A CASE OF SINOPULMONARY DISEASE WITH SITUS INVERSUS

- HARSHIT SHARMA

- MBBS 2011

COMPLAINTSThe patient is a 40 year old female, housewife who presented with

•Cough with expectoration x 18-20 years

• Chronic nasal congestion x 18-20 years

•Shortness Of Breath x 5-6 years

(With aggravated symptoms in the last 20 days)

HISTORY OF PRESENT ILLNESS

Since 18-20 years patient has experienced recurrent episodes of productive cough.

Mucopurulent sputum in copious amounts, greenish in colour, foul smelling, increased in morning without seasonal variation.

Accompanied with intermittent haemoptysis, with small amounts of fresh blood in sputum.

Recurrent fever, mild to moderate grade, relieved with medication.

Nasal congestion, chronic in nature, with post-nasal drip and hyposmia, accompanied with headache in periorbital region.

Dyspnea since 5-6 years. Gradual in onset, progressing from MRC grade II to MRC grade IV in this time period.

No history of smoking, chest pain, orthopnea, PND, hearing loss, vomiting, diarrhoea, weight loss.

DRUG HISTORY : Use of inhalers and frequent antibiotic therapy

PERSONAL HISTORY : Married since 25 years, failure to conceive even after treatment.

EXAMINATION Patient was conscious and oriented.

Respiratory Rate : 24/min , BP : 124/70 mm Hg, Pulse Rate : 90/min, regular.

Clubbing Grade III

Bilateral diffused Rhonchi

CVS : Apex beat at right 5th intercostal space.

ENT : Nasal congestion, erythematous mucosa with edema.

*Rest of the systemic examination unremarkable.

RESPIRATORY SYSTEM : Patient tachypneic. Bilateral coarse crepitations in the infrascapular,

infraaxillary and inframammary regions.

Palor⁻/Icterus⁻/Cyanosis⁻/JVPNR

Lymphadenopathy⁻/Clubbing⁺/

Pedal Edema⁻

INVESTIGATIONS

CHEST XRAY• Dextrocardia• Consolidation

CT ABDOMEN• Descending Aorta

towards right• Left-sided Liver

Normal CT Abdomen

ULTRASONOGRAPHY• Spleen – R• Liver – L• Gall Bladder – L

(Situs Inversus Totalis)

CHEST CT• Multiple cystic cavitary

lesions having “signet ring” appearance

• Characteristic tram-track sign due to bronchial wall thickening.

• Fibrotic changes• Loss of volume of left

lung

Findings confirming BRONCHIECTASIS

CT PARANASAL SINUSES• Mucosal hypertrophy

almost obliterating nasal passage

• Opacification of ethmoidair cells

INDICATIVE OF SINONASAL DISEASE

PULMONARY FUNCTION TESTSFEV1/FVC ratio reduced to 55% indicative of moderate airflow obstruction

ARTERIAL BLOOD GASPO2 reduced to 47.4 mmHg indicating Hypoxemia(normal : 80-100 mmHg)

Left sided ECG shows poor R wave progression

R wave progression becomes normal when chest leads are right sided consistent with dextrocardia

DIAGNOSIS Patient presented with a triad of symptoms :

• Bronchiectasis

• Chronic Sinusitis

• Situs Inversus Totalis

In addition, her history strongly suggests infertility, which corroborates our diagnosis of Kartagener’s syndrome

After investigations we can also conclude

Moderate airway obstruction

Respiratory failure Type 1 (Hypoxia without hypercapnia)

Characteristic of Kartagener’ssyndrome

DISCUSSION

Kartagener’s syndrome is a subset of Primary CiliaryDyskinesia (PCD) an autosomal recessive disorder related to abnormal cilia structure and function.

Patients typically present in childhood but this may be delayed due to slow progression.

MANAGEMENT MEDICAL MANAGEMENT

• Antibiotic therapy for recurrent infections. Prophylactic long term, low dose antibiotics may be prescribed.

• Vaccination against Influenza A and pneumococcus.

• Inhaled bronchodilators and mucolytic for obstructive lung disease and nasal decongestants for sinusitis.

• O2 inhalation at the rate of 2L/min for hypoxemia.

SURGICAL MANAGEMENT

• IVF to treat infertitlity

• Lung transplantation in severe cases