Neuroendocrine Cell Hyperplasia of Infancy: Diagnosis With High-Resolution CT

Abstract

OBJECTIVE-Neuroendocrine cell hyperplasia of infancy is a form of childhood interstitial lung disease originally reported as persistent tachypnea of infancy. Reports of small series of cases and anecdotal experience have suggested that this disorder may have a consistent CT pattern. The purpose of this study was to review the CT findings in children with neuroendocrine cell hyperplasia of infancy to determine the findings at high-resolution CT, the diagnostic accuracy of CT compared with biopsy, and interrater reliability. MATERIALS AND METHODS-Images from 23CT examinations of children with biopsyproven neuroendocrine cell hyperplasia of infancy and six CT examinations of children with other childhood interstitial lung diseases were reviewed by two pediatric radiologists with special expertise in thoracic imaging. Identifying digital data were removed, and images were reviewed without clinical data. A CT assessment form was completed for each patient.RESULTS-Ground-glass opacification was the most common finding in patients with neuroendocrine cell hyperplasia of infancy. The right middle lobe and lingula were most commonly involved. Air trapping with a mosaic pattern was the second most common finding. Interrater reliability was very good with a kappa value of 0.93. The sensitivity and specificity of CT in the diagnosis of neuroendocrine cell hyperplasia of infancy were at least 78% and 100%.CONCLUSION-Neuroendocrine cell hyperplasia of infancy can have a characteristic appearance on high-resolution CT scans, the imaging findings being useful in differentiating neuroendocrine cell hyperplasia of infancy from other types of childhood interstitial lung disease.© American Roentgen Ray Society Address correspondence to: A. S. Brody (alan.brody@cchmc.org). The appearance aids radiologists in suggesting a specific diagnosis but does not exclude this diagnosis; in 17-22% of cases, the readers in this study did not suggest the diagnosis of neuroendocrine cell hyperplasia of infancy when it was present. Immunostaining for bombesin, a procedure not routinely performed, is necessary for pathologic confirmation of the diagnosis, therefore, CT findings suggesting the diagnosis can affect the pathologic evaluation. Improved understanding of the imaging appearance of neuroendocrine cell hyperplasia of infancy on thin-section CT scans should help determine the role of imaging in the evaluation of these children. NIH Public AccessWe reviewed the CT findings of 23 patients with histologically confirmed neuroendocrine cell hyperplasia of infancy and of six patients with other pathologically confirmed forms of childhood ILD with the intent of determining the high-resolution CT (HRCT) findings of neuroendocrine cell hyperplasia of infancy, the accuracy of CT findings of neuroendocrine cell hyperplasia of infancy compared with biopsy findings; and interrater reliability with use of a standardized CT assessment form in the diagnosis of neuroendocrine cell hyperplasia of infancy. Materials and MethodsImages from 29 CT examinat...

Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis

“…[11][12][13][14][15][16] Furthermore, computed tomography (CT) scan patterns have been shown to be specific for several types of ILD, 17,18 including neuroendocrine cell hyperplasia of infancy (NEHI), enabling diagnosis without lung biopsy in some cases. 19 Thus, the routine use of lung biopsy to diagnose and characterize ILD in children may no longer be the standard of care, although the classification scheme is predicated upon this information. 4 Recent studies of childhood ILD have been performed at specialized referral centers.…”

“…Therefore, in the current era of clinically available genetic testing, we anticipate reductions in the numbers of surgical lung biopsies in this patient population. Lastly, in retrospect, 5 of the 8 NEHI cases met criteria for diagnosis based on clinical and radiographic findings, 19,22 and therefore lung biopsy would be unlikely to be performed in the current clinical practice pattern at our center and others. 23 We recognize the limitations inherent to this type of retrospective study.…”

“…4,19,21,22 Of the 8 NEHI cases, 5 were recognized via the retrospective review process. Three of these 5 cases came to clinical attention before the original description of NEHI in the medical literature in 2005.…”

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

“…The observation that diagnostic testing provides a specific diagnosis for more than 50% of patients suspected of having ILD derives from three multicenter retrospective studies (6,11,15) and two single-center prospective case series (13,16) that described the occurrence of ILDs and DLDs in children and the utility of diagnostic testing, as well as four diagnostic accuracy studies for high-resolution CT (HRCT) in DLDs (17)(18)(19)(20). Our confidence in the results of these studies is diminished by the study design, the absence of studies using the current chILD syndrome definition in a general pediatric pulmonary patient population, and the small numbers of patients with some forms of DLD, particularly the more recently recognized entities.…”

CT chest under general anesthesia: pulmonary, anesthetic and radiologic dilemmas

“…For infants, it is frequently indicated in the evaluation of suspected interstitial lung diseases or congenital anomalies [1]. In some cases of interstitial lung diseases, typical presentations of neuroendocrine cell hyperplasia of infancy for example, a properly performed chest CT is diagnostic [2], thus alleviating the need for more invasive procedures like surgical lung biopsy. Even when surgical lung biopsy is necessary, prebiopsy chest CT highlights areas of greatest pathological involvement increasing the likelihood of diagnosis.…”