Pediatr Surg Int (2014) 30:961–963

DOI 10.1007/s00383-014-3572-0


A case of congenital diaphragmatic hernia
with intradiaphragmatic pulmonary sequestration:
case report and literature review
Soichi Shibuya • Yuki Ogasawara • Hiroshi Izumi • Masato Kantake •

Kaoru Obinata • Koyo Yoshida • Geoffrey J. Lane •
Atsuyuki Yamataka • Tadaharu Okazaki

Accepted: 15 July 2014 / Published online: 22 July 2014
Ó Springer-Verlag Berlin Heidelberg 2014

Abstract Extralobar pulmonary sequestration (EPS) can with the rest being intralobar [1]. EPS is commonly found
occasionally be found incidentally in congenital dia- in the thoracic cavity, also in the peritoneal cavity, but
phragmatic hernia (CDH). Extralobar pulmonary seques- rarely in the diaphragm [2]. Here we present an extremely
tration usually arises in the chest or the abdomen; rarely in rare case of congenital diaphragmatic hernia (CDH) asso-
the diaphragm. We report a neonatal case of antenatally ciated with intradiaphragmatic EPS.
diagnosed CDH associated with intradiaphragmatic EPS.

Keywords Congenital diaphragmatic hernia  Case report
Pulmonary sequestration  Extralobar  Intradiaphragmatic
A female with antenatally diagnosed CDH was delivered
vaginally at 39 weeks’ gestation weighing 3,009 g.
Introduction Although fetal ultrasound (US) and magnetic resonance
imaging (MRI) at 37 weeks’ gestation revealed left-sided
Pulmonary sequestration (PS) is a congenital malformation CDH, no other abnormalities were detected. Upon transfer
defined as immature, nonfunctional pulmonary tissue to our neonatal intensive care unit she was found to be in
without bronchial communication. Only some 25 % of PS no respiratory distress but had signs of moderate pulmon-
exist outside the lobes of the lungs as extralobar PS (EPS) ary hypertension. After clinical stabilization, a laparotomy
was performed on day 7 of life. A hernia sac containing
stomach and small intestine was found and its contents
S. Shibuya  Y. Ogasawara  T. Okazaki (&) reduced gently into the abdomen. During excision of the
Department of Pediatric Surgery, Juntendo University Urayasu
hernia sac, a cystic mass containing yellowish mucus was
Hospital, 2-1-1 Tomioka, Urayasu-shi, Chiba 279-0021, Japan
e-mail: found in the abnormally thickened posterior rim of the
diaphragm (Fig. 1). The mass and the sac were resected en
S. Shibuya  G. J. Lane  A. Yamataka block and the defect in the diaphragm was approximated by
Department of Pediatric General and Urogenital Surgery,
direct closure. Although major feeding vessels to the mass
Juntendo University School of Medicine, Tokyo, Japan
from the descending aorta did not exist, several vessels
H. Izumi were observed to enter the mass from the posterior rim of
Department of Pathology, Juntendo University Urayasu the diaphragm. Her postoperative recovery was uneventful,
Hospital, Chiba, Japan
being extubated on the 1st postoperative day and dis-
M. Kantake  K. Obinata charged home on the 13th postoperative day. On histopa-
Department of Pediatrics, Juntendo University Urayasu Hospital, thology, the mass comprised immature alveolar tissue and
Chiba, Japan bronchial component containing cartilages compatible with
EPS (Fig. 2), and a final diagnosis of CDH with intradia-
K. Yoshida
Department of Obstetrics, Juntendo University Urayasu phragmatic EPS was made. She is now 5 months old with
Hospital, Chiba, Japan no signs of recurrence.


diaphragm (hernia sac). accessory spleen. The divertic- ulum undergoes multiple divisions to form the tracheo- bronchial tree [3]. retroperitoneal. Intratho- racic or abdominal EPS appear to have a propensity for CDH over other pulmonary malformations. a definitive diag- Discussion nosis of intradiaphragmatic EPS was made. They account for up to 6 % of cious and led to diagnosis. We could speculate that in addition to these abnormal events. Meanwhile. The cystic lesion is the intradiaphragmatic components could be a cause of CDH with intradiaphrag- EPS (arrowheads) in the abnormally thickened posterior rim of the diaphragm (arrows). there have only been five cases reported [3. 2]. During development at the end of the posterior rim of the diaphragm.962 Pediatr Surg Int (2014) 30:961–963 forms from the embryonic foregut. In their case. but when an incision result of an accessory tracheobronchial bud originating was made into the relatively thickened hernia sac and from the foregut. [10] presented the case of Fig. Escobar and Acierno [12] pre- congenital pulmonary malformations and are classified into sented the case of a 7-month-old female diagnosed with intralobar and extralobar types. Pulmonary sequestration can usually be identified using diagnostic imaging modalities such as US. [8] examined 166 infants born with CDH and found that 5 % had associated pulmonary anomalies. a laryngotracheal diverticulum Although there was no evidence of aberrant vascular 123 . 7]. An association between pulmonary malforma- tions and CDH has been described previously [6. a cystic lesion was found. depending on whether the intradiaphragmatic EPS both thoracoscopically and lapa- lesions are contained within the visceral pleura of the roscopically. [3] experienced two cases of intradiaphragmatic EPS Pulmonary sequestrations are masses of nonfunctioning (4-week-old male and 7-week-old female) both resected lung tissue that are supplied by an anomalous systemic thoracoscopically. adrenal bleeding. development of the diaphragm begins with fusion of the septum transversum. mesoesophagus. but a bulge in the diaphragm was suspi- native tracheobronchial tree. 1 Operative findings. the arterial blood supply to the lesion arose from the celiac axis and its venous drainage was the splenic vein. renal tumor. no mass was observed initially normal lung or not [1. i. adrenal tumor. and body wall musculature [4]. Intradiaphragmatic EPS is extremely rare in children and. The pleuroperitoneal canal closes from the eighth to tenth week of gestation [5] and a wan- dering accessory bud forming at the level of the diaphragm in association with the fusing diaphragmatic components could be the mechanism of intradiaphragmatic EPS occurring. They are considered to arise as a during laparotomy for CDH repair. This ventral outpou- ching separates from the primitive esophagus with the formation of the tracheoesophageal groove. third week of gestation. incomplete fusion of the diaphragmatic Fig. pleuroperitoneal membranes. Fauza et al. or peridiaphragmatic [9].. intraabdominal. However. intradiaphragmatic EPS can be difficult to differentiate from other anomalies such as intraabdominal EPS. Sc. In our case. or MRI. stomach matic EPS. 2 Histopathology reveals immature alveolar tissue (Al) (left panel) and bronchial component (Br) containing cartilages (Ca) (right a 16-month-old boy with EPS diagnosed on prenatal US panel) compatible with EPS and MRI. and lymphatic or vascular malformation [3]. foregut duplication. They could not identify feeding or artery and only rarely have a bronchial connection to the draining vessels. computed tomography (CT). After exploring the diaphragm both laparoscopically and thoracoscopically. St. Meier et al. 10–12] (Table 1). as far as we know. EPS can exist rarely outside of the thoracic cavity. McAteer et al.e. An abnormally placed accessory bud forming at any level along the primitive foregut can form a pulmonary sequestration.

Flageole H (2004) The management of sequestration. and reports of CDH with intradiaphragmatic EPS in the English laryngotracheoesophageal cleft: two case report. J Pediatr Surg 47:2129–2133 pulmonary sequestration: advantages of the thoracoscopic approach. Caulet T (1962) Intradiaphragmatic estra-lobar pulmonary 2. and impact on prognosis. extralobar sequestration. MRI magnetic resonance imaging.A N.A. Surgery 84:413–420 temic blood supply.A N.A N. and associated anomalies: their incidence. Stocker JT et al (1997) tralobar sequestration-a rare pulmonary anomaly.A. Rosado de Christenson ML. there are no previous phragmatic hernia: associated malformations – cystic adenoma- toid malformation. Surg Clin North Am 86:329–352 123 . CXR chest X-ray. Am J Clin Pathol 72:917–925 intradiaphragmatic EPS to be made. J Pediatr Surg Intralobar sequestration: radiologic-pathologic correlation. Kays DW (2006) Congenital diaphragmatic hernia and neonatal lung lesions. Ricca R et al (2012) Intradiaphragmatic review of the literature. Woolley MM. prenatal Intraabdominal Laparoscopic/thoracoscopic MRI and CT EPS excision 3 [3] 7 weeks F Asymptomatic Prenatal US and CT Intraabdominal Laparoscopic exploration and mass thoracoscopic excision 4 [3] 4 weeks M Asymptomatic Prenatal US and CT Intrathoracic Thoracoscopic exploation and mass excision 5 [12] 10 months F Asymptomatic Prenatal US and CT Intrathoracic Laparoscopic/thoracoscopic EPS excision 6 [Present case] 7 days F Respiratory problem CXR and CT Incidental Excision during CDH repair by due to CDH laparotomy CT computed tomography. J Pediatr Surg literature. Mahour GH et al (1977) Pulmonary branches of diaphragmatic arteries arising from the sys. McAteer J. F female.Pediatr Surg Int (2014) 30:961–963 963 Table 1 Reported cases of intradiaphragmatic EPS Case no.A 2 [10] 16 months M Asymptomatic Prenatal US.A. Ryan A. These operative findings and histopa. doi:10. Acierno SP (2012) Laparoscopic resection of an Suppl A:S305–S312 intradiaphragmatic pulmonary sequestration: a case report and 3. Kagan-Hallet K (1979) Extralobar pulmonary thology allowed a definitive diagnosis of sequestration: analysis of 15 cases.1002/ppul. Stephenson J. Pediatr Res Rev 5 12. Berteloot L. US ultrasound supply from the aorta. not available. Buntain WL. Fauza DO.22891. References [Epub ahead of print] 10. Bratu I. 44:e27–e29 diographics 17:725–745 11. N. M man. Finer NN. Etches PC et al (1995) Congenital dia- To the best of our knowledge. Cilley RE (2009) Intradiaphragmatic ex- 1. 7. This is the first report of CDH with incidental 30:883–885 intradiaphragmatic EPS in a neonate that was treated 8. Escobar MA Jr. identification. Wilson JM (1994) Congenital diaphragmatic hernia successfully. Pediatr Pulmonol. J Pediatr Surg 47:1607–1610 4. Stocker JT. J Pediatr Surg 29:1113–1117 9. the mass may have been fed by 5. Frazier AA. Laberge JM. Chouikh T. N. Age Sex Symptom Imaging studies Initial diagnosis Surgical treatment 1 [11] N. sequestration in children: a twenty-five year experience. Ra. 6. Archives d’anatomie pathologique 10:150–151 asymptomatic congenital lung malformations. Eggli KD. N. Meier AH. Revillon Y et al (2013) Extralobar pulmonary sequestration with combined gastric and intradia- phragmatic locations.

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