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Weekly Chest CasesArchive of Old Cases

Case No : 170 Date 2001-01-27

  • Courtesy of Kyungneoyh Jeon, M.D., Duk-Sik Kang, M.D. / Kyungpook National University Hospital, Taegu, Korea
  • Age/Sex 17 / F
  • Chief ComplaintExertional dyspnea for 1 week. She has a past history of seizure.
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Tuberous sclerosis
Radiologic Findings
High-resolution CT at the level of aortic arch and ventricles shows multiple thin-walled, rounded cysts with random distribution. The lung parenchyma between the cystic spaces looks normal.
Postcontrast CT scan at upper abdomen demonstrates bilateral renal masses with fatty components, diagnostic of angiomyolipoma. Multiple aneurysms are also found within left renal mass.
At brain MRI, cortical tubers are noted (not presented here).
Brief Review
Tuberous sclerosis is a neurocutaneous syndrome with a wide variety of clinical, pathologic, and radiologic manifestations.
It is characterized by a unique array of multiorgan hamartomatous involvement.
The most frequently involved organs are the skin, brain, retina, lungs, heart, skeleton, and kidneys.
Inheritance is autosomal dominant with a high penetrance and variable expressivity.

Significant pulmonary findings occur in less than 1% patient with tuberous sclerosis.
On pathologic study the lungs show perivascular smooth muscle proliferation and small adenomatoid nodules.

Principal HRCT findings are multiple thin-walled cysts distributed at random in all parts of the lungs.
Cysts are usually less than 20 mm in diameter and are rounded. Lung tissue between cysts appears normal.
The cause of these cysts is unclear, but some investigators have postulated that they result from air trapping.
The clinical, pathologic, and radiologic features are very similar to those of pulmonary lymphangiomyomatosis.

Presenting symptoms include exertional dyspnea, spontaneous pneumothorax, and hemoptysis.
Eighty-four percent of patients with tuberous sclerosis and pulmonary findings are female, with average age at presentation of 34 years. Eighty-six percent of these patients will die of a respiratory cause.
References
1. Webb WR, Muller NL, Naidich DP. High resolution of the lung. 2nd ed. Philadelphia; Lippincott 1996: 231-234
2. Stern EJ, Webb WR, Golden JA, Gamsu G. Cystic lung disease associated with eosinophilic granuloma and tuberous sclerosis: Air trapping at dynamic ultrafast high-resolution CT. Radiology 1992; 182: 325-329
3. Bell DG, King BF, Hattery RR, et al. Imaging characteristics of tuberous sclerosis. AJR 1991; 156: 1081-1086
Keywords
Lung, Multiple organ, Interstitial lung disease, Tuberous sclerosis, ILD,

No. of Applicants : 59

▶ Correct Answer : 59/59,  100.0%
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  • - 11th Air Force Hospital Byung-June Jo
  • - AZ vesalius Tongeren, Belgium Rudi Stokmans
  • - CHU Nancy-Brabois France Denis Regent
  • - Dong-A university Hospital Ki-Nam Lee
  • - Gospel Hospital Kosin University Kyung Hwa Jung
  • - Matsuyama Red Cross Hospital, Matsuyama, Japan Shunya Sunami
  • - Ospedale di Fabriano, Italy Giancarlo Passarini
  • - Pol. S.Orsola-Malpighi, Bologna, Italy Maurizio Zompatori
  • - Santa Maria delle Grazie Hospital-Pozzuoli, Naples, Italy Bianca Cusati
  • - Seoul National University Hospital Tae Jung Kim
  • - Sri Gokulam Hospitals, Salem, India Rajendran Vilvendhan
  • - Stedelijk OLV Ziekenhuis Mechelen, Belgium Ivan Pilate
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