Congenital Diaphragmatic Hernia with Associated Pulmonary Sequestration
A
full term infant born after an uneventful pregnancy and delivery presented with tachypnea. At birth, he was noted to have respiratory distress and poor feeding. He was transferred to the neonatal intensive care unit and a chest radiograph was obtained, which demonstrated a possible left diaphragmatic anomaly concerning for congenital diaphragmatic hernia (CDH). An upper gastrointestinal series was performed, which clearly demonstrated a left CDH (Figure 1). Once clinically stable, the child was taken to the operating room where a thoracoscopy demonstrated a CDH and an extralobar sequestration on top of the left kidney (Figure 2; available at www.jpeds.com). After a diaphragmatic hernia repair with a Gore-Tex patch and a resection of the sequestration via laparotomy, the child recovered well and in follow-up is asymptomatic and free of complications.
CDH is a congenital malformation of the diaphragm resulting in respiratory distress and feeding difficulties in the newborn. Pulmonary sequestration is an associated anomaly in 15%-30% of patients with CDH.1-3 Pulmonary sequestration is a congenital malformation of lung tissues resulting in lung tissue that is not connected to the tracheobronchial tree or to the pulmonary arterial blood supply. There are two types of pulmonary sequestration, extralobar and intralobar sequestration. Extralobar sequestration is more commonly associated with other anomalies, including CDH, congenital pulmonary airway malformations, pulmonary hypoplasia, congenital lobar emphysema, bronchogenic cyst, congenital heart disease, vertebral anomalies, and intestinal duplications.3,4 Awareness of the association of pulmonary sequestration with CDH and recognition of the sequestration in a stable CDH patient will permit resection of an extralobar sequestration at the time of the CDH repair, preventing operation at a later date for a “mediastinal mass.” n Diana L. Diesen, MD Steve Megison, MD Division of Pediatric Surgery Department of Surgery University of Texas Southwestern Medical Center Dallas Children’s Medical Center Dallas, Texas
References 1. Grethel EJ, Farrell J, Ball RH, Keller RL, Goldstein RB, Lee H, et al. Does congenital diaphragmatic hernia associated with bronchopulmonary sequestration portend a better prognosis? Fetal Diagn Ther 2008;23: 250-3. 2. Harris K. Extralobar sequestration with congenital diaphragmatic hernia: a complicated case study. Neonatal Netw 2004;23:7-24. 3. Corbett HJ, Humphrey GM. Pulmonary sequestration. Paediatr Respir Rev 2004;5:59-68. 4. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol 1986;3: 106-21.
Figure 1. An upper gastrointestinal series was performed, which demonstrated a left CDH (arrow).
J Pediatr 2014;165:1268. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.08.032
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Figure 2. The CDH and an extralobar sequestration (arrow) on top of the left kidney are visible in this thoracoscopic view from the left chest. The edges of the diaphragmatic defect are visible on the perimeter of the image (asterisk).
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A
full term infant born after an uneventful pregnancy and delivery presented with tachypnea. At birth, he was noted to have respiratory distress and poor feeding. He was transferred to the neonatal intensive care unit and a chest radiograph was obtained, which demonstrated a possible left diaphragmatic anomaly concerning for congenital diaphragmatic hernia (CDH). An upper gastrointestinal series was performed, which clearly demonstrated a left CDH (Figure 1). Once clinically stable, the child was taken to the operating room where a thoracoscopy demonstrated a CDH and an extralobar sequestration on top of the left kidney (Figure 2; available at www.jpeds.com). After a diaphragmatic hernia repair with a Gore-Tex patch and a resection of the sequestration via laparotomy, the child recovered well and in follow-up is asymptomatic and free of complications.
CDH is a congenital malformation of the diaphragm resulting in respiratory distress and feeding difficulties in the newborn. Pulmonary sequestration is an associated anomaly in 15%-30% of patients with CDH.1-3 Pulmonary sequestration is a congenital malformation of lung tissues resulting in lung tissue that is not connected to the tracheobronchial tree or to the pulmonary arterial blood supply. There are two types of pulmonary sequestration, extralobar and intralobar sequestration. Extralobar sequestration is more commonly associated with other anomalies, including CDH, congenital pulmonary airway malformations, pulmonary hypoplasia, congenital lobar emphysema, bronchogenic cyst, congenital heart disease, vertebral anomalies, and intestinal duplications.3,4 Awareness of the association of pulmonary sequestration with CDH and recognition of the sequestration in a stable CDH patient will permit resection of an extralobar sequestration at the time of the CDH repair, preventing operation at a later date for a “mediastinal mass.” n Diana L. Diesen, MD Steve Megison, MD Division of Pediatric Surgery Department of Surgery University of Texas Southwestern Medical Center Dallas Children’s Medical Center Dallas, Texas
References 1. Grethel EJ, Farrell J, Ball RH, Keller RL, Goldstein RB, Lee H, et al. Does congenital diaphragmatic hernia associated with bronchopulmonary sequestration portend a better prognosis? Fetal Diagn Ther 2008;23: 250-3. 2. Harris K. Extralobar sequestration with congenital diaphragmatic hernia: a complicated case study. Neonatal Netw 2004;23:7-24. 3. Corbett HJ, Humphrey GM. Pulmonary sequestration. Paediatr Respir Rev 2004;5:59-68. 4. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol 1986;3: 106-21.
Figure 1. An upper gastrointestinal series was performed, which demonstrated a left CDH (arrow).
J Pediatr 2014;165:1268. 0022-3476/$ - see front matter. Copyright ª 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.08.032
1268
Vol. 165, No. 6 December 2014
Figure 2. The CDH and an extralobar sequestration (arrow) on top of the left kidney are visible in this thoracoscopic view from the left chest. The edges of the diaphragmatic defect are visible on the perimeter of the image (asterisk).
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