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Management and outcomes of scoliosis in children with congenital diaphrag-
matic hernia

Ryan M. Antiel, John S. Riley, Patrick J. Cahill, Robert M. Campbell,


Lindsay Waqar, Lisa M. Herkert, Natalie E. Rintoul, William H. Peranteau,
Alan W. Flake, N. Scott Adzick, Holly L. Hedrick

PII: S0022-3468(16)30336-0
DOI: doi: 10.1016/j.jpedsurg.2016.09.013
Reference: YJPSU 57782

To appear in: Journal of Pediatric Surgery

Received date: 6 August 2016


Accepted date: 12 September 2016

Please cite this article as: Antiel Ryan M., Riley John S., Cahill Patrick J., Campbell
Robert M., Waqar Lindsay, Herkert Lisa M., Rintoul Natalie E., Peranteau William H.,
Flake Alan W., Adzick N. Scott, Hedrick Holly L., Management and outcomes of scoliosis
in children with congenital diaphragmatic hernia, Journal of Pediatric Surgery (2016), doi:
10.1016/j.jpedsurg.2016.09.013

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MANAGEMENT AND OUTCOMES OF SCOLIOSIS IN CHILDREN WITH

CONGENITAL DIAPHRAGMATIC HERNIA.

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Ryan M. Antiel, John S. Riley, Patrick J. Cahill, Robert M. Campbell, Lindsay Waqar,

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Lisa M. Herkert, Natalie E. Rintoul William H. Peranteau, Alan W. Flake, N. Scott

Adzick, Holly L. Hedrick

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The Center for Fetal Diagnosis and Treatment, Center for Thoracic Insufficiency
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Syndrome, and the Division of Orthopedics, The Children’s Hospital of Philadelphia,

Philadelphia, PA, USA


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Corresponding Author: Holly L. Hedrick, MD


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The Center for Fetal Diagnosis and Treatment


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The Children’s Hospital of Philadelphia

34th Street and Civic Center Boulevard

Philadelphia, PA 19104-4318

Tel.: +1 215 590 2733; fax +1 215 590 2447

E-mail address: hedrick@email.chop.edu (H.L. Hedrick)

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Abstract

Purpose: The purpose of this study was to evaluate the management and outcomes of

CDH patients with scoliosis.

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Methods: From January 1996 to August 2015, 26 of 380 (7%) CDH patients were

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diagnosed with scoliosis. Six (23%) were prenatally diagnosed by ultrasound, and 9

(35%) were diagnosed postnatally. The remaining 11 (42%) developed scoliosis after

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discharge. Mean follow-up was 6.6 years.

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Results: Among the 15 patients with congenital scoliosis, there were 2 (13%) perinatal

deaths. Five of the 13 (38%) survivors required orthopedic surgery, and 2 have required
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bracing. The mean age at initial surgery was 7 years. These five children underwent an

average of 2.8 (range 1-7) expansions or revisions. All surgical patients required
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supplemental oxygen at 28 days of life, and 1 required a tracheostomy. None of the 11


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patients who developed scoliosis later in life required surgery, but 3 have required

bracing. Six of the 11 (55%) required a patch repair for CDH compared to 158 of 264
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(60%) CDH patients without scoliosis (p=0.73).


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Conclusions: Early diagnosis of scoliosis in CDH patients is associated with a high rate

of surgery. There was not a higher incidence of patch repair among patients who

developed scoliosis.

Keywords: Congenital diaphragmatic hernia; scoliosis

Prognosis.Retrospective Study, Level II

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Introduction

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that occurs in

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approximately 1 in every 3000 live births. [1] The incomplete formation of the diaphragm

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musculature during development allows the abdominal viscera to migrate into the

thoracic cavity. [2] Compression of the developing fetal lungs causes pulmonary

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hypoplasia and pulmonary hypertension, which results in a high mortality rate, estimated

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between 20-60%. [3-4] Small diaphragmatic defects can be repaired by primary closure

while larger defects require repair with a synthetic patch or autologous muscle flap.
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Survivors with CDH can have multiple comorbidities including respiratory insufficiency,

reflux, failure to thrive, hernia recurrence, and musculoskeletal deformities.[5]


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One of the more common musculoskeletal deformities associated with CDH is


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scoliosis. Defined as a lateral curvature of the vertebral column of greater than 10

degrees, scoliosis can occur in CDH patients before birth (congenital scoliosis) or
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develop later in childhood. The purpose of this study was two-fold: first, to describe the
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prevalence of scoliosis in a large cohort of CDH patients; and, second, to evaluate the

management and clinical and radiological outcomes.

Methods

This study was approved by The Children’s Hospital of Philadelphia Institutional

Review Board.

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Study population

We reviewed 380 patients with CDH who are part of the Pulmonary Hypoplasia

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Program Database at the Children’s Hospital of Philadelphia from January 1996 through

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August 2015. Many of these patients underwent prenatal evaluation at our Center for

Fetal Diagnosis and Treatment, which included a fetal ultrasound and magnetic resonance

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imaging. All CDH patients with a diagnosis of scoliosis from the Pulmonary Hypoplasia

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Program database or Orthopedic scoliosis database were included in this study. Patients

were categorized by the timing of scoliosis diagnosis: prenatal, postnatal diagnosis during
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the neonatal hospitalization, or sometime after hospital discharge. Prenatal records were

reviewed to determine demographics, CDH side (right vs. left), liver position (above or
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below the diaphragm), and presence of other genetic abnormalities. Postnatal records
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were reviewed for survival, gestational age at birth, birth weight, timing of CDH repair,

need for prosthetic patch, and postnatal pulmonary outcomes as available.


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Orthopedic evaluation

We reviewed orthopedic records to determine need for bracing, need and timing

of orthopedic surgery, type of repair (growing rod, spinal fusion, or vertical expandable

prosthetic titanium ribs), and the number of surgical expansions or revisions.

We reviewed available radiology reports from prenatal high-resolution ultrasound

to determine which patients had prenatal diagnosis of scoliosis. Optimal imaging of the

spine required positioning the fetal spine in the near field and away from the uterine wall.

[6] Coronal views of the spine were best for identifying the location of curvature. The

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direction was indicated by stating the side to which the convex portion of the spine

pointed. The approximate level of the center of the curvature was noted. The Cobb angle

was measured from coronal images using the same principles used when measuring from

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a radiograph, however the severity was usually described qualitatively.

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Preoperative and postoperative upright anteroposterior spine and chest

radiographs were reviewed for all patients who underwent orthopedic surgery, as was the

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most recently obtained x-ray for all patients, in order to determine the Cobb angle and to

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assess space available for lung (SAL). X-rays were performed in standing (children and

adolescents), sitting (toddlers), or supine (infants) position as dictated by patient age.


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Cobb angles were determined by taking the most tilted vertebrae above and below the

apex of the curve of the spine.[7-8] A line was drawn along the superior end plate of the
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top vertebra and a second line was drawn along the inferior end plate of the bottom
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vertebra. The angle between these two intersecting lines was designated as the Cobb

angle. The SAL was calculated as a ratio, expressed as a percentage, by dividing the
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height of the concave hemithorax by the height of the convex hemithorax.[9] The height
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of the hemithorax was measured by taking the distance from the middle of the most

cephalad rib down to the center of the hemidiaphragm. Cobb angles were measured and

recorded by the orthopedic investigators (P.J.C. and R.M.C.) and the SAL was calculated

by a trained member of our research team (J.S.R.).

Statistical analysis

Descriptive statistics for categorical variables were reported as frequency and

percentage, and continuous variables were reported as mean and standard deviation or

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median and interquartile range. Categorical variables were compared between CDH

patients with and without scoliosis using the Chi square test. Pre and post-operative

continuous variables were compared using a paired Student’s t-test. All statistical tests

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were two-sided with the α level set at 0.05 for statistical significance. Statistical analysis

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was performed using Stata statistical software, version 13.1 (StataCorp, College Station,

TX).

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Results

Patient population
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From January 1996 to August 2015, a total of 26 of 380 (7%) CDH patients were

diagnosed with scoliosis. Fifteen patients (58%) had congenital scoliosis. Six (40%)
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were diagnosed prenatally by ultrasound and 9 (60%) were diagnosed postnatally during
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their neonatal hospitalization. Among these 15 patients with congenital scoliosis, 3

(20%) had abnormal karyotypes and there were 2 (13%) perinatal deaths of neonates with
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multiple congenital anomalies.


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Of 274 survivors at NICU discharge without congenital scoliosis, an additional 11

(4%) patients were diagnosed with scoliosis later in life, sometime after their neonatal

hospital discharge. There have been no deaths among the patients who developed

scoliosis later in life. The incidence of scoliosis among survivors at NICU discharge was

24/274 (9%). Patient characteristics and neonatal hospital course are summarized in

Table 1. Mean follow-up for this cohort was 6.6 years.

The mean gestational age at delivery was 36.6 weeks (SD 2.9 weeks). Of the 24

surviving patients, 7 (29%) also had multiple segmentation vertebral anomalies

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documented. Of the survivors, 12 of 22 (55%) patients had a documented curve convex

towards the diaphragmatic defect, 2 of 22 (14%) had an s-shaped curve, and 7 of 22

(32%) a curve convex away from the diaphragmatic defect. The average timing of hernia

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repair was 11 days (SD 12 days). Thirteen of the 24 patients (54%) required a prosthetic

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patch for the repair of their diaphragm. Six of the 11 (55%) who developed scoliosis

later in life had required a patch repair for CDH compared to 158 of 264 (60%) of all

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CDH survivors at NICU discharge without scoliosis during the study period (p=0.73).

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Musculoskeletal and pulmonary outcomes
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Five of the 13(38%) survivors with congenital scoliosis required orthopedic surgery and 2

have required bracing. There were 3 VEPTR placements, 1 growing rod placement, and
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1 spinal fusion. The mean age at initial surgery was 7 years. These five children
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underwent an average of 2.8 (range 1-7) expansions or revisions. The mean pre-

operative Cobb angle was 50° (range 21-69) reducing to a post-operative mean of 31°
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(range 11-47, p=0.01) and then increasing to a mean of 47° (range 11-65, p=0.55) at time
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of maximal follow-up. The average time between preoperative imaging and most recent

imaging was 30.8 months. The mean pre-operative space available for lung was 90%

(range 81-96) increasing to a post-operative mean of 95% (range 85-99) and a mean of

95% (range 92-99) at maximal follow-up (p=0.24). These results are consistent with

other reported results of early onset scoliosis. [10] The scoliosis initially improves

following surgery yet worsens over time until the patient requires an expansion or

revision. All surgical patients required supplemental oxygen at 30 days of life for

underlying pulmonary hypoplasia and 1 has required a tracheostomy.

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None of the 11 patients who developed scoliosis later in life required orthopedic

surgery to date, however 3 have required bracing. The mean Cobb angle of brace treated

patients was 32° (range 7-58) compared to a mean of 12° (range 0-34) for patients being

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observed. The mean space available for lung was 88% (range 83-98) and 92% (range 83-

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100), respectively.

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Discussion

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Although advances in surgical and neonatal care have led to significantly

improved overall survival rates for neonates with CDH, long-term survivors face
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numerous morbidities including musculoskeletal deformities.[5] Patients with CDH

suffer from underlying pulmonary hypoplasia and pulmonary hypertension. Scoliosis can
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further compromise pulmonary growth and function leading to thoracic insufficiency


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syndrome.[9, 11-12] In this retrospective review of 380 patients with CDH over twenty

years, the incidence of scoliosis was 7% (9% among survivors at NICU discharge). This
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percentage represents an underestimation of the true prevalence, given variance in


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follow-up times and later deaths. Of the CDH patients diagnosed with scoliosis prior to

NICU discharge, just over half (58%) were diagnosed with congenital scoliosis and a

third of these patients (40%) were diagnosed on prenatal ultrasound. A prenatal

diagnosis of scoliosis is associated with a relatively high rate of orthopedic surgery.

Thirty-eight percent of the CDH patients diagnosed with congenital scoliosis required

orthopedic surgery, whereas none of the patients in our cohort who have developed

scoliosis later in life have required surgery to date.

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The prevalence of idiopathic scoliosis, defined as a curvature of the spine greater

than 10 degrees in the coronal plane, is approximately 3%.[13-14] Infants who have

undergone thoracic surgery for congenital heart defects, esophageal atresia, and rib or

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chest wall resection have a higher than expected incidence of scoliosis in

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adolescence.[15-18] Patients with CDH also have a significantly higher rate of

scoliosis.[19] Most recent series report rates ranging from 8% - 27%.[20-22] Ours is the

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first study that describes the rate of prenatal diagnosis as well as the orthopedic

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management and outcomes of all patients with CDH and scoliosis.

For patients with a curvature less than 20 degrees, careful monitoring with serial
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radiographic exams is sufficient. For patients with curvature between 20 and 40 degrees,

back bracing can be an effective nonsurgical treatment. For children with a curvature
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greater than 40 degrees and for those with thoracic insufficiency syndrome, surgical
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management is warranted. Of the surgical treatments for scoliosis, growing rods have

been the most common treatment for children who have not reached skeletal maturity,
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whereas spinal fusion is the most common surgical treatment for children who have
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reached skeletal maturity. More recently vertical expandable prosthetic titanium ribs

(VEPTR) has become the preferred treatment for patients who suffer from thoracic

insufficiency syndrome secondary to scoliosis.[23-25] In contrast to growing rods, in

which the devise is attached to the top and base of the spine, VEPTR is also attached to

the patient’s ribs. This not only straightens the spine but also separates the ribs resulting

in better lung growth and pulmonary function. VEPTR and growing rods require

adjustment surgeries every 6-8 months until the child reaches skeletal maturity.

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While the association between CDH and scoliosis is well established, the etiology

still remains unclear. Although the etiology is likely multifactorial, there are at least two

general ways to think about this association. First, congenital scoliosis is due to

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structural abnormalities of the spine and primarily associated with CDH given the close

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embryological relationship between the development of the lungs, diaphragm, and

thoracic spine. However, as indicated by our data, a significant minority of CDH patients

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will develop scoliosis later in life. Second, scoliosis as a secondary development is

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hypothesized to be associated with restricted growth of the hemithorax secondary to

ipsilateral pulmonary hypoplasia or from tension of the diaphragm on its spinal


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insertion.[22] While our previous work has demonstrated a significantly increased risk of

pectus malformations among patients who have required patch repair, the current data
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does not support an association between patch repair and scoliosis.[26] There was not a
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statistically significant difference in requirement for patch repair among patients who

developed scoliosis compared to those who did not develop scoliosis (55 vs. 60 %,
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p=0.73).
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Our study has important limitations. First, the incidence of scoliosis was

identified retrospectively from the Pulmonary Hypoplasia Program database and

Orthopedic scoliosis database. The true incidence of antenatal diagnoses of scoliosis

remains unknown. The published incidence of hemivertebra in the fetus is 0.33 in 1000

births. [27] However, only 25% of fetuses diagnosed with hemivertebra have associated

scoliosis/kyphosis. [28]. We are the first group, to our knowledge, to publish on the

antenatal diagnosis of scoliosis in CDH patients. It is possible that very mild scoliosis

was not identified on prenatal ultrasound or during the neonatal hospitalization. It is also

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probable that there were patients who developed mild scoliosis later in life that either

went undetected or who have been lost to follow-up. Therefore, our total incidence rate

of 7% is most likely an underestimation of the actual incidence of scoliosis among CDH

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patients. This study does not propose a specific mechanism for the development of

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scoliosis in CDH; rather we sought to report the incidence, management, and outcomes in

our practice. Nonetheless, the type of surgical repair did not affect the incidence of

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scoliosis. This finding challenges the hypothesis that the tension after repair leads to the

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development of scoliosis.[29]

In summary, we found a 7% incidence of scoliosis among all CDH patients (9%


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among survivors at NICU discharge) at our institution over a twenty-year period. We

recommend that all anatomic surveys for fetuses with CDH include a complete evaluation
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of the spine. Early diagnosis is associated with a relatively high rate of surgery and
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bracing, whereas late diagnosis appears to be associated with relatively mild scoliosis.

Among CDH patients who are diagnosed prenatally or in the neonatal hospitalization
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with congenital scoliosis, there was a 13% mortality rate and 38% of survivors with
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congenital scoliosis have required orthopedic surgery. In contrast, none of the patients

who have developed scoliosis later in life have required orthopedic surgery to date.

These data support careful musculoskeletal monitoring of CDH patients and early

evaluation by an orthopedic surgeon.

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29. Chiu PPL, Sauer C, Mihailovic A, et al. The price of success in the management
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of congenital diaphragmatic hernia: is improved survival accompanied by an

increase in long-term morbidity? J Pediatr Surg 2006; 41:888–92.


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Figure Legends:

Figure 1. Pre and post surgical chest radiographs.

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A. Pre-surgical chest radiograph with Cobb angel 69.1 and SAL 96%.

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B. Post-surgical chest radiograph with Cobb angel 55.1 and SAL 99%.

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Fig. 1A

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Fig. 1B

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Table 1 Characteristics of CDH patients with scoliosis


All patients Perinatal Diagnosis after P value
(n = 26) diagnosis discharge
(n = 15) (n = 11)
N (%)

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Demographics
Female sex 10 (38%) 5 (33%) 5 (45%) 0.53

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Race 0.90

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White 18 (69%) 10 (66%) 8 (73%)
Black 4 (15%) 3 (20%) 1 (9%)

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Asian 2 (8%) 1 (7%) 1 (9%)
Other 2 (8%) 1 (7%) 1 (9%)
Perinatal information

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Side 1.0
LCDH 18 (69%) 10 (66%) 8 (73%)
RCDH 7 (27%) 4 (27%) 3 (27%)
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Midline defect 1 (4%) 1 (7%) 0
Liver up 13 (50%) 6 (39%) 7 (64%) 0.43
Normal karyotype 20 (77%) 12 (80%) 8 (73%) 1.0
Survival 24 (92%) 13 (87%) 11 (100%) 0.49
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GA at birth 36.65 (2.9) 36.6 (3.0) 36.5 (3.1) 0.93


Mean (SD), weeks
Birth weight 2628.8 (813.2) 2623.3 (831.9) 2599.0 (880.6) 0.94
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Mean (SD), grams


CDH repair
Age at repair 11 (12) 12 (SD 12) 11 (13) 0.84
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Mean (SD), days


Need for prosthetic 13 (54%) 7 (54%) 6 (55%) 0.69
patch repair
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Orthopedic interventions
VEPTR 3 (13%) 3 (23%) 0 -
Growing rod 1 (4%) 1 (8%) 0 -
Spinal fusion 1 (4%) 1 (8%) 0 -
Bracing 4 (17%) 1 (8%) 3 (27%) 0.28
Age at surgery 7 (3.27) 7 (3.27) - -
Mean (SD), years
Number of - 2.8 (1-7) - -
operations (range)

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Table 2: Cobb angles and space available for lung (SAL) of 24 CDH patients with
scoliosis

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Pre-surgical X-ray Post-surgical X-ray Most Recent Follow-up X-ray

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Cobb angle SAL Cobb SAL Cobb SAL
mean (range) mean (range) angle mean angle mean

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mean (range) mean (range)
(range) (range)

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Surgical 50° (21-69) 90% (81-96) 31° (11- 95% 44° (11- 95%
treatment 47) (85-99) 65) (92-99)
(n = 5)
Bracing - - - - 32° (7- 88%

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(n = 5) 58) (83-98)
Observation - - - - 12° (0- 92%
alone@ 34) (83-
(n = 14) 100)
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