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Impact of Bowel Rotation and Fixation On Obstructive Complications in CDH
Impact of Bowel Rotation and Fixation On Obstructive Complications in CDH
a r t i c l e i n f o a b s t r a c t
Article history: Aim of the study: Small bowel obstruction (SBO) is a known complication after congenital diaphragmatic
Received 5 October 2022 hernia (CDH) repair, which can require surgery and even extensive bowel resection causing short bowel
Accepted 11 October 2022
syndrome (SBS). We investigate whether specific bowel rotation and fixation can be used as a predictor
for SBO including volvulus.
Keywords: Methods: A retrospective review of 256 CDH survivors following repair from 2003 to 2020 was per-
Malrotation formed. Operative notes and upper gastrointestinal series (UGI) were screened to determine the rotation
Nonfixation and fixation of the bowel. Primary outcomes included SBO occurrence, SBO treated surgically, and volvu-
Small bowel obstruction lus. For statistical analysis Fisher’s exact test was utilized.
Volvulus
Results: Twenty-two (9%) patients presented with SBO and majority, 19 (86%), required surgery. Adhesion
were observed in 10 (45%), recurrence in 5 (23%), and extensive volvulus leading to SBS in 3 (14%). Both
rotation and fixation were recorded in 117 (46%). Presence of left CDH with malrotation and nonfixation
was a significant predictor for SBO requiring surgery (P<0.05 vs all other groups). All 3 patients with
extensive volvulus had left CDH with nonfixed bowel (100%), however only 1 had malrotation (33%).
Conclusions: Malrotation and nonfixation are associated with increased SBO in CDH. Normal rotation is
not protective and patients are still at risk for volvulus resulting in SBS. SBO requiring surgical interven-
tion is common in CDH. Bowel rotation and fixation are important determinants that, should be routinely
documented and education about the risk of SBO should be included in family counseling.
Level of Evidence: Level IV – Case Series
© 2022 Elsevier Inc. All rights reserved.
https://doi.org/10.1016/j.jpedsurg.2022.10.019
0022-3468/© 2022 Elsevier Inc. All rights reserved.
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210 K. Moskowitzova, J.M. Zalieckas, C.A. Sheils et al. / Journal of Pediatric Surgery 58 (2023) 209–212
upper gastrointestinal series (UGI) showing an abnormal anatomic number, median with range or in percentage, when appropriate.
location of duodenal-jejunal junction (DJJ) [13]. However, CDH pa- Fisher’s exact test was utilized for statistical analysis comparing
tients may have normal rotation, but have abnormal fixation of the complication rates and significance was defined as P<0.05.
bowel because of herniation into the chest, which may predispose
them to obstructive complications. As both bowel rotation and fix- 3. Results
ation in CDH patients are not routinely documented, there is lim-
ited knowledge about the incidence of rotational abnormalities and 3.1. Cohort characteristics
the potential impact on later obstructive complications.
The purpose of this study was to precisely document both the Two hundred fifty-six eligible CDH survivors were identified at
rotation and fixation status of the bowel in CDH survivors at our our institution between 2003 and 2020 (Table 1). The majority
institution, and to investigate if these are predictive of obstructive were males (156, 61%). Median age at repair was 3 days (range 0–
complications, including volvulus. 14.8 years; interquartile range (IQR) 2–5.25 days); 26 had their re-
pair outside of the neonatal period. Median age at follow up was 5
2. Methods years (range 0.1–18 years). CDH was mostly located on the left side
(L CDH, N = 205, 80%). The defect size was type A in 60 (23%), type
2.1. Study cohort B in 76 (30%), type C in 83 (32%) and type D in 12 (5%). Twenty-
five patients (10%) had an undetermined defect. Open repair was
A single center retrospective cohort study was performed in all the most prevalent type of approach in 182 patients (71%). A min-
surviving patients following CDH repair at Boston Children’s Hospi- imally invasive approach, either thoracoscopic or laparoscopic, was
tal between the years 2003 and 2020 via review of electronic med- utilized in 74 patients (29%). Patch repair was performed in 146
ical records. CDH patients are enrolled in the Congenital Diaphrag- patients (57%).
matic Hernia Program at discharge, a multidisciplinary clinic es- The bowel rotation and fixation status were both documented
tablished in 1991, which provides long term multidisciplinary fol- in 117 patients (46%). For 98 patients (38%) only one bowel status
low up care to hundreds of CDH survivors. Our institution has also was evaluable, rotational status in 8 patients, and fixation alone
been continuously participating in the CDH Study Group (CDHSG) in 90 (3% and 35% respectively). No information about bowel sta-
registry since its inception, contributing data outcomes collected tus was discernable in 41 patients (16%). No rotational status was
from the prenatal period through the index hospitalization dis-
charge. Table 1
Patients who did not follow up after discharge, and/or patients Overall cohort characteristics (N = 256).
who had their initial operative repair performed at an outside in-
Variable Data point
stitution were excluded. Only patients with a Bochdalek CDH were
included. Patients with a Morgagni CDH and eventration were not Sex
Male 156 (61%)
included in the analysis.
Female 100 (39%)
The operative notes and UGI studies in our study cohort of Age
CDH patients were reviewed to determine the rotation and fixation Median age at repair 3 (2–5.25; IQR∗ ) d
of the bowel as rotational status is not collected by the CDHSG. Median age at obstruction 1 (0.1–10) y
Records were further screened for the occurrence of SBO as the Median age at follow up 5 (0.1–18) y
Defect side
primary outcome, either treated conservatively or surgically. Fur- L CDH 205 (80%)
thermore, the etiology of bowel obstruction requiring surgical in- R CDH 51 (20%)
tervention was categorized as either secondary to adhesions, CDH Defect type
recurrence, or volvulus (both segmental and midgut). Additionally, Type A 60 (23%)
Type B 76 (30%)
data points including sex, age at repair, defect side, size and type
Type C 83 (32%)
according to the CDHSG classification (type A-D) [14], herniated or- Type D 12 (5%)
gans, type of operative approach, a Ladd procedure at the index Not defined 25 (10%)
operation, age at obstructive complication, and age at the most re- Surgical approach
cent follow up were collected. Open 182 (71%)
Minimally invasive 74 (29%)
Thoracoscopic 68 (26.6%)
2.2. Evaluation of fixation and rotation Laparoscopic 5 (2%)
Combined thoraco- and laparoscopic 1 (0.4%)
We utilized the standard definition of malrotation as an abnor- Repair type
Primary 110 (43%)
mal position of ligament of Treitz upon direct visualization during
Patch 146 (57%)
the initial CDH repair as reported in the operative note or on later Determination of bowel status
UGI. The normal anatomical and radiologic position of the DJJ is to Both rotation and fixation known 117 (46%)
the left of the left vertical body pedicle at the level of the inferior Only 1 bowel status known
margin of the duodenal bulb and located posteriorly on the lateral Only fixation 90 (35%)
Only rotation 8 (3%)
view confirming its retroperitoneal positioning [15]. We defined Unknown bowel status 41 (16%)
nonfixation as lack of lateral colonic attachments observed during Determination of rotation
the initial surgery. CDH survivors were then accordingly categorize Known rotation 125 (49%)
to 6 groups based on bowel status and laterality of diaphragmatic Malrotation 54 (43%)
Normal rotation 71 (57%)
defect as follows: left or right CDH with no malrotation/fixed, no
Unknown rotation 131 (51%)
malrotation/nonfixed, and malrotation/nonfixed. Determination of fixation
Known fixation 207 (81%)
2.3. Statistical analysis Nonfixation 202 (98%)
Normal fixation 5 (2%)
Unknown fixation 49 (19%)
Data were analyzed to determine the impact of bowel rotation
∗
and fixation on obstructive complications and presented as a total IQR (interquartile range).
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K. Moskowitzova, J.M. Zalieckas, C.A. Sheils et al. / Journal of Pediatric Surgery 58 (2023) 209–212 211
Table 3
Obstructive complications in patients with fully determined bowel status (N = 117).
Bowel Rotation and Fixation Small Bowel Small Bowel Obstruction Requiring Surgical Intervention
Obstruction
Overall Adhesions Recurrence Volvulus w/o SBS Volvulus w/ SBS
Left CDH no malrotation / fixed 0/1 (0%) 0/1 (0%) 0/1 (0%) 0/1 (0%) 0/1 (0%) 0/1 (0%)
Left CDH no malrotation / 5/53 (9.4%) 4/53 (7.6%) 2/53 (4%) 0/53 (0%) 0/53 (0%) 2/53 (4%)
nonfixed
Left CDH malrotation / nonfixed 10/39 (25.6%)∗ 9/39 (23.1%)∗∗ 6/39 (15.4%) 1/39 (2.6%) 1/39 (2.6%) 1/39 (2.6%)
Right CDH no malrotation / fixed 1/2 (50%) 1/2 (50%) 1/2 (50%) 0/2 (0%) 0/2 (0%) 0/2 (0%)
Right CDH no malrotation / 0/7 (0%) 0/7 (0%) 0/7 (0%) 0/7 (0%) 0/7 (0%) 0/7 (0%)
nonfixed
Right CDH malrotation / nonfixed 1/15 (6.7%) 0/15 (0%) 0/15 (0%) 0/15 (0%) 0/15 (0%) 0/15 (0%)
∗
P = 0.025 vs. all other groups.
∗∗
P = 0.014 vs. all other groups.
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212 K. Moskowitzova, J.M. Zalieckas, C.A. Sheils et al. / Journal of Pediatric Surgery 58 (2023) 209–212
Table 4
Summary of 3 patients with volvulus leading to short bowel syndrome.
Case CDH Side Type of Repair Age at Fixation Rotation Age at Volvulus Short Bowel
Repair Presentation Syndrome
1 Left open, primary DOL 2 nonfixation Malrotation identified 6 years old midgut volvulus 41 cm of residual
identified in OR on routine UGI for identified on CT small bowel
GERD
2 Left open, patch DOL 0 nonfixation no malrotation 7 weeks old segmental volvulus 95 cm of residual
identified in OR identified in OR for identified on UGI small bowel
volvulus
3 Left open, patch DOL 5 nonfixation no malrotation 4 years old segmental volvulus 130 cm of residual
identified in OR identified on UGI identified in OR small bowel
during hospitalization
for volvulus
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