Absence of abdominal muscles syndrome (Orbinsky Syndrome) is a condition characterized by underdeveloped abdominal muscles, often accompanied by malformations in the limbs, urinary system, gastrointestinal tract, and heart.

bubble_chart Clinical Manifestations

The main clinical manifestations include an abdominal wall as thin as paper, with internal organs faintly visible and more distinct upon palpation. Patients cannot transition from lying to sitting without using their arm strength. Insufficient abdominal pressure during coughing often leads to respiratory infections. The skin below the ribs appears red. In incomplete cases, the abdominal wall is normal, presenting only with bilateral cryptorchidism and urinary tract malformations.

Urinary tract malformations may manifest as: ① Complete urethral obstruction, leading to renal failure shortly after birth. Even with surgical urinary tract drainage, oliguria persists, resulting in death from uremia within a short period. ② Severe structural and functional damage to the upper urinary tract, with rapid progression of uremia. Emergency renal or ureteral stoma drainage is performed, revealing significantly dilated ureters lacking peristalsis during surgery. ③ Mild urinary tract obstruction, with symptoms not severe during childhood, allowing for surgical correction, but conservative treatment has an 80% failure rate.

bubble_chart Treatment Measures

Internal medicine treatment

primarily focuses on preventing respiratory tract infections and urinary tract infections.

Surgical treatment

1. Reconstruct the abdominal wall, which can be achieved through abdominoplasty, but currently, abdominal binders are more commonly used for fixation.

3. For patients with urinary tract malformations, surgical correction can improve kidney function. In cases of renal agenesis or severe uremia, kidney transplantation may be considered.

4. For grade I upper urinary tract changes with significant lower urinary tract obstruction due to sexually transmitted disease-related conditions such as urethral valves or urethral strictures, simple surgical intervention to remove the lesion may suffice.

5. Reconstructive surgery of the urinary system.

6. In cases of massive hydronephrosis and ureteral dilation where the ureter and bladder have completely lost function, intestinal ureter replacement and abdominal wall stoma creation may be performed.