Laparoscopic sacral hysteropexy for pelvic organ prolapse in a patient affected by marfan syndrome: a case report

Background Marfan Syndrome (MS) is a dominantly inherited connective tissue disorder with consequences on the strength and resilience of connective tissues that may predispose to Pelvic Organ Prolapse (POP). Literature lacks studies investigating POP surgery in patients affected by MS that might help surgical management decisions. Objective The objective of this paper is to describe the surgical procedure of laparoscopic sacral hysteropexy (LSHP) in a 37 years old woman affected by MS with symptomatic POP. Materials and Methods and main outcome measures We performed a nerve-sparing laparoscopic sacral hysteropexy without complications and looked for anatomical and subjective outcomes. The patient completed The Female Sexual Distress Scale (FSDS), Pelvic Floor Disability Index (PFDI-20), and Wexner questionnaires preoperatively and postoperatively. Results The patient stated a complete resolution of all POP related symptoms and there was a total correction of the descensus. Furthermore, no perioperative and postoperative complications were noted. Conclusions LSHP could be an effective and safe procedure for the treatment of POP in women affected by MS and this case report is the first to describe a reconstructive procedure in this category of patients. What is new? The literature lacks studies investigating POP surgery in women with MS, that might help surgeons, thus we present this case to describe surgical and functional outcomes in this patient category, underlying the higher risk of complications and relapses related to the weakness of connective tissue. This case report may represent the basis of future studies to confirm the safety, efficacy and feasibility of LSHP and sacral colpopexy in patients with MS.

[1]  G. Scambia,et al.  Laparoscopic sacral hysteropexy versus laparoscopic sacral colpopexy plus supracervical hysterectomy in patients with pelvic organ prolapse , 2021, International Urogynecology Journal.

[2]  S. Brucker,et al.  Absorbable versus non-absorbable sutures for vaginal mesh attachment during sacrocolpopexy: a randomized controlled trial , 2021, International Urogynecology Journal.

[3]  G. Scambia,et al.  Laparoscopic sacrocolpopexy plus ventral rectopexy as combined treatment for multicompartment pelvic organ prolapse , 2020, Techniques in Coloproctology.

[4]  G. Scambia,et al.  Minimally invasive surgery in urogynecology: a comparison of standard laparoscopic, minilaparoscopic, percutaneous surgical system and Robotic sacral colpopexy. , 2020, Minerva medica.

[5]  C. Iglesia,et al.  Hydronephrosis Associated With Pelvic Organ Prolapse: A Systematic Review. , 2020, Female pelvic medicine & reconstructive surgery.

[6]  M. Emanuelli,et al.  Expression of extracellular matrix and adhesion proteins in pelvic organ prolapse. , 2018, Cellular and molecular biology.

[7]  G. Scambia,et al.  Laparoscopic supracervical hysterectomy and sacral colpopexy for pelvic organ prolapse with percutaneous surgical system: Results from a pilot study. , 2018, European journal of obstetrics, gynecology, and reproductive biology.

[8]  Helen Jefferis,et al.  Pregnancy following laparoscopic hysteropexy—a case series , 2017, Gynecological Surgery.

[9]  J. Rosenberg,et al.  Increased Need for Gastrointestinal Surgery and Increased Risk of Surgery-Related Complications in Patients with Ehlers-Danlos Syndrome: A Systematic Review , 2016, Digestive Surgery.

[10]  J. Janis,et al.  Collagenopathies—Implications for Abdominal Wall Reconstruction: A Systematic Review , 2016, Plastic and reconstructive surgery. Global open.

[11]  G. Scambia,et al.  Anatomical insights into sacrocolpopexy for multicompartment pelvic organ prolapse , 2016, Neurourology and urodynamics.

[12]  A. Child,et al.  Genitourinary Tract in Women with Marfan Syndrome , 2016 .

[13]  Z. Kamalak,et al.  Is There a Relationship Between Pelvic Organ Prolapse and Tissue Fibrillin-1 Levels? , 2015, International neurourology journal.

[14]  K. Kluivers,et al.  Risk factors for pelvic organ prolapse and its recurrence: a systematic review , 2015, International Urogynecology Journal.

[15]  S. Jackson,et al.  Laparoscopic hysteropexy: a novel technique for uterine preservation surgery , 2013, International Urogynecology Journal.

[16]  A. Malmström,et al.  Decreased gene expression of fibrillin‐1 in stress urinary incontinence , 2010, Neurourology and urodynamics.

[17]  M. Barber,et al.  Short forms of two condition-specific quality-of-life questionnaires for women with pelvic floor disorders (PFDI-20 and PFIQ-7). , 2005, American journal of obstetrics and gynecology.

[18]  H. Dietz,et al.  Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology. , 2004, Physiological genomics.

[19]  M. Barber,et al.  Adoption of the pelvic organ prolapse quantification system in peer-reviewed literature. , 2003, American journal of obstetrics and gynecology.

[20]  L. Derogatis,et al.  The Female Sexual Distress Scale (FSDS): Initial Validation of a Standardized Scale for Assessment of Sexually Related Personal Distress in Women , 2002, Journal of sex & marital therapy.

[21]  J. Schaffer,et al.  Urinary incontinence and pelvic organ prolapse in women with Marfan or Ehlers Danlos syndrome. , 2000, American journal of obstetrics and gynecology.

[22]  J. Fleshman,et al.  Fecal incontinence quality of life scale , 2000, Diseases of the colon and rectum.

[23]  M. Mclaren,et al.  Endothelial Cell and Platelet Function in Marfan's Syndrome , 1993 .

[24]  L. Sandberg,et al.  Structural features of tropoelastin related to the sites of cross-links in aortic elastin. , 1971, Biochemistry.

[25]  K. Nath,et al.  Marfan's syndrome. , 1959, Journal of the Indian Medical Association.