Late presentation of diaphragmatic hernia in a Pacific Island pediatric population.

BACKGROUND: Congenital diaphragmatic hernia (CDH) usually presents at birth with respiratory distress syndrome (RDS) and has a high mortality rate if not promptly recognized and treated. The incidence of CDH is reported to be 0.8 - 1.0/10,000 registered births. Less than 3% present after the neonatal period. In the latter, ie. late-presenting CDH (L-pCDH), the prognosis is improved because pulmonary hypoplasia does not develop. With the creation and application of a store-and-forward telemedicine system to the Pacific Island Health Care Project (PIHCP), we have come to realize that this rare condition ie., (L-pCDH) occurs with unusual frequency in the United States Associated Pacific Islands (USAPIs), especially those of the Federated States of Micronesia (FSM).

METHODS: Information concerning CDH was identified from the archived data base of the over 3, 100 cases of the PIHCP as well as the composite data base of Tripler Army Medical Center (TAMC). The cases of CDH diagnosed after the neonatal period (> 30 days of age) were considered to have L-pCDH. The study period was from 1997 - 2006.

RESULTS: During the study period (1997-2006), 12 cases of CDH were referred from the PIHCP. There was 1 case of CDH born at TAMC (beneficiary from Micronesia) during the same period. The number of births over the 10-year period of study was similar for the referral base (PIHCP) and TAMC. Five of the 12 cases in which the age at diagnosis could be established, were L-pCDH from the PIHCP. The one case of CDH born at TAMC, during the 10 year period, was diagnosed in the neonatal period (< 30 days of age).

DISCUSSION: These results confirm that CDH occurs with increased frequency in the USAPIs as contrasted to the frequency in the developed world. The incidence of L-p CDH is far in excess in the USAPIs. This review highlights the fact that this group of patients can be easily identified using tele-techology (attached chest X-rays demonstrating stomach and/or bowel gas in the chest) thus allowing remote diagnosis and referral for ultimate surgical correction of this potentially lethal condition. Infants and children with CDH: provided excellent graduate medical education (GME) for residents-in-training; are afforded advanced surgical treatment unavailable in the remote jurisdictions of the Pacific; and, following surgery, are restored to health to return to their homes so as to become contributing members of society.

CONCLUSION: CDH, especially L-pCDH occurs with increased frequency in the USAPIs. The Web-based-store-and-forward telemedicine PIHCP allows for remote diagnosis. Patients so identified are transported to TAMC for definitive treatment. These patients provide excellent GME to our residents-in-training. Following surgical correction these patients are restored to health to return to their homes to mature and become contributing members of society.