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Weekly Chest CasesArchive of Old Cases

Case No : 997 Date 2016-12-05

  • Courtesy of Ye Kyeong Jun, Hyun Jin Park , Jeong Min Ko / St. Vincent's Hospital, The Catholic University of Korea
  • Age/Sex 18 / F
  • Chief ComplaintLeft flank pain after trauma
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Tuberous sclerosis complex
Radiologic Findings
Chest CT scan with lung window setting shows multiple small solid and ground glass opacity nodules in both lungs. On mediastinal window setting images, there are a few fatty foci in the cardiac ventricular septum and left ventricular wall.
Brain CT and MRI revealed subependymal nodules and cortical/subcortical tubers. Abdominal CT revealed multiple renal angiomyolipomas.
Brief Review
Multifocal micronodular pneumocyte hyperplasia (MMPH) is a rare pulmonary disorder that can be associated with tuberous sclerosis complex. MMPH can occur in patients with or without lymphangioleiomyomatosis (LAM), predominantly in female patients. At CT, multiple tiny nodular opacities (1–8 mm in diameter) are diffusely scattered throughout the lungs in a random distribution. Although differentiation of MMPH from other diseases with nodular opacities is difficult, MMPH should be considered in the differential diagnosis when multiple tiny pulmonary nodules are present in patients with tuberous sclerosis.
Congenital rhabdomyomas, which occur during infancy and typically regress spontaneously during early childhood, are known as typical cardiac manifestation of tuberous sclerosis. Recently, myocardial fatty foci are increasingly being thought of as another characteristic of tuberous sclerosis complex.
References
1. Franz DN, Brody A, Meyer C, et al. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med 2001; 164:661-668.
2. Ristagno RL, Biddinger PW, Pina EM, Meyer CA. Multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis. AJR Am J Roentgenol 2005; 184:S37-39.
3. Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M. Fatty foci in the myocardium in patients with tuberous sclerosis complex: common finding at CT. Radiology 2009; 253:359-363.
Keywords
Lung, Interstitial lung disease,

No. of Applicants : 81

▶ Correct Answer : 33/81,  40.7%
  • - Saitama-Sekishinkai Hosptal , Japan Mihoko Yamazaki
  • - Otsu Municipal Hospital , Japan Noriatsu Ichiba
  • - Kinki University Faculty of Medicine, , Japan Mitsuru Matsuki
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - King Abdulaziz University Hospital , Saudi Arabia Amr Ajlan
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Korea University Guro Hospital , Korea (South) Bit Na Park
  • - Ajou university hospital , Korea (South) Pae Sun Suh
  • - Korea University Guro Hospital , Korea (South) PARK SOYEON
  • - Centro Hospitalar S. Joao , Portugal Marcio Rodrigues
  • - HUEC , Brazil Diogo Lago Pinheiro
  • - Asan Medical Center , Korea (South) Hyung Jung Koo
  • - The Jikei university , Japan Taku Gomi
  • - university of montreal , Canada Andrei Gorgos I
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - , Korea (South) BAEK YOOLIM
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - University of Tsukuba, Dept of Radiology , Japan Manabu Minami
  • - Ajou university hospital , Korea (South)
  • - Seoul national university hospital , Korea (South) Kumju Chae
  • - Asan Medical Center , Korea (South) Chan Ho Park
  • - Kanto Rosai Hospital , Japan Taku Tajima
  • - Korea university medical center, guro hospital , Korea (South) Lee chong
  • - Ajou University Hospital , Korea (South) Seulgi You
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Korea University Guro Hospital , Korea (South) Jeong Woo Kim
  • - Kyoto University , Japan Akihiko Sakata
  • - Korea University Guro Hospital , Korea (South) Hwanseok Yong
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - Ajou University Hospital , Korea (South) Youngjin Yoo
▶ Correct Answer as Differential Diagnosis : 2/81,  2.5%
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - GHICL , France manuel toledano
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