Dear collegue

the risk is that if the drains do not work well or maybe you accidentally remove one of them the patient can risk a lot.

It is necessary tonsolve the problem radically.

In such cases of bilateral pneumothorax I would put the patient in safety with surgical resection of the cystic lesion and mechanical pleurodesis (not chemical) at a side. The same thing must be done on the other side later.

Let us known the evolution please

Could the cysts be hydatidosis?

Thank you Francesco Inzirillo for your suggestion. But how will you explain nodules if it hydatidosis? The drains are quite well working. We ultimately removed the drain after 1 month or so after doing pleurodesis though it was chemical. But how will we explain all these...multiple cysts of varying sizes with nodules having bilateral pneumothorax and cervical lymphadenopathy in a 13 year old boy?

It 's always very difficult to express opinions without being able to view images and other examinations. I did a little research and with the keywords pneumothorax, pulmonary nodules and lymphadenopathy you can find some rare diseases.

For example:

"Pulmonary involvement of Wilms' tumor"

http://www.ncbi.nlm.nih.gov/pubmed/179338

"Pulmonary manifestations of ankylosing spondylitis"

http://www.ncbi.nlm.nih.gov/pubmed/20692546

"lymphangioleiomyomatosis"

http://www.ncbi.nlm.nih.gov/pubmed/21904573

"pulmonary Langerhans' cell histiocytosis"

http://www.ncbi.nlm.nih.gov/pubmed/19035226

"Lymphangioleiomyomatosis and Langerhans cell histiocytosis"

http://www.ncbi.nlm.nih.gov/pubmed/16457015

I think that a diagnosis of certainty can only be achieved by surgery

Interstitial pneumonitis

Thank you very much Francesco Inzirillo for your effort...we did consider some of the d/ds mentioned by you. Lets see wat the reports tell us

Dear Colleague,

I agree with Dr Inzirillo about the necessity to view the CT images in order to have a clear vision of the radiological status of the young boy.

The diagnosis could be from infectious disease (tuberculosis, aspergillosis, hydatid cysts, Pneumocystis carinii pneumonia, staphylococcus) to non infectious diseases (lymphangioleiomyomatosis, Kartagener disease, Pulmonary Langerhans’ cell histiocytosis, Tuberous sclerosis, Lymphoid interstitial pneumonia).

The following steps in my opinion should be considered:

- to perform fiberoptic bronchscopy with BAL for microbiology and citology;;

- to perform microbiological and citological examination of the both, right and left, pleural fluid;

- to perform cervical nodal biopsy (ine needle or surgical) and pathological and microbiological exams of the specimen.

fibre-optic bronchoscopy with BAL didnt reveal any organism...we did mycobacterial, fungal and bacteriological culture...cell types were neutrophils 80%

Cervical LN excision biopsy was done, report of which is still not available

I will upload the CT features

Ok!

HRCT scan thorax

The images let me think first about an interstitial disease than infectious

yes i am agreed with u...interstitial disaese presenting with pneumothorax are mainly LAM and PLCH.....LAM is not suiting with patient profile...though PLCH is not also suiting coz it occurs more commonly in middle aged smoker...but can occur in childhood...we keep PLCH as our one of the most desirable D/Ds...lets see wat HPE report brings for us

Dr Wanis Ibrahim (Doha-Qatar)

Dear Dr Arnab, This is a very interesting case. Please allow me to contribute to the discussion.

1. The available cuts of the CT scan shows bilateral pneumothoaces, multiple thick-walled cysts (only the upper lobe cuts available) as well as fibrotic changes. I guess also some pleural thickening of the left posterior (mediastinal cuts not available). Together, with the presence of cervical lymphadenopathy, infectious process or malignant process should be ruled out.

2. Lymphangioleiomyomatosis unles associated with tuberous sclerosis is a remote possibility as it is exclusively in females (one case reported with kilnefilter !) - look for signs of Tuberous sclerosis

3. Lymphocytic interstitial pneumnia usually occurs with Sjogren's disease, autoimmune disaeses and HIV and cysts usually predominate in the lower lobes rather than upper.

4. Pulmonary Langerhan's histiocytosis a disease of heavy smokers. I guess being a 13 year old doesn't smoke or it is early to develop now

5. Bert-Hogg-Dube syndrome is associated with cutaneous lesions, papules and renal tumors.

Conclusion: to diagnse cystic lung diseases you need careful look at the distribution, size and characters of the cystic changes and therfore , more ct cuts are required. I guess the scinario of your case fits more an infectious or maliganant process

Kindest rgards

Here, all of us, on the basis of a fast clinical description and a few low-resolution images, we are making beautiful elegant poetry. The patient need an accurate diagnosis for a specific therapy. Objective data are necessary. While waiting for the lymph node I resume the proposal of Dr Galetta about bronchoscopic examinations. If everything should not lead to anything then it will be necessary an histologic tissue confirmation.

Are there any skin lessions, renal, or cerebral lesion? as a differential you could also be dealing with tuberous sclerosis

http://www.medigraphic.com/pdfs/neumo/nt-2012/nt123g.pdf

Greetings

thank Walid Dajer-Fadel

If no lesions in other organs, and the more acute onset, the case may be viral infection

This is a very interesting case. The acute onset of the disease do not necessarily means that it is viral or infectious. I think it is just spontaneous perforation of the bullous lesions. Although the presence of lymph node let us think malignancy, the CT scans let me think firstly interstitial etiology. However, detailed histopathological examination of lung biopsy is needed to rule out a amlignancy or an interstitial disease of lung.

This interesting case reminds me of a young patient that we operated in VATS about 15 years ago. I attach the PDF of the paper that we published back then.

The CT findings favor interstitial lung disease. Asymmetric chest wall (larger in left side) indicates possible spinal and/or other bony deformity. Therefore, connective tissue disorder(s) with lung involvement should be excluded. My two cents!

I am completely agreed with Dr Ozlem Boybeyi. It is pneumothorax for which the disease came out so sudden, underlying process may be chronic. It is the rupture of the cysts in the pleural space causing pneumothorax that makes the disease so sudden. Langerhans cell histiocytosis may also occur in childhood in the form of Letterer Siwe disease or Hand-Schuller Christian disease but we didnt yet find the extra-pulmonary features except the enlarged lymphnode in this case....no skin lesions, skull x-ray was normal, no hepatosplenomegaly etc etc. We also assayed serum alfa 1 anti-trypsin level but it came to be normal today. Previously BAL fluid study as i have earlier uttered failed to give clue about any infections even after all forms of cultures. Serum procalcitonin is normal. HPE report of lymph node biopsy sample is yet to come. What else we can do in such scenario. Yes we will go for lung biopsy as a last resort if biopsy report of lymphnode failed to prove anything. Thank you all for your kind suggestion.

Bullous miliary tuberculosis?

To end all our speculations here, Ihave the excision biopsy report available at my hand now which shows histopathlogical featires suggestive of Langerhan's cell histiocytosis with immunohistochemistry positive for S-100 and CD-1a....

Finally!

Hi Arnab,

The first thing we need to establish is to identify the possible cause and ascertain the category if it is Primary, Secondary, traumatic or Tension Pneumothorax. The probable diseases could be Emphysema, Tuberculosis, Sarcoidosis, Cystic Fibrosis, or Lung cancer or unexplained presence of air blebs. As per your narration we shall probe for Tuberculosis, Sarcoidosis or Lung Cancer, a proper history here will be of great use, so you need to talk to parents of the child for complete relevant medical history.

This is a serious condition so it will be better if you refer the case to Tertiary Care Hospital where his present condition could be managed and an effective treatment could be carved out.

Regards

Dr.Rajinder Singh

Hello Dr Arnab Maji,

Thank you for a very interesting case. Sorry I cannot add anything but I have a question. Was the lung biopsy done by transbronchial route, or by means of thoracotomy/VATS ? The other question which i do not expect an answer to is why he presented at that particular time ie the trigger for the pneumothoraces.

Thanks again,

Lawrence Adu-Boateng

Dear Dr Lawrence Adu-Boateng,

As there was a clinically palpable cervical lymph node and diagnosis was established by excision biopsy, histopathological examination and immunhistochemistry we didnt further go for lung biopsy and if it is done we prefer to go for VATS biopsy.

Secondly the boy had his SOB while playing on the ground and it was sudden. So probably due to that stress some of his cysts in the lung ruptured in the pleural space causing pneumothorax.

This seems to be a case of secondary spontaneous pneumothorax due to rupture of cysts seen in Langerhan's cell histiocytosis (LCH). Lymphadenopathy is seen in approximately 20 percent of patients with LCH and cervical nodes are most commonly involved.

For me are two concomitant disease. The firths is secondary spontaneous PNX by rupture of cysts seen in LCH and the second is SOB in the base of primary disease(LCH)

Dear college i would like to thank for the exposure to us of such an interesting case .I would like to add that my position is to verify the diagnosis of LCH. I would strongly recomend to procced to the VATS biopsy for definitive diagnosis, of course in the absence of other possible lesions in the liver, bone espessialy skull and ribs or any dermatological fetures such as rush, or any mastoid deformations.Most data support the concept that LCH is a diversed disease characterized by a clonal growth of immature Langerhans cells that appear to have mutations of BRAF in about half the cases. Excess production of cytokines also appears to be important. Information has been collected in various studies which show that bone involvement occurs in approximately 78% of patients with LCH and often includes the skull (49%), hip/pelvic bone (23%), upper leg bone (17%) and ribs (8%). Skin LCH is seen in as many as 50% of patients. Lung lesions are seen in 20% to 40% of patients, while 30% of patients have lymph node involvement. In case of LCH there is very interesting treatment protocols depending on the severity and the risk of the patient, and they inclyde VELBAN/PREDNISONE/MERCAPTOPURINE+METHOTREXATE. I wich the best to your little patients and proceed fast accurate and adequately.

With respect and honor.

Thank you very much for your information. Would you kindly give some link about the above said informations especially about the statistical part and data?

This is classical LCH. VATS lung biopsy is not necessary in this case.

http://www.cancer.gov/cancertopics/pdq/treatment/lchistio/HealthProfessional/page2

Hi Arnab, I read the interesting case brought by you on the thirteen year old boy with sudden dyspnea.

Elements related to the presence of cysts of different sizes, sometimes bizarre morphology and the presence of centrilobular nodules that may cavitate also make the diagnosis suggestive of Langerhans cell histiocytosis.

In my experience radiological cases related to the disease were subjects in 90% of cases habitual smokers. The differential diagnosis of the disease in question turns out to be the relata lymphangioleiomyomatosis almost exclusively to women (estrogen-progestin hormones), LIP (lymphocitic interstitial pneumonia). In this boy the diagnosis of histiocytosis X with cyst rupture into the pleural cavity seems to me the only possible one.

LHC is primary on the list but wait for the HPE results - is he on a trial of ATDs already? No other systems involved? What bloods have been done & what do the results look like - I am reasonably aware of what is possible in your institution. VATS is unlikely to be necessary to clinch the diagnosis. Let us know the HPE reports.

In your poor center, no options left in your hand to reach the final diagnosis except to wait for lymph node biopsy result.

no there is option of VATS in our centre but if lymph node biopsy can fetch the diagnosis y we sd go for further invasive procedures...we already have a chain of investigations...

HPE results from LN Bx???

HPE - Langerhan's cell histiocytosis with CD 1a positive.........@samrat roychowdhury....would you kindly suggest a journal where we can send this case report for possible publication?

@ Arnab Maji: try European Journal of Internal Medicine (EJIM). The problem with case report is that it lets down the impact factor of any medical journal. And precisely that is the reason why most high impact journals do not accept case reports nowadays. Keep in touch with me through my e-mail [email protected] as I will be in Kolkata from 20th July for a fortnight & am happy to sit down with you to discuss where this could go.....take care!

Check his trypsin levels, it can happen with alpha 1 anti trypsin deficiency. 2ndly check for genes of cystic fibrosis

CF seems unlikely as it doesn't present with sudden onset pnemothoraces in early adolescence with no prior history of GI or respiratory illness. A1ATD is an interesting thought. It seems unnecessary to do further workup in this case.

What do you think about Marfan syndrome caused by the misfolding of the protein fibrillin-1. Fibrillin-1 is coded by the gene FBN1? Pulmonary symptoms are not a major feature of Marfan syndrome, but spontaneous pneumothorax is common

And Ehlers-Danlos syndrome or Birt-Hogg-Dubé syndrome?

Http://www.hindawi.com/crim/pulmonology/2011/414165/

muchos días de drenajes dan posibilidades a que aparezca empiemas pleurales.Se debe pensar en asegurar que no hará un neumotorax hipertensivo por lo que pensaría en practicar pleurodesis y esperar el resultado de la biopsia del ganglio cervical.

cystic lung disease

cause COPD

I agree that Marfan, Ehlers-Danlos or Birt-Hogg-Dubé syndromes can present with spontaneous pneumothoraces but lymphadenopathy is not a feature of any of the above.

My first opinion is histiocytosis x...

Tuberculosis, fungal infection, blood-lymph malignancy

Esperar el resultado de la biopsia de la adenopatia.

Vigilar y prevenir el neumotórax hipertensivo.

Dificil relación de la patología pulmonar y adenopatia cervical.

La disnea la explica el neumotorax en un pulmón quistico.

Biopsia pulmonar si la adenopatia no es concluyente.

No pleurodesis si hay posibilidades de trasplante pulmonar .Posibilidades deconocidas