Original article
The diagnosis and management of intradiaphragmatic extralobar pulmonary sequestrations: a report of 4 cases

https://doi.org/10.1016/j.jpedsurg.2011.11.066Get rights and content

Abstract

Background/Purpose

Intradiaphragmatic extralobar pulmonary sequestrations (IDEPSs) are a rare subset of bronchopulmonary sequestrations (BPS). We report the largest series of patients with IDEPS and describe the diagnostic and operative challenges associated with this condition.

Methods

We retrospectively reviewed our experience with fetal and pediatric BPS from 1995 to 2010 to identify patients with IDEPS.

Results

We identified 27 patients with BPS and 4 patients in whom the masses were within the diaphragm. In 1 patient, the prenatal ultrasound correctly identified the mass as being within the diaphragm itself, whereas the remaining cases were thought to be intraabdominal or had discordant preoperative imaging findings. The diagnosis of an IDEPS proved challenging to make prospectively using prenatal ultrasound, computed tomography, or magnetic resonance imaging. All patients underwent attempted resection. Two cases required a combined laparoscopic and thoracoscopic approach to accurately localize the mass. The postoperative recovery of these patients was uneventful.

Conclusions

We present the largest reported experience of IDEPS. Because preoperative imaging studies cannot always determine whether a sequestration is intraabdominal, intrathoracic, or intradiaphragmatic, operative planning may pose a challenge. However, the use of minimally invasive approaches can allow exploration of both the thoracic and abdominal cavities with low morbidity.

Section snippets

Methods

We reviewed all cases of fetal and pediatric BPS at University of California, San Francisco, from 1995 to 2010 and classified each patient based on the location of the lung lesion at the time of operative resection. Information was obtained from hospital and pediatric surgery clinic records. Patients with congenital pulmonary airway malformations were not included in this series. We identified a small cohort of patients with IDEPS, each of whom underwent operative resection by 1 of 4

Results

From 1995 to 2010, we identified 27 patients who underwent operative resection for BPS at our institution. We stratified the location of these lesions based on their intraoperative findings. Most cases were found within the thoracic cavity (n = 18, or 66.7%). Of the 5 cases that were located within the abdominal cavity (18.5%), 3 were densely adherent to the diaphragm. An additional 4 sequestrations (14.8%) were located within the diaphragm itself (Table 1).

The prospective diagnosis of IDEPS

Discussion

Bronchopulmonary sequestration is a type of bronchopulmonary foregut malformation that is thought to arise either from anomalous budding of the primitive bronchial tree and gastrointestinal tract or from in utero bronchial obstruction [6], [7]. Although the location (intrathoracic vs intraabdominal) of BPS is often accurately identified on prenatal imaging, our experience indicates that some lesions may be completely hidden within the diaphragm and, therefore, pose a unique diagnostic

Acknowledgments

We thank Drs Doug Miniati and Diana Lee Farmer for helpful discussions and Liz Gress and Vilma Zarate for their assistance in coordinating the study.

References (16)

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Presented at the 2010 American College of Surgeons Clinical Congress, Washington, DC.

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