinical data collected included age, gender, presenting symptoms, diagnostic tests, type of operation, operative time, narcotic requirement, time to oral intake, length of hospital stay, and outcome. The groups (open vs laparoscopic) were comparatively analyzed using two-sample t-tests and Wilcoxon rank sum tests. Results The two groups were similar in terms of age, clinical presentation, and diagnostic tests performed. The most common presenting symptoms were chronic abdominal pain, nausea, and repeated vomiting. Upper gastrointestinal barium studies (UGI/SBFT) were diagnostic for all patients with malrotation as compared with computed tomography (CT) scanning, which was falsely negative in 25% of patients. A total of 21 patients underwent the Ladd procedure, either open (n = 10) or laparoscopic (n = 11). Three laparoscopic procedures were converted to open. Overall, the laparoscopic group resumed oral intake earlier than the open group (1.8 vs 2.7 days; p = 0.092), had a shorter hospital stay (4.0 vs. 6.1 days; p = 0.050), and required less intravenous narcotics on postoperative day 1 (4.9 vs 48.5 mg; p = 0.002). The laparoscopic group underwent a longer operation (194 vs 143 min; p = 0.053). Sixteen of eighteen patients available for follow-up reported complete resolution of symptoms, 2 felt greatly improved. No patient required a second operation related to volvulus or recurrent symptoms. Conclusions The laparoscopic Ladd procedure is feasible, safe, and as effective as the standard open Ladd procedure for the treatment of adults who have intestinal malrotation without midgut volvulus. Patients also benefit from this minimally invasive approach, as manifested by an earlier oral intake, a decreased need for intravenous narcotics, and an earlier discharge from the hospital.     

Acute colonic obstruction from Ladd bands: a unique complication from intestinal malrotation

In patients with intestinal malrotation, Ladd bands are known to cause obstruction of the duodenum and small bowel. However, acute obstruction of the colon from Ladd bands has not been previously described. We report a case of complete obstruction of the hepatic flexure of the colon secondary to a Ladd band in a previously healthy teenage boy.     

Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation

Purpose

Heterotaxy syndrome (HS) patients often present with congenital heart disease and intestinal malrotation. Controversy exists regarding the management of these patients. Risk of midgut volvulus, morbidity from elective operations, and overall prognosis must be weighed when considering a Ladd procedure on asymptomatic HS/intestinal malrotation patients.

Methods

This is a retrospective review comparing HS and non-heterotaxy syndrome (NHS) patients undergoing a Ladd procedure at Children's Hospital Boston (Mass) from January 1997 to September 2007.

Results

Thirty-one HS and 51 NHS patients were identified. After a Ladd procedure, HS patients remained in the hospital 12.9 days, had a 9.7% risk of small bowel obstruction, and a 9.7% in-hospital mortality. If allowed to develop abdominal symptoms, 27% of HS patients with intestinal malrotation had a midgut volvulus at surgery. Intra-HS group comparison demonstrated longer hospital stays in symptomatic patients (P = .01). Mortality was greater in the HS than NHS patients, but deaths were related to cardiac disease and not to the Ladd procedure.

Conclusions

Elective Ladd procedures are well tolerated by HS patients. Given the risk of midgut volvulus and in light of improved survival beyond infanthood, once identified, HS patients with asymptomatic malrotation should be offered a prophylactic Ladd procedure.     

Value of laparoscopy in children with a suspected rotation abnormality on imaging

Background

Although imaging is usually used for the diagnosis of rotation abnormalities, significant false-positive and false-negative rates have been reported. We studied the utility of laparoscopy in the management of children with a suspected rotation abnormality on imaging.

Methods

The charts of all children undergoing laparoscopy for a suspected intestinal rotation abnormality from January 2000 to August 2009 were retrospectively reviewed.

Results

There were 51 patients. Preoperative diagnosis based on upper gastrointestinal contrast study with or without contrast enema or ultrasound was malrotation without volvulus in 47%, malrotation with volvulus in 10%, and nonrotation in 6%; the other 37% had equivocal or inconclusive imaging studies. Of the patients who had a “definitive” preoperative diagnosis, 41% had a discrepant finding at laparoscopy. For those with inconclusive imaging studies, 32% were found on laparoscopy to have a narrow mesenteric base, which put them at significant risk of midgut volvulus.

Conclusion

Imaging studies may be inaccurate in differentiating malrotation from nonrotation or normal rotation. Laparoscopy provides an excellent opportunity to assess the base of the mesentery. Those children without a narrow-based mesentery can undergo laparoscopy alone, and those with malrotation should undergo either laparoscopic or open Ladd procedure.     

Assessment of recurrent abdominal symptoms after Ladd procedure: clinical and radiographic correlation

Background/purpose

Obstructive symptoms in a patient post-Ladd procedure raise the question of recurrent volvulus. Our objective is to determine the incidence and the radiographical evaluation of recurrent volvulus and abdominal complications after a Ladd procedure.

Methods

One hundred ninety-five patients who underwent a Ladd procedure for malrotation for 10 years were retrieved from a database. Forty-eight patients were excluded (false-positive studies, heterotaxy, diaphragmatic hernia, abdominal wall defects). Of the remaining 147, 38 patients presented with abdominal symptoms. Surgical records and imaging were reviewed.

Results

Of 38 patients, 33 had imaging studies including abdominal radiographs (AXR) and/or upper gastrointestinal examinations. Of these 33 patients, 17 had normal or expected imaging findings on AXR and/or upper gastrointestinal. Of the 11 patients who had surgery, 8 had an AXR. Of these, only a single patient had a normal AXR. The most common post-Ladd complication found at reoperation was small bowel obstruction secondary to adhesions (5.4%). One patient (0.7%) had midgut volvulus.

Conclusion

Although recurrent volvulus is a feared postoperative Ladd complication, it rarely occurs. Adhesive small bowel obstruction is more common, and an AXR with clinical findings is sufficient for diagnosis.     

Computed tomographic diagnosis of midgut volvulus in a 5-year-old child

Malrotation with midgut volvulus requires prompt recognition and urgent treatment. We describe a 5-year-old boy who presented with malrotation and midgut volvulus in whom the diagnosis was made by computed tomography. We describe the diagnostic images and operative findings in the patient. This case highlights the importance of recognizing the inversion in orientation of the superior mesenteric vessels and “whirlpool” sign in making the diagnosis of midgut volvulus when few other clues may be present.     

Laparoscopic Ladd's procedure for malrotation in infants and children is still a controversial approach

BackgroundOpen Ladd's procedure is the gold standard for the correction of intestinal malrotation and laparoscopic approach remains controversial. This study aimed to evaluate our experience in laparoscopic management of malrotation.MethodsSingle center retrospective study including patients who underwent a laparoscopic assessment of intestinal malrotation with correction if appropriate between 2007 and 2017.ResultsSixty-five patients (median age 7 months) had a laparoscopic assessment with and without correction of malrotation. Forty-five (69%) were symptomatic, including 16 (25%) with a midgut volvulus. The procedure was completed laparoscopically in 55 (86%) patients in 110 min (30–190). Conversions happened more frequently at the beginning of the experience. With a follow-up of 12.5 months (8 days–5.3 years), morbidity rate was 15% and 4 (6%) patients underwent a redo surgery, all in the first 5 months after surgery, compared with 3/53 (6%) in a contemporaneous group undergoing open Ladd's.ConclusionThis is the largest series reported so far of the laparoscopic management of malrotation. Laparoscopic Ladd's procedure is reliable but still exposes to open conversion which may be in part owing to a learning curve. A low conversion threshold is important in cases with volvulus. The redo rate is similar to that of the open procedure.Level of evidenceLevel III retrospective comparative treatment study.     

Heterotaxy syndrome and malrotation: Does isomerism influence risk and decision to treat

Purpose

Controversy remains regarding the management of the asymptomatic heterotaxy syndrome (HS) patient with suspected intestinal rotational abnormalities. We evaluated the outcomes for our HS population to identify frequency of malrotation and identify characteristics of children who might benefit from expectant management.

Methods

After IRB approval, a retrospective review of all patients treated for HS at a large tertiary care children’s hospital between January 2008 and June 2012 was performed. For the purpose of this paper, malrotation was defined as an operative note that described the presence of Ladd’s bands and a narrow mesentery.

Results

Thirty-eight patients with HS were identified, including 18 who underwent abdominal exploration. Left atrial isomerisation (LAI) was identified in 13 individuals, and right atrial isomerisation (RAI) was noted in 25. The rate of surgical intervention did not vary between the 2 groups (54%). Malrotation was found in 8 patients: one with LAI and 7 with RAI. This difference in incidence was statistically significant (p = 0.04).

Conclusion

These data suggest that the direction of atrial isomerisation influences the likelihood of true malrotation, where RAI patients are more likely to be malrotated. Given the inherent risk of surgery on this medically fragile patient population, surgeons should consider expectant management for asymptomatic LAI patients.     

Outcomes After Ladd Procedures for Intestinal Malrotation in Newborns with Heterotaxy Syndrome

PurposeIntestinal malrotation may be asymptomatic in patients with heterotaxy syndrome (HS), and whether these newborns benefit from prophylactic Ladd procedures is unknown. This study sought to uncover nationwide outcomes of newborns with HS receiving Ladd procedures.MethodsNewborns with malrotation were identified from the Nationwide Readmission Database (2010-2014) and stratified into those with and without HS utilizing ICD-9CM codes for situs inversus (759.3), asplenia or polysplenia (759.0), and/or dextrocardia (746.87). Outcomes were analyzed using standard statistical tests.Results4797 newborns with malrotation were identified, of which 16% had HS. Ladd procedures were performed in 70% overall and more common in those without heterotaxy (73% vs. 56% HS). Ladd procedures in newborns with heterotaxy were associated with higher complications compared to those without HS including surgical site reopening (8% vs. 1%), sepsis (9% vs. 2%), infections (19% vs. 11%), venous thrombosis (9% vs. 1%), and prolonged mechanical ventilation (39% vs. 22%), all p < 0.001. HS newborns were less frequently readmitted with bowel obstructions (0% vs. 4% without HS, p < 0.001) with no readmissions for volvulus in either group.ConclusionLadd procedures in newborns with heterotaxy were associated with increased complications and cost without differences in rates of volvulus and bowel obstruction on readmission.Type of studyRetrospective Comparative.Level of evidenceIII.     

Delayed presentation of familial intestinal malrotation with volvulus in two adult siblings

Intestinal malrotation is an uncommon cause of abdominal pain and normally presents during infancy. Familial cases of malrotation are extremely rare in the absence of other congenital malformations. We present the case of a 22-year-old woman with undiagnosed chronic abdominal pain and her previously well 16-year-old brother who presented within 18 months of each other with acute midgut volvulus secondary to intestinal malrotation. Clinicians should be aware of this rare but serious cause of abdominal pain.     

Conditions required for laparoscopic repair of subacute volvulus of the midgut in neonates with intestinal malrotation: 5 cases

Background This study aimed to evaluate the optimal conditions for laparoscopic management of neonatal subacute volvulus with malrotation.Methods Between 1994 and 2002, 13 neonates with midgut volvulus and malrotation entered the authors institution. Five of these neonates met the eligibility criteria for laparoscopy: good hemodynamic parameters, no gut perforation, and no severe ischemic distress of the bowel shown on preoperative ultrasonography.Results The results were excellent for only three infants, associated with a shorter postoperative course. One underwent reoperation for a residual duodenal band, and one conversion to open laparotomy was necessary. The predictors of success were no difficulty identifying the abnormal anatomy, no important chylous stasis, and the learning curve of the surgeon.Conclusions Despite its previously reported feasibility, neonatal laparoscopy for volvulus with intestinal malrotation is appropriate only for a small number of patients. Strict selection criteria with a clear understanding of the optimal conditions for success may improve the outcome.     

Laparoscopic evaluation and treatment of intestinal malrotation in infants

Infants with intestinal malrotation present with bilious emesis and the diagnosis is generally obtained by an upper gastrointestinal barium study. Malrotation is suspected if the ligament of Treitz is not positioned to the left of the vertebral body. Barium enema may also be used to detect malrotation by noting the abnormal position of the cecum from its usual placement in the right lower quadrant, but this study is not as reliable due to the mobility of the cecum. Some infants may not have classic radiographic findings for malrotation, yet the contrast studies are not entirely normal. We recently treated two infants with recurrent vomiting whose UGI studies suggested intestinal malrotation. Laparoscopic exploration confirmed the diagnosis of malrotation. Laparoscopic correction (Ladd's procedure) of malrotation was carried out in one infant. The second infant underwent a traditional Ladd's procedure. The technique of laparoscopic Ladd's procedure is described. Laparoscopy may be used for the diagnosis and treatment of infants with intestinal malrotation. It may be especially helpful to verify the diagnosis in patients who do not have classic radiographic findings. Whether laparoscopy should be used in patients with midgut volvulus is debatable. Laparoscopic derotation of the volvulus in a setting where the bowel is markedly distended may be difficult and dangerous.     

The changing spectrum of intestinal malrotation: diagnosis and management

Background

Management of typical malrotation is universally accepted, but management of atypical malrotation is less well defined in both children and adults.

Methods

Records of patients with malrotation diagnosed over 6 years were reviewed. Patients were grouped into typical or atypical based on ligament of Treitz location. Outcomes were evaluated using chi-square analysis.

Results

Of 275 patients, 148 diagnosed with typical malrotation underwent Ladd’s procedure. Based on symptoms, 91 of 127 patients with atypical malrotation were managed operatively. The remaining 36 patients were asymptomatic or had reflux symptoms only and were observed. Six of 36 subsequently required surgery due to symptoms, but 30 remain asymptomatic. No observed patients developed acute midgut volvulus. The overall postoperative complication rates were higher for atypical versus typical malrotation, 27% versus 16% (P < .05).

Conclusions

Close observation with repeat contrast study is an acceptable management option for patients with atypical malrotation who are asymptomatic or exhibit only reflux symptoms.     

The optimal management of malrotation diagnosed after infancy: a decision analysis

BACKGROUND: The benefit of a prophylactic Ladd's procedure in older children and adults with malrotation is controversial. The purpose of this study was to determine the role of the Ladd's procedure in patients with asymptomatic malrotation diagnosed after infancy. METHODS: A Markov decision analysis was used to compare the quality adjusted life expectancy with and without a Ladd's procedure among patients with asymptomatic malrotation. Data obtained from the Nationwide Inpatient Sample were used to estimate the age-related probability of emergency surgery or volvulus among patients with malrotation. Estimates of the mortality of elective and emergency surgery, mortality of volvulus, and utilities of each health state were obtained from the literature. RESULTS: After infancy, the gain in quality adjusted life expectancy associated with a prophylactic Ladd's procedure was highest when asymptomatic malrotation was treated at 1 year old and steadily declined until asymptomatic malrotation was treated at 20 years old. An increasing advantage of observation over prophylactic surgery on life expectancy was observed after the second decade of life. A 2-fold increase in mortality risk for an elective Ladd's procedure decreased the age threshold to 14 years, whereas a 4-fold increase decreased the threshold to 7 years. These results were found to be robust by sensitivity analyses and Monte Carlo simulation. CONCLUSION: A Ladd's procedure should be considered for children diagnosed with asymptomatic malrotation, particularly those who are younger and with a low risk of postoperative mortality. The rare occurrence of midgut volvulus does not justify performing a prophylactic Ladd's procedure on most adults with malrotation.     

Small bowel volvulus with no malrotation after laparoscopic appendicectomy: Case report and literature review

INTRODUCTION

Small bowel volvulus, as a complication of laparoscopic surgery, is a rarely reported clinical entity. We present a case of a young female who developed small intestinal volvulus after laparoscopic appendicectomy. She had this complication in the absence of malrotation or other previous abdominal operations.

PRESENTATION OF CASE

A 17-year-old woman presented with acute appendicitis. After an uneventful laparoscopic appendicectomy, she developed acute small intestinal obstruction on the second post-operative day. A prompt laparotomy showed small bowel volvulus, which was reduced, with no evidence of malrotation. She had an uneventful recovery and was discharged within 3 days of the second operation, in a stable condition.

DISCUSSION

This article presents a review of the literature of this rare cause of small intestinal obstruction after laparoscopic surgery, stressing on the importance of early diagnosis and treatment. A discussion of the potential factors predisposing to this entity is presented, emphasising the need of a higher-evidence study as to its aetiology and prevalence.

CONCLUSION

Small bowel volvulus is a rare complication of laparoscopic surgery, but its early diagnosis and prompt treatment is essential to avoid morbid outcomes. Surgery is the therapeutic mainstay.     

Colocolic intussusception in a preterm infant with intestinal malrotation

The association of intussusception and intestinal malrotation is referred to as Waugh syndrome. A prospective study among 49 children with intussusception found a 40% incidence of malrotation. We describe the rare occurrence of colocolic intussusception in a neonate with malrotated intestine. We believe this is the first reported case in the neonatal period where the pathogenic lead point was an intestinal lymphangioma.