PMC7530213_01
Electron microscopic image (magnification: 15.000x) with cross section of a collagen fibre bundle in the reticular dermis: multiple collagen fibrils show ultrastructural abnormalities, such as variable diameter, irregular shape and differing electron density.
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The diagnosis of EDS classical subtype had been previously confirmed by electron microscopy (Fig. 1), biochemical collagen analysis and whole exome gene analysis, revealing mutations in COL5A1 and COL5A2.
Intraoperative photographs of abdominoplasty with two-layered plication of the rectus aponeurosis in the midline and onlay VIPRO-mesh implantation. In a), the midline, medial and lateral borders of the rectus muscle are marked with blue lines. White arrows indicate the medial and blue arrows the lateral aponeurotic plication. In b), the medial plication is shown in the lower abdomen, while the medial and lateral plication have already been completed in the upper abdomen. In c), the outer circumference of the VIPRO-mesh covering the rectus and external oblique abdominal muscles is marked by a blue dotted line.
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Preoperative blood and coagulation test were normal and a standard abdominoplasty with umbilical transposition, extended two-layered plication of the rectus aponeurosis in the midline and onlay VIPRO-mesh implantation covering the oblique and rectus abdominis muscles from the xiphoid to symphysis was performed (Fig. 2).
Abdominal MRI with T2- and T1-weighted, no fat suppression images. In a) and c), the upper lumbar muscle defect with herniation of the visceral fat pad is marked with blue arrows (Grynfellt hernia). In b), the lower lumbar muscle defect is marked with blue arrow (Petit hernia). In d), a stable midline repair following rectus abdominis muscle plication and mesh implantation is visible. The blue arrow marks a defect at the lateral border of the rectus abdominis muscle as an incidental finding with no clinical symptoms.
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There was also an interruption of the fascia between the atrophic latissimus dorsi muscle and the obliquus externus abdominis muscle (Petit hernia) with a gap of several centimeters (Fig. 3).
Intraoperative photographs of lumbar hernia repair. In a), the lumbar muscle defect between the latissimus dorsi muscle (LD) and external oblique abdominis muscle (OA) is marked. In b), the abdominal muscles are deflected with clamps and the upper and lower lumbar defects (marked in blue) are exposed. In c), the defects are covered with VIPRO-mesh and in d), the muscles are sutured over the mesh in a double layer.
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Because the hernias were not detectable laparoscopically, open repair of the superior and inferior lumbar hernias with insertion of a mesh was performed (Fig. 4).
Postoperative photographs 8 years after the abdominoplasty with rectus muscle plication and onlay VIPRO-mesh implantation and 1 year after lumbar hernia repair. The frontal (a), oblique (b) and lateral (c) views demonstrate a stable, long-lasting abdominal wall repair and inconspicuous scar formation on the lower abdomen and right flank.
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Healing was uneventful and the patient reported complete and persistent relief of lumbar pain and recaptured full elevation of the right arm above shoulder level at 1 year follow-up (Fig. 5).