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Full thickness skin graft neovagina: an alternative in Mayer-Rokitanski-Hauser syndrome and pelvic kidney

Mila Torii Corrêa Leite, Camila Girardi Fachin, Renato Frota de Albuquerque Maranhão, Márcia Emília Francisco Shida, José Luiz Martins

Pediatric Surgery Department, Federal University of São Paulo, Brazil


 

Correspondence

Mila Torii Corrêa Leite, MD, PhD
Rua Bagé, 230 Apto 113 C
04012-140 Vila Mariana São Paulo-SP, Brazil
Tel :+ 55 11 5581-1490
Cel : + 55 11 9114-4663
Fax : +55 11 5576-4337
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Abstract

The Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is the most common cause of vaginal absence and is frequently associated with malformations of the urinary tract. We report a case of a MRKHS with a right solitary pelvic kidney which prevented a sigmoid or ileal vaginoplasty. A full thickness skin graft neovagina was performed. Follow-up revealed no stenosis with regular dilatations and nighttime stenting. The full thickness skin graft neovagina can be an alternative with little complications when malformations of the urinary tract impede a sigmoid vaginoplasty

Key words: vagina, urogenital abnormalities, reconstructive surgical procedures

 

INTRODUCTION

The Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is the most common cause of vaginal absence. It occurs in about 1 per 5,000 female births and is frequently associated with malformations of the urinary tract [1]. We report a case of MRKHS with solitary pelvic kidney which prevented a sigmoid vaginoplasty.

CASE REPORT

The patient was 16 years old, 46,XX karyotype, with MRKHS (diagnosed during an investigation for abdominal pain when she was 12 year old) and a right solitary pelvic kidney without previous infections (fig. 1). She had the general physical and psychological appearance of a normal young woman.

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Figure 1: MRI reveling a right solitary pelvic kidney

A Pfannestiel incision laparotomy was performed which revealed normal ovaries and an atresic uterus (which was resected). A short pedicullated segment of sigmoid colon was isolated for the vaginoplasty but it didn’t reach the perineum because of the presence of the solitary kidney in the pelvic cavity. We could not use the ileum by the same reason.

A 10 X 5 cm2 full thickness skin graft was harvested from the groin, defatted, tubularized and placed into a tunnel created bluntly between the bladder and rectum (fig. 2). A silicone mold was left for 14 days. The dilatations were done first weekly and, after six months, every 15 days. She used the silicone mold daily for one year and then only during the night. In a year and a half follow-up, the neovagina was 10 cm in depth with no stenosis, and biopsies revealed vaginal mucosa (fig. 3).

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Figure 2: The tubularized full thickness skin graft (a) was placed into a tunnel created bluntly between the bladder and rectum (b).

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Figure 3: Final surgical aspect with an excellent cosmetic result.

DISCUSSION

The most common cause of congenital absence of the vagina is the Mayer-Rokitansky- Kuster-Hauser syndrome, estimated to occur in 1/4000 to 5000 births. The diagnosis is often made during adolescence in primary amenorrhea with normal puberty. The uterus is absent or hypoplastic and the ovaries are normal. The cause of MRKHS is unclear and is frequently associated with malformations of the urinary tract (40%) and skeletal system (10%) [1].

Numerous techniques for vaginal reconstruction have been described. Rudimentaries vaginas can be treated with non operative dilatations (Frank technique) but require strict follow-up. Perineal cleavage covered with a split-thickness skin graft described as Abbe- McIndoe procedure is frequently used by gynecologists. Despite a good functional result,this procedure requires lubrication and periodic dilatation [2].

The sigmoid vaginoplasty is considered by many the best choice for vaginal replacement in patients with the Mayer-Rokitansky-Kiister- Hauser syndrome, avoiding contraction, shrinkage, narrowing or stenosis at the introitus. It results in a self-lubricant neovagina and requires temporary dilatations and irrigations [3,4].

Intestinal vaginoplasty is our preference to treat MRKHS, but the solitary pelvic kidney prevented the pediculated segment of sigmoid colon to reach the perineum. Then, we performed a full thickness skin graft neovagina without complications with regular dilatations and nighttime stenting.

Malformations of the urinary tract are frequently associated with this syndrome, so the knowledge of the various techniques of vaginal reconstruction is very important to propose alternatives in cases like this one.

Despite studies in split-thickness skin graft showing that it takes months or sometimes years until the graft loses all of its skin properties and adapts to its new environment, the neovaginal epithelium of the full thickness skin graft microscopically was identical to normal vaginal mucosa, in a year in a half after surgery [5].

CONCLUSIONS

The full thickness skin graft neovagina can be an alternative with little complications when malformations of the urinary tract prevent a sigmoid or ileal vaginoplasty. Further studies are necessary to determine the mechanism by which the skin graft developed into vaginal mucosa.

 

 

 

References

1. Ludwig KS. The Mayer-Rokitansky-Kuster syndrome. An analysis of its morphology and embryology. Part II: Embryology. Arch Gynecol Obstet 1998; 262: 27-42.

2. Klingele CJ, Gebhart JB, Croak AJ et al: McIndoe procedure for vaginal agenesis: long-term out-come and effect on quality of life. Am J Obstet Gynecol 2003; 189: 1569-72.

3. Khen-Dunlop N, Lortat-Jacob S, Thibaud E, et al. Rokitansky Syndrome: Clinical Experience and Results of Sigmoid Vaginoplasty in 23 Young Girls. J Urol.2007; 177:1107-11.

4. Del Rossi C, Aattanasio A, Domenichelli V et al. Treatment of the Mayer-Rokitansky-Koster-Hauser Syndrome in bangladesh: results of 10 total vaginal replacements with sigmoid colon at a missionary hospital. J Urol.1999; 162:1138-40.

5. Bekerecioglu M, Balat O, Tercan M, et al. Adaptation process of the skin graft to vaginal mucosa after McIndoe vaginoplasty. Arch GynecolObstet . 2008, 277:551-4.