Right middle lobe (RML) syndrome describes recurrent RML collapse, infection or a combination of the two. Of all the lobes of the lung, the RML has the poorest drainage or clearance. The airway to this lobe is long and thin. As a result mucus gets trapped. Infection frequently fails to clear and the result is permanent damage to the RML. If a person has rare recurrences, nothing needs to be done other than antibiotic treatment. When infection is recurrent, the only cure is surgical removal of the RML.
Without knowing what tests were done that "show nothing" I would recommend this 3 year old be seen by a pediatric pulmonologist. Almost certainly this lung specialist will do a bronchoscopy. Surgical removal of the right middle lobe (RML) will probably be suggested.
Consider having your child evaluated at a GREAT teaching hospital which specializes in pulmonology and immunology, such as National Jewish, Johns Hopkins, or Mayo Clinic, or similar.
Your child may have an infection that needs aggressive treatment to knock it out, or have immunity issues. You can get more info about becoming a NJewish patient at www.NationalJewish.org; many of the other hospitals also have websites & toll-free info. Many insurance companies participate with these hospitals.
I suggest you telephone the LUNG LINE nurses, at 1-800-222-LUNG(5864), (call between 8am & 4:30pm, Mon-Fri, Mountain standard time) and ask your good questions! I agree with Starion, and if you can have your child seen at Nat'l Jewish (Denver, CO), you may get some real answers, and help for her, quickly!
Follow your gut instincts, and get another opinion from experts! Good luck to you, in getting help for your child!
this child sees a pediatric lung specialist. Did autoimmune test said low for age but can't dx auto immune. CF test neg., She is amazing usually no signs of sickness she'll lay down for nap and wake with high fever. Many of the pnemonias to listen her lungs sound clear. She was on zythromax for 6 months and still not totally clear Dr. wanted to try her off and she became sick 3 times in 2 months untill she was finnally readmitted for IV antibiotics. Personally I feel her color is bad, doc says its fine, her o2sats are staying around 90. She still is very active, eats very little, Her pumologist says there isn't allot else to do except she may need to stay on antibiotic indefinate. I feel there should be more or we are just missing something. Also is it possible for them to come up with a false neg. CF test? Should I request another? What about a bronch? Her Dr. doesnt feel it's needed but if the problem is fixable??
A bronchoscopy may be advisable, and even therapeutic in many cases, see below:
Int J Pediatr Otorhinolaryngol. 1987 Jun;13(1):11-23.
Right middle lobe syndrome in children.
Livingston GL, Holinger LD, Luck SR.
Right middle lobe syndrome (RMLS) is characterized by a spectrum of diseases from recurrent atelectasis and pneumonitis to bronchiectasis of the middle lobe. It has been described among all age groups, although the diagnosis in pediatrics may be delayed or missed because of non-specific symptoms or findings. Twenty-one children with RMLS were evaluated during the past 10 years with particular attention to the history, bronchoscopic and pathologic findings. Most of these patients had asthma or a family history of atopic disorders; 3 patients had a family history of RMLS. Only two of the 21 patients had sufficient obstruction on bronchoscopy to account for their disease. Four had evidence of concomitant laryngeal pathology. The various theories of pathogenesis are discussed. In this series, the non-obstructive (impaired collateral ventilation) theory appeared to be most plausible. Bronchoscopy was performed in all instances to rule out obstruction due to foreign body or tumor. It was therapeutic in two-thirds of the cases. Resolution occurred
promptly in one-third, and eventually in another third. Of the remaining patients, 4 required lobectomy and were cured; two have decreased but persistent symptoms. An aggressive medical management following bronchoscopy is warranted in all cases, especially when there is a possibility of asthma.
PMID: 3623806 [PubMed - indexed for MEDLINE]
Also see: http://www.emedicine.com/ped/topic2018.htm
Right Middle Lobe Syndrome
Last Updated: November 18, 2003
Author: Nemr S Eid, MD, Director of Pediatric Pulmonary Medicine, Professor, Department of Pediatrics, University of Louisville School of Medicine
The value of bronchoscopy is 2-fold as follows: (1) It is immediately therapeutic in removing mucus and clearing the airway
and can be curative in some cases. (2) It allows visualization of the airway and the ability to determine patency of the right
middle lobe bronchus and to discern whether endobronchial obstruction is the cause.
Bronchoalveolar lavage can be performed concurrently to determine cellular elements in the right middle lobe. It can also be used to assess the presence of infections by culturing and staining for bacterial, fungal, viral, and mycobacterial pathogens."
*Long-term follow-up of children shows that most patients do not experience recurrent or persisting symptoms. This indicates that the first line of treatment in all cases is conservative medical management, except in cases involving neoplastic origin and those with bronchiectasis.
*Chest physical therapy and postural drainage are the hallmarks of therapy.
*Treat the asthmatic child with aggressive anti-inflammatory therapy such as inhaled steroids. Consider systemic steroids.
*Provide the patient with chest physical therapy and postural drainage. In unresponsive patients or patients who have
a predisposition to airway colonization, an appropriate antibiotic, as determined by a bronchoalveolar lavage (BAL)
culture, should be added to their regimen.
*Patients with fungal infections (eg, histoplasmosis) or tuberculous infections who have hilar adenopathy and complete
blockage of their right middle lobe should be treated aggressively. The addition of systemic corticosteroids may be
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