Hindawi Publishing Corporation

Canadian Respiratory Journal
Volume 2016, Article ID 1460480, 4 pages

Case Report
Coexistent Congenital Diaphragmatic Hernia with
Extrapulmonary Sequestration

Nao Kawamura and Samarjeet Bhandal
Alberta Children’s Hospital, Calgary, AB, Canada T3B 6A8

Correspondence should be addressed to Samarjeet Bhandal; bhandal.samarjeet@ahs.ca

Received 23 June 2015; Accepted 13 March 2016

Academic Editor: Franz Stanzel

Copyright © 2016 N. Kawamura and S. Bhandal. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Bronchopulmonary foregut malformations are a heterogeneous but interrelated group of abnormalities that may contain more
than one histologic feature. It is helpful to be familiar with the presentation and imaging features of bronchopulmonary foregut
malformations presenting as a congenital mass or mass-like lesion, as imaging plays a central role in the evaluation of these lesions
since, when symptomatic, clinical features are usually nonspecific. With imaging, the presence of other associated lesions can be
determined, facilitating appropriate management to prevent the potential complications. We report a case of coexisting extralobar
pulmonary sequestration and ipsilateral diaphragmatic hernia in a term neonate.

1. Introduction and 4-day gestation as a uniformly hyperechoic left-sided
chest mass, displacing mediastinal structures to the right.
Bronchopulmonary foregut malformations (BPFMs), also
known as congenital lung malformations, are a heteroge- Prenatal-MRI examination at 28-week and 2-day ges-
neous group of disorders involving conducting airways, tation (Figures 1(a) and 1(b)) confirmed a feeding vessel
lung parenchyma, and pulmonary vasculature. These include arising from the aorta into the centre of the pulmonary
bronchogenic cyst, pulmonary sequestration, congenital pul- mass in keeping with systemic arterial supply. The MRI
monary airway malformation, congenital lobar overinflation, examination also diagnosed a coexisting left-sided congenital
bronchial atresia, and pulmonary cyst. Combinations of these diaphragmatic hernia with stomach, multiple bowel loops,
malformations are called hybrid malformations [1]. spleen, and partial liver herniation into the thoracic cavity.
Congenital diaphragmatic hernias (CDH) are serious The prenatal-MRI also showed markedly decreased pre-
and life threatening anatomic lesions that are frequently dicted total fetal lung volumes. The infant was born at 39-
associated with additional anatomic malformations [2]. week gestation by spontaneous vaginal delivery and was
Lesions may be detected by prenatal ultrasound or by fetal electively intubated at one minute of life. Initial chest X-ray
magnetic resonance imaging. The early diagnosis of the lesion (Figure 2) demonstrates herniation of bowel loops into the
with imaging facilitates appropriate management to prevent left chest with mediastinal shift to the right.
potential complications. At eight days of life, a contrast-enhanced CT examination
(Figures 3 and 4) was performed which confirmed sys-
temic arterial blood supply to the pulmonary mass. Venous
2. Case Report drainage from the mass was to the left atrium. Left-sided
We present a case of a term female neonate born with congenital diaphragmatic hernia was present with herniation
coexisting left-sided congenital diaphragmatic hernia and of small bowel loops and spleen into the thoracic cavity with
bronchopulmonary foregut malformation. marked mediastinal shift to the right.
The pulmonary abnormality was first detected on routine The congenital diaphragmatic hernia was repaired at 11
prenatal fetal anatomic sonographic evaluation at 18-week days of life. Intraoperative findings were a left diaphragmatic

and spleen. Once the hernia loops and the superior mesenteric artery are seen in the left was reduced. The sequestrated lung is supplied by an anomalous artery She was on oxygen supplementation and medication for arising from the aorta and its venous drainage is via the approximately six months for treatment of the pulmonary azygous system. lung. or the IVC [3]. monary sequestration in a left paraspinal location. being extralobar and was left in situ along the posterior which is located in the left lower paraspinal region. mediastinum. Figure 2: Chest radiograph showing left diaphragmatic hernia and contralateral shift of the heart and mediastinum. Figure 3: Coronal postcontrast CT image showing bronchopul- hernia with hernia contents of the left lobe of the liver. small bowel. A branch from coeliac artery is seen supplying spleen. Venous drainage is usually via pulmonary veins. colon. (b) T2-weighted: coronal MRI imaging at 28-week gestation showing left pulmonary bronchopulmonary foregut malformation and herniated bowel in the left hemithorax and displacement of the cardiac structures to the right side. An intralobar 3. hypertension which has since resolved. bar and extralobar types. . in which the former accounts for 75% of all pulmonary sequestrations [4]. The patient is now Sequestrations classically have been divided into intralo- over the age of two and is clinically doing well. Our case is atypical as an extralobar sequestration with that has no normal connection with the pulmonary arteries. the pulmonary sequestration was identified as hemithorax. Her postoperative course was significant for pulmonary hypertension as a sequela to her diaphragmatic hernia. the pulmonary veins.2 Canadian Respiratory Journal (a) (b) Figure 1: (a) T1-weighted: coronal MRI imaging at 28-week gestation showing left pulmonary bronchopulmonary foregut malformation and herniated bowel in the left hemithorax and displacement of the cardiac structures to the right side. Sequestered lung is is classically described as having systemic venous drainage defined as “a congenital mass of aberrant pulmonary tissue [5].” venous drainage to the left atrium. and is intimately connected to adjacent BPFMs refer to a subset of pulmonary developmental anoma. has no separate pleural covering. Herniated bowel stomach. Discussion sequestration is contained within the lung. An lies that result from abnormal budding of the embryonic extralobar sequestration has its own pleural covering and foregut and trachea-bronchial tree.

The authors declare that they have no competing interests. especially in the left fied the case and wrote the paper. that is. evaluation of possible with additional malformations. The antenatal MRI is especially useful for the prenatal diagnosis of and postnatal imaging features of these abnor- thoracic lesions that are atypical or complicated by multiple malities guide prenatal counselling and appro- abnormalities and for assessing lung volumes. . the mass effect from a left lower lobe pulmonary sequestra. (i) Imaging is important for characterization of the the remainder have complex CDH. ment for diaphragmatic defects. and is intimately separate anomalies in one lesion suggests that they are ends connected to adjacent lung. The coexistence of a CDH may be masked by the presence of pulmonary sequestration. An intralobar sequestrations and CPAM with congenital diaphragmatic sequestration is contained within the lung. (2) To be aware that congenital pulmonary abnormalities can be associated with congenital diaphragmatic her- nia. Venous drainage of a spectrum. delaying the herniation of abdominal contents until after delivery and allowing prenatal lung development [6]. and it has been is usually via pulmonary veins. Figure 4: Coronal CT image showing feeding artery to sequestrated Pretest lung segment arising from the descending aorta and draining vein (1) What are the typical distinguishing features of intra- from the sequestration entering into left atrium. Therefore. CanMeds competency: Medical Expert. evaluation of possible associated congenital anomalies. In some cases. Learning Objectives (1) To recognize the salient features of pulmonary sequestrations. Congenital diaphragmatic hernia is characterized by incomplete formation/muscularization of the diaphragm (2) Why is it important to perform tertiary level diagnos- resulting in absence or deficiency of the diaphragm. usually has systemic venous drainage. About 50%–60% of affected individuals have isolated CDH. The advantage of fetal MRI is that it offers operator-independent imaging in a number of different Competing Interests planes with excellent soft tissue contrast and a large field of view. management of pregnancy and delivery.and postnatal management. Fetal MRI can priate peri. In many cases fetal MRI affords better patient counselling. (1) What are the typical distinguishing features of intra- tion is speculated to cause a protective effect in concomitant pulmonary versus extrapulmonary sequestrations? CDH. identification of aberrant arterial and venous connec- tions. CDH occurring pulmonary abnormality. provide useful information on developmental abnormalities of the lung and thorax. rather than separate entities. Samarjeet Bhandal identi- with prenatally diagnosed lung masses. The coexistence of no separate pleural covering. No radiation or contrast agents are required [11]. An extralobar suggested that it is more logical to describe all congenital sequestration has its own pleural covering and cystic lung lesions as “congenital thoracic malformations” [8]. pulmonary versus extrapulmonary sequestrations? (2) Why is it important to perform tertiary level diagnos- tic imaging on prenatally diagnosed pulmonary chest The imaging of suspected pulmonary sequestration is masses? directed to identification of sequestered or dysplastic lung tissue. and neonatal care Authors’ Contributions planning. It occurs tic imaging on prenatally diagnosed pulmonary chest in one in 1000–2000 live births. should be delivered at a tertiary centre and parents should be counselled about the possibility of delayed presentation of an occult CDH [6].Canadian Respiratory Journal 3 lower lung. has hernias and bronchogenic cysts [7]. and assess- CDH are detected prenatally by ultrasound examination [10]. infants Nao Kawamura wrote the paper. Frequent complications masses? are pulmonary hypoplasia and pulmonary hypertension [9]. More than 50% of cases with associated congenital anomalies. Posttest and assessment for diaphragmatic defects [5]. (i) Both pulmonary sequestrations have aberrant Other studies have reported associations of pulmonary aortic or systemic blood supply.

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