Clinical Features of Congenital Cystic Lung Diseases

A Report on a Nationwide Multicenter Study in Japan

Tatsuo Kuroda, Eiji Nishijima, Kosaku Maeda, Yasushi Fuchimoto, Seiichi Hirobe, Yuko Tazuke, Kumiko Nozawa, Kentaro Matsuoka, Noriaki Usui

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Aim The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. Methods Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. Results In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p <0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. Conclusions Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

Original languageEnglish
Pages (from-to)91-95
Number of pages5
JournalEuropean Journal of Pediatric Surgery
Volume26
Issue number1
DOIs
Publication statusPublished - 2015 Oct 27

Fingerprint

Multicenter Studies
Japan
Lung
Cystic Disease Of Lung
Hydrops Fetalis
Apgar Score
Vital Capacity
Cysts
Ultrasonography
Carcinogenesis
Thorax
Parturition
Incidence
Growth
Infection

Keywords

  • congenital cystic adenomatoid malformation
  • congenital cystic lung disease
  • lung development
  • prenatal diagnosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Clinical Features of Congenital Cystic Lung Diseases : A Report on a Nationwide Multicenter Study in Japan. / Kuroda, Tatsuo; Nishijima, Eiji; Maeda, Kosaku; Fuchimoto, Yasushi; Hirobe, Seiichi; Tazuke, Yuko; Nozawa, Kumiko; Matsuoka, Kentaro; Usui, Noriaki.

In: European Journal of Pediatric Surgery, Vol. 26, No. 1, 27.10.2015, p. 91-95.

Research output: Contribution to journalArticle

Kuroda, T, Nishijima, E, Maeda, K, Fuchimoto, Y, Hirobe, S, Tazuke, Y, Nozawa, K, Matsuoka, K & Usui, N 2015, 'Clinical Features of Congenital Cystic Lung Diseases: A Report on a Nationwide Multicenter Study in Japan', European Journal of Pediatric Surgery, vol. 26, no. 1, pp. 91-95. https://doi.org/10.1055/s-0035-1566095
Kuroda, Tatsuo ; Nishijima, Eiji ; Maeda, Kosaku ; Fuchimoto, Yasushi ; Hirobe, Seiichi ; Tazuke, Yuko ; Nozawa, Kumiko ; Matsuoka, Kentaro ; Usui, Noriaki. / Clinical Features of Congenital Cystic Lung Diseases : A Report on a Nationwide Multicenter Study in Japan. In: European Journal of Pediatric Surgery. 2015 ; Vol. 26, No. 1. pp. 91-95.
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abstract = "Aim The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. Methods Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. Results In the 194 patients who were prenatally diagnosed to have CCLD, 16.7{\%} presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4{\%}. As of postnatal day 30, 14.0{\%} of the neonatal patients required respiratory support, and 3.3{\%} (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6{\%} of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1{\%} did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p <0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. Conclusions Approximately 10 to 15{\%} of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.",
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N2 - Aim The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. Methods Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. Results In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p <0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. Conclusions Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

AB - Aim The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood. Methods Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis. Results In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p <0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series. Conclusions Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

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