Online Quiz

CASE 4 ( Closed )

History :
2 years old male child presented with history of tachypnea, retractions, hypoxemia, on examination bilateral crackles were present.

Question:

Review the imaging series and give your diagnosis.
What are the differentials diagnosis?

Please see the answer below.

ANSWER

FINDINGS:




















CT IMAGES SHOW

  • prominent ground-glass opacification (GGO) RML, lingula, RUL, LUL, RLL, LLL
  • Air trapping

DIAGNOSIS

Neuroendocrine cell hyperplasia of infancy


Teaching Points:

  • Typical HRCT appearance differs from the appearance of other forms of childhood ILD.
  • The imaging appearance of neuroendocrine cell hyperplasia of infancy can lead the radiologist to suggest a specific diagnosis and help in treatment.

DISCUSSION / GENERAL CONSIDERATIONS

CHILDHOOD ILD

  • Has no predominant type.
  • Children also have been found to have unique disorders not found in adults.
  • Biopsy is performed far less frequently on children than on adults.
  • Alveolar growth abnormality due to bronchopulmonary dysplasia.
  • Neuroendocrine cell hyperplasia of infancy.
  • Lymphangiectasia.
  • Surfactant protein abnormality due to defective adenosine triphosphate binding cassette A3.
  • Surfactant protein C abnormality.
  • Lymphocytic interstitial pneumonitis and.
  • Niemann-Pick disease.

Neuroendocrine cell hyperplasia of infancy

  • Is a form of childhood interstitial lung disease originally reported as persistent tachypnea of infancy.
  • Affects infants aged 6-24 months ,but can persist for 5-6 years.
  • Has a prolonged course with eventual improvement.
  • Corticosteroids have not been effective in improving lung status.
  • Correct identification of this disorder thus has prognostic and therapeutic importance.
  • Immunostaining for bombesin -necessary for pathologic confirmation of the diagnosis.
  • Understanding of the imaging appearance of neuroendocrine cell hyperplasia of infancy on thin-section CT scans necessary.

Pulmonary neuroendocrine cells (NEC)

  • Granulated epithelial cells found in the conducting airways and as neuroepithelial bodies in lung parenchyma.
  • Play a role in regulating fetal lung development.
  • Levels of cells peak in mid gestation, decline rapidly after neonatal period.
  • Produce bombesin-like peptide, serotonin, and calcitonin that play roles in bronchoconstriction, vasoactivity, epithelial differentiation & smooth muscle alteration.

Bombesinimmunostaining

  • A marked increase in neuroendocrine cells within the bronchioles and increased size and number of neuroepithelial bodies in the lobules.

IMAGING FINDINGS:

  • Prominent ground-glass opacification (GGO) RML, lingula, RUL, LUL, RLL, LLL.
  • Air trapping.

CT Scan

  • Most severe abnormality being GGO involving the right middle lobe and lingula with involvement of at least four lobes .
  • The second most severe abnormality being mosaic attenuation air trapping.
  • Consolidation.

Very rare- Bronchiectasis
Not seen- Linear or reticular opacities, Bronchial wall thickening, Honeycombing ,Interlobular septal thickening

  • differs from that of other forms of childhood ILD characterized by air trapping and GGO.
  • Bronchiectasis is a primary finding in bronchiolitis obliterans.
  • Air trapping is a prominent finding in asthma, but GGO is not seen.
  • GGO is a major feature of surfactant protein abnormality but is more diffuse than in neuroendocrine cell hyperplasia of infancy and is associated with septal thickening.
  • Consolidative patterns were seen in aspiration syndromes, BOOP , and vasculitides.
  • Characteristic thin-walled, heterogeneous cysts, alternating with small nodules, were seen only in patients with LCH.
  • Septal thickening was correlated with lymphangiomatosis and pulmonary capillary hemangiomatosis.
  • The term ''NEHI syndrome'' is used to indicate a diagnosis of NEHI without lung biopsy, based on a consistent clinical presentation and radiographic ļ¬ndings.

TREATMENT:

  • Supplemental O2, nutritional support.
  • No sustained response to bronchodilators and glucocorticoids.
  • Common colds and flu can be more severe in NEHI patients, so limiting exposure to respiratory infections is also important.
  • Most improve with time.
  • So early diagnosis of NEHI can help to prevent unnecessary corticosteroid administration to the child.

REFERENCES:

  1. Brody AS, Guillerman RP, Hay TC, Wagner BD, Young LR, Deutsch GH, Fan LL, DeterdingRR.Neuroendocrine cell hyperplasia of infancy: diagnosis with high-resolution CT. AJR Am J.
  2. Roentgenol. 2010 Jan;194(1):238-44.Deterding RR, Pye C, Fan LL, Langston C. Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia. PediatrPulmonol. 2005 Aug;40(2):157-65.
  3. Deutsch GH, Young LR, Deterding RR, Fan LL, Dell SD, Bean JA, Brody AS, Nogee LM, TrapnellBC, Langston C; Pathology Cooperative Group, Albright EA, Askin FB, Baker P, Chou PM, Cool CM, Coventry SC, Cutz E, Davis MM, Dishop MK, Galambos C, Patterson K, Travis WD, Wert SE, White FV; ChILD Research Co-operative. Diffuse lung disease in young children: application of a novel classification scheme. Am JRespirCrit Care Med. 2007 Dec1;176(11):1120-8. KerbyGS, Wagner BD, Popler J, Hay TC, Kopecky C, Wilcox SL, Quinones RR, Giller RH, AccursoFJ, Deterding RR.
  4. Abnormal infant pulmonary function in young children withneuroendocrine cell hyperplasia of infancy. Manuscript under review. Popler J, Gower WA, MogayzelPJ Jr, Nogee LM, Langston C, Wilson AC, Hay TC, Deterding RR.
  5. Familial neuroendocrine cell hyperplasia of infancy. PediatrPulmonol. 2010 Aug;45(8):749-55.Popler J, Young LR, Deterding RR.
  6. Beyond infancy: persistence of chornic lung disease in neuroendocrine hyperplasia of infancy. Am j RespirCrit Care Med 2010;181:A6721. Young LR, Brody AS, Inge TH, Acton JD, Bokulic RE, Langston C, Deutsch GH.
  7. Neuroendocrinecell distribution and frequency distinguish neuroendocrine cell hyperplasia of infancy from other pulmonary disorders. Chest. 2011 May;139(5):1060-71.