Another case of congenital lobar

emphysema ??

Kamal MasarwehOctober 2018

Case Presentation

• born term 40 weeks GA

• spontaneous vaginal delivery

• birth weight – 3.57 kg, Apgar scores 9/10

• pregnancy:

• uneventful pregnancy

• normal antenatal ultrasound (13 and 26

weeks of gestation)

• firstborn to healthy non-consanguineous parents

• 15/2/2018

• 2 months old

• Presented in a northern hospital:

• 5 days URTI

• cough, dyspnea, cyanosis, restlessness

• afebrile

• ER: dyspneic, use of accessory muscles

• Vitals:

• tachypneic 70 BPM

• saturation 88% on room air, 98% +O2

• heart rate – 180 bpm, BP – 115/57

• Physical examination:

• moderate respiratory distress

• intercostal retractions

• decreased breath sounds on the left

• displaced heart sounds to the right side

• Chest CT – hyperinflation on the left with septations, with a mediastinal shift to the right

• transferred to Rambam

• working diagnosis- CLE and significant respiratory compromise

• 16.2.2018 –

• left-sided thoracotomy

• lobectomy - left upper lobe

• uneventful surgery

• PICU

• extubated within one day

• analgesia and drainage

• post-operative pneumonia - antibiotics

Chest X-ray after surgery

• Is it congenital lobar emphysema??

typical age of presentation

typical location

typical herniation

× evidence of septations

× normal prenatal US (weeks 13 & 26) including revision after birth

Differential diagnosis

• Congenital Lobar Emphysema

• CPAM

• Pleuropulmonary Blastoma

• Bronchogenic cyst

• Others

• macroscopically - multiple septated cystic lesion measuring 9x6x2 cm

• microscopically - cystic lesions of various sizes, lined with a monolayer of endothelial cells

x40

X100

• Markers used in immunohistochemical staining for differentiating lymphatic lesions from other etiologies:

• D2-40- monoclonal antibody to podoplanin, highly reliable lymphatic endothelial marker. Stains the endothelium of lymphatic channels but not that of blood vessels

• CD31, CD34, ERG, factor VIII-related antigen – endothelial markers

• Minato H , Solitary intrapulmonary cystic lymphangioma in an infant: a case report with literature review. Pathol Res Pract. 2010 Dec 15;206(12):851-6

• Immunohistochemical staining - specific lymphatic vessel marker D2-40 -positive

x100

•Diagnosis:

Intrapulmonary macrocystic

lymphangioma

Pulmonary lymphatic system disorders

• rare lesions

• occur in a variety of non-neoplastic and neoplastic settings

• classification challenging

• inconsistent terminology

• mostly case reports and case series

Intrapulmonary Lymphangiomas

• extremely rare

• approximately 20 cases in literature

• patient ages – neonates (3 cases) to 54 yr

• frequently in the lower lobes of the lung, probably associated with a rich lymphatic supply

Clinical features

• symptoms vary widely depending on the age of the patient and the extent of disease

• neonates and infants - often present with pneumothorax and respiratory distress

• may remain asymptomatic until compression of vital structures

• may present as incidental findings on roentgenograms

Radiology

• Chest X-rays, US, CT scan, and MRI

• CT appearance - cystic mass with smooth margins

• no published data about antenatal US

• hypothesis that lymphangiomas become clinically significant in later stages of pregnancy or even after birth, possibly due to obstruction of lymphatic vessels

Intrapulmonary Lymphangiomas

• Treatment:

• surgical resection - frequently mandated both to confirm the diagnosis, and to prevent complications that arise from compressive effects on vital structures

• CT-guided Sclerosis

• natural history of intrapulmonary lymphangiomas in infants is not well-known

• 3 neonatal cases (up to 4 years follow up) –no recurrence

28 days 2 months 2 months

Takahashi et al, 2011 Minato el at, 2016 Hun Lee et al, 2004

Take home message

• Not all that looks like CLE is actually CLE

• Intrapulmonary lymphangioma mimics CLE and CPAM

• Does is it really matter to diagnose??

• is natural history of pulmonary lymphangiomas different than CLE/CPAM?

• is there a chance of recurrence?

• tendency for multiple lesions?

• is there a need for a different follow up?