Intestinal Malrotation and Volvulus in Neonates: Laparoscopy Versus Open Laparotomy

Introduction

Intestinal malrotation is a common name for a large variety of abnormalities of intestinal rotation and attachments, ranging from a mobile cecum, with the duodenojejunal junction to the right of the spine, to a complete nonrotation with or without an associated midgut volvulus and reversed rotation. ¹ Intestinal malrotation implies the risk of a midgut volvulus because of a narrowed mesenteric base. Most patients with midgut volvulus present with this complication during the first year of life, usually within the first 7 days. ² A severe vascular compromise urges to surgery either by laparoscopy or by laparotomy and consists in derotation, widening of the mesenteric base, and, when appropriate, division of Ladd's bands. The procedure described by Ladd in 1938 is still the basis for surgical treatment of malrotation. An incidental appendectomy may be performed as well.

Successful laparoscopic management of malrotation has been described in a number of series since the first reports in 1998.^3,4 However, there have been scarce reports on the feasibility and the safety of laparoscopy for the treatment of malrotation and volvulus in neonatal age (0–40 days of life). This report compares retrospective analysis of the clinical outcomes of a group of neonates who underwent laparoscopic treatment for intestinal malrotation and volvulus with that of a similar group subjected to classical open Ladd's procedure in four different European University Institutions.

Materials and Methods

Between January 1993 and January 2014, 56 neonates underwent surgery for suspected intestinal malrotation and volvulus. The indication was based on clinical signs and symptoms as well as investigations. Clinical information including age, sex, associated malformations, presenting symptoms, imaging studies, surgical management, and outcome were documented. Among 56 patients, we decided to consider the group of neonates laparoscopically treated (Group A, n = 20) and to compare it with an equal number of neonates treated with the classical open Ladd's procedure (Group B, n = 20). Patients in Group B were selected on the basis of homogeneous data than patients in Group A, including gestational age, APGAR score, associated malformations, age, and weight at surgery time.

All surgeries were performed at one of the four European University Hospitals included in this study (University Hospitals of Strasbourg [France], University Hospital of Lausanne [Switzerland], Lapeyronie University Hospital of Montpellier [France], and University Hospital of Luxembourg [Luxembourg]) done by or under direct supervision of senior attending pediatric surgeons. Laparoscopic surgeries were performed with the child in supine position, initially with three 5 mm trocars and later with two 3 mm trocars for instruments. The duration of the follow-up was 19 years (range, 2–19 years) for Group A and 22 years (range, 1–22 years) for Group B.

Concerning the statistical analysis, groups were compared either using a two-sample t-test for continuous variables or using Fisher's exact test for categorical variables. One-sided P values were calculated for the 95th confidence interval, and those P values less than or equal to .05 were considered significant.

Results

Discussion

The incidence of intestinal malrotation is about 1 in 500 live births, although autopsy studies estimate that it may be as high as 1% of the total population. ⁵ The prevalence of volvulus is difficult to quantify, but any patient with malrotation is considered to bear a risk of midgut volvulus. Our sex ratio of 30 boys and 10 girls on neonates diagnosed with intestinal malrotation is in accordance with the literature, in which males are said to be slightly more affected than females. ⁶ A total of 17.5% of patients had concomitant abnormalities, such as urological malformations (hypospadias and renal dysplasia), cardiac malformations, or others (palpebral hemangioma and mucoviscidosis). A total of 67.5% of patients presented during the first week of life and more than 85% before the end of the first month.

Clinical diagnosis of malrotation with volvulus is based on a high index of suspicion. The most frequent symptom was bile-stained emesis. Bile vomiting should be considered to have a surgical cause until proven otherwise, as clinical distinction from nonsurgical causes is difficult. ⁷ Pain or irritability is not a prominent clinical feature in the neonate, but is a common feature in the toddler and older child. This finding was replicated in our study with all the neonates presenting with bilious vomiting. In this report, 15 patients (37.5%) had disturbance of stooling pattern: variation of stooling pattern was noted in 16% of patients with malrotation by Millar et al. ⁸

Upper gastrointestinal series contrast study is generally considered to be the preferred examination for the diagnosis of intestinal malrotation. Despite this, in our series ultrasound scanning of the mesenteric vessels was the diagnostic test most frequently used in emergency because it was performed on 97.5% of the patients. In view of these data, we can suggest that ultrasound scanning of the mesenteric vessels can be a reasonable alternative to upper gastrointestinal contrast study (especially in the cases of suspected volvulus with risk of perforation) and may be a useful adjunctive to assess the peristalsis and vascularity of the bowel in equivocal cases.

Orzech et al. reported sensitivity of 86.5%, specificity of 75%, positive predictive value of 42%, and negative predictive value of 96%. ⁹ Millar et al. found that abdominal radiographs may show a dilated duodenum with a fluid level and some gas in the distal bowel, but that can be misinterpreted as normal in 20% of cases. In this report, 65% of neonates with clinical suspicion of malrotation and volvulus were subjected to a plain abdominal radiograph that proved to be positive only in 50% of the cases. The contrast examination with a volvulus could show a dilated duodenum with a typical whirlpool sign projecting forward away from the posterior abdominal wall on an oblique view. ¹⁰ It was done in 42.5% of our patients and was suggestive of malrotation or duodenal–jejunal atresia/stenosis in 90% of cases.

Ladd's procedure is the treatment of choice for all types of malrotations. This involves counterclockwise reduction of the volvulus if present, division of any coloduodenal bands, widening of the mesenteric base to prevent repeated volvulus, and prophylactic appendectomy to prevent diagnostic confusion in the future. Finally, it is important to replace the bowel in a position of nonrotation with the cecum placed into the left upper quadrant.

The debate between open and laparoscopic approaches on Ladd's procedure is still open. On one hand, as Fraser et al. ¹¹ and Nasir et al. ⁸ suggested, the steps of the Ladd's open procedure decrease the risk for volvulus because they required much handling and manipulation of the bowel, thus facilitating adhesion development. However, this can also be a major cause of morbidity and mortality. On the other hand, the newly evolving laparoscopic approach for Ladd's procedure may reduce the incidence of postoperative adhesion in addition to the three main advantages of the laparoscopic approach that include minimized postoperative pain, decreased postoperative ileus, and brief hospital stay. Houben et al. ¹ suggested the fixation of the cecum to the left lateral abdominal wall in the laparoscopic approach, but, because the role of postoperative adhesion formation in the efficacy of Ladd's procedure is doubtful, we suggest that cecopexy can be omitted, just as it was eventually abandoned in the classic Ladd operation.

With regard to the treatment of malrotation with volvulus specifically in neonatal age, there is a paucity of data on the laparoscopic approach. Therefore, we have analyzed our experience with the open and laparoscopic Ladd's procedures in neonates to attempt to identify the feasibility, the safety, and any advantages based on approaches. In our series, the rates of conversion are acceptable and do not differ significantly from other reports in the literature. ¹²

Concerning the perioperative outcomes, a significant difference in the average operating time between the groups was noticed: 80 minutes in Group A versus 61 minutes in Group B (P = .04). This finding could be a point in favor of the laparoscopic approach if we consider that a shorter operating time could reduce further complications in neonates such as excessive decrease of corporeal temperature, postoperative ileus, and decrease of narcotic usage.

Analyzing the postoperative outcomes, we noticed a significant difference between the groups both in the median hospital stay (10.3 days in Group A versus 20.7 days in Group B, P = .04) and in the median time to full diet (4.3 days in Group A versus 6.7 days in Group B, P = .02). These results could further support the use of laparoscopy in children with malrotation. Recurrence of occlusive symptoms is not a rare event both in laparoscopic and in open approaches. This may be because of both insufficient division of the Ladd's bands and postsurgical adhesive syndrome. ¹³ However, it requires surgical management in most cases. ¹⁴ In our experience, recurrence of occlusive symptoms occurred in 30% of patients in Group A and 40% of patients in Group B, with a follow-up time of 19 years for the laparoscopic group (range, 2–19 years) and 22 years (range, 1–22 years) for open surgery group. The mean time of readmission for the 6 patients of Group A was 15.3 days (range, 4–60 days) and that for the 8 patients of Group B was 924 days (range, 5 days–15 years).

In our study, we had a higher rate of redo procedures in the laparoscopic group (6/6 in Group A versus 4/8 in Group B), but finally mild obstructive adhesions were found in 4 of them, whereas in 2 cases, any evident occlusive causes were found. This result suggests that we probably performed two “unnecessary” redo procedures in Group A because of our initial insecurity on the laparoscopic approach. Most of the redo surgeries were precocious in our experience (4, 5, 6, 11, and 60 days after the first intervention). We expect the rate of such cases will reduce in the future, thanks to the major experience in laparoscopy. In Group B readmitted patients, we found both precocious occlusive symptoms (3, 5, 7, and 10 days after the first intervention) and long-term complications (5 and 15 years after). In all the patients subjected to a redo procedure (50%), the surgical findings were mild obstructive adhesions as in the laparoscopically treated group.

Many authors do not recommend the laparoscopic approach in case of volvulus because of the fragility of the ischemic bowel, which in some cases can easily disintegrate with handling. In our experience, we did not have any bowel perforation during the laparoscopic procedure, but we agree that this should be performed when expertise and proper equipment are available, and overall it is necessary to convert to an open procedure when there is any doubt that an adequate result can be reached laparoscopically or when a resection of necrotic midgut is requested.

As stated in previous studies, we surely agree that outcome may depend on the ability to completely identify, assess, and correct the intestinal and mesenteric anatomy. ¹⁵ Our results show that laparoscopy is feasible and safe for correction of malrotation with volvulus even in the neonatal population without additional risks compared with classical open Ladd's procedure, as long as the surgeon may ensure a total release of the bands.