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Clinical Journal of Gastroenterology

, Volume 11, Issue 3, pp 217–219 | Cite as

Laparoscopic fenestration for a huge symptomatic splenic cyst in a patient with Gaucher’s disease

  • Kyohei Abe
  • Hiroaki Shiba
  • Junichi Shimada
  • Shinji Onda
  • Taro Sakamoto
  • Katsuhiko Yanaga
Case Report

Abstract

A 34-year-old woman visited our hospital for treatment of a huge splenic cyst with epigastric pain. She had been diagnosed with Gaucher’s disease (Type 1) at 3 years of age and had been receiving enzyme replacement therapy (ERT) from 15 years of age. Abdominal MRI showed a low-intensity area, 30 cm in diameter, with a well-defined border on T1-weighted images. The patient underwent laparoscopic fenestration of the huge splenic cyst for relief of worsening epigastric pain caused by the cyst. The cyst contained 2,500 ml of brownish-red fluid. The patient recovered satisfactorily and was discharged on postoperative day 11. Gaucher’s disease is an extremely rare metabolic disease; only about 6,000 patients are registered in the International Collaborative Gaucher Group Gaucher Registry. An extremely rare case of a huge symptomatic splenic cyst treated by laparoscopic fenestration in a patient with Gaucher’s disease is described.

Keywords

Gaucher’s disease Splenic cyst Laparoscopic surgery 

Notes

Compliance with ethical standards

Conflict of interest

Kyohei Abe, Hiroaki Shiba, Junichi Shimada, Shinji Onda, Taro Sakamoto and Katsuhiko Yanaga declare that they have no conflict of interest.

Human rights

All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent was obtained from all patients for being included in the study.

References

  1. 1.
    Beutler E, Grabowski GA. Gaucher disease. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. Metabolic and molecular bases of inherited disease. New York: McGraw-Hill; 2001. p. 3635.Google Scholar
  2. 2.
    Gaucher PCE. De l’epithelioma primitif de la rate, hypertrophie idiopathique de la rate sans leucemie. Paris: BIUM; 1882 (Academic thesis).Google Scholar
  3. 3.
    Linari S, Castaman G. Clinical manifestations and management of Gaucher disease. Clin Cases Miner Bone Metab. 2015;12:157–64.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Rose JS, Grabowski GA, Barnett SH, et al. Accelerated skeletal deterioration after splenectomy in Gaucher type 1 disease. AJR Am J Roentqenol. 1982;139:1202–4.CrossRefGoogle Scholar
  5. 5.
    Walker W. Splenectomy in childhood: a review in England and Wales, 1960–1964. Br J Surg. 1976;63:36–43.CrossRefPubMedGoogle Scholar
  6. 6.
    Salky B, Kreel I, Gelernt I, et al. Splenectomy for Gaucher’s disease. Ann Surg. 1979;190:592–4.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2018

Authors and Affiliations

  • Kyohei Abe
    • 1
  • Hiroaki Shiba
    • 1
  • Junichi Shimada
    • 1
  • Shinji Onda
    • 1
  • Taro Sakamoto
    • 1
  • Katsuhiko Yanaga
    • 1
  1. 1.Department of SurgeryJikei University School of MedicineTokyoJapan

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