Surgical treatment of Jeune syndrome in a child (rare clinical case and review)

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Abstract

Objective: possibility of surgical treatment of chest deformity in children with Jeune syndrome (analysis of the clinical case), review of literature on Jeune syndrome in children.

Methods: clinical, radiological, laboratory, instrumental, statistical.

Results: Positive outcome of surgical treatment of a 3-year-old patient with severe chest deformity and severe respiratory failure.

Conclusions: Jeune syndrome is a severe disease with a high mortality rate. A multidisciplinary approach is required in the treatment of patients with this pathology. Surgical intervention aimed at reducing the compression of the chest organs and increasing its volume, even in conditions of decompensation, allows you to stop the phenomenon of respiratory failure.

About the authors

Igor A. Komolkin

St. Petersburg State Research Institute of Phthisiopulmonology; St. Petersburg State Pediatric Medical University

Email: igor_komolkin@mail.ru
ORCID iD: 0000-0002-0021-9008

MD, PhD, Dr. Sci. (Med.)

Russian Federation, Saint Petersburg; Saint Petersburg

Aleksandr Yu. Mushkin

St. Petersburg State Research Institute of Phthisiopulmonology

Email: aymushkin@mail.ru
ORCID iD: 0000-0002-1342-3278

MD, PhD, Dr. Sci. (Med.), Professor

Russian Federation, Saint Petersburg

Olga E. Agranovich

H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery

Email: olga_agranovich@yahoo.com
ORCID iD: 0000-0002-6655-4108

MD, PhD, Dr. Sci. (Med.)

Russian Federation, Pushkin, Saint Petersburg

Ardan P. Afanasiev

N.N. Priorov National Medical Research Center of Traumatology and Orthopedics

Author for correspondence.
Email: afanasevap@cito-priorov.ru
ORCID iD: 0000-0002-5089-2524

MD, PhD, Cand. Sci. (Med.)

Russian Federation, 10 Priorova str., Moscow, 127299

References

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Supplementary files

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2. Fig. 1. CT scan of patient M. with Jeune syndrome (type А2S0F1cor, br). Axial section at the top of the deformity: a — before treatment (HCCI = 60.1%); b — sternum is narrow, segmented, and short; ribs are wide and flat with an abnormal structure of the growth zones, and “wine glass” extensions of the anterior areas (shown by the arrow); c — fibrotic atelectases in the lungs (shown by the arrow) in the area of compression by the chest framework of the broncho-pulmonary complex (See the text for the details)

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3. Fig. 2. Calculation of an arbitrary lung volume: a — 3D-MVR in the front-rear projection of the lungs; b — 3D-MVR in lateral projection of the lungs (See the text for the details)

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4. Fig. 3. Axial sections of CT scans (a) and a 3D-CAT reconstruction (b) after the I stage of surgical treatment: a-HCCI = 71.8%: the right hemithorax has the corrected shape (the position of one of the rib plates is marked with an arrow); c - 3D multi-volume lung reconstruction after the I stage of surgical treatment (See the text for the details).

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5. Fig. 4. Axial sections of CT scan (a), 3D-CAT scan (b), frontal chest 3D-CAT (c), and lung 3D-MVR (d) after the II stage of the surgery (See the text for the details)

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