Female pelvic congenital malformations. Part I: embryology, anatomy and surgical treatment

This review covers the most important female congenital pelvic malformations. The first part focuses on the embryological development of the urogenital and anorectal apparatus, morphological features, and the diagnostic and surgical approach to abnormalities.Comprehension of the embryological development of the urogenital and anorectal apparatus is essential to understand the morphology of congenital pelvic abnormalities and their surgical treatment.Congenital pelvic malformations are characterized by specific common features; the severity of which often subverts the pelvic morphology completely and makes it difficult to comprehend before surgery. The development of imaging, mainly magnetic resonance imaging and ultrasound, in the investigation of pelvic floor disorders has recently become a fundamental tool for surgeons to achieve better understanding of the anatomy.Forty years ago, the primary aim of clinicians was to save the lives of such patients and to achieve anatomical normality. However, nowadays, functional reconstruction and recovery are essential parts of surgical management. Introduction of minimally invasive surgery has allowed the improvement of cosmetic results that is so important in paediatric or adolescent patients after reconstructive surgery.The option of sharing the complexity of pelvic congenital diseases by entrusting specific competencies to subspecialists (paediatric urologists, urogynaecologists, neurourologists, paediatric endocrinologists and neonatologists) has improved the quality of care for patients. However, at the same time, active interaction between various specialists remains fundamental. The exchange of knowledge and expertise, not only during the diagnostic-therapeutic process but also during follow-up, is crucial to obtain the best anatomical and functional results throughout the life of the patient.     

Uterine arteriovenous malformations: diagnosis and treatment in 2011

Uterine arteriovenous malformations (UAVM) are rare. They mostly occur after endo-uterine trauma, or are less often congenital. When symptomatic, they may be a cause of uterine recurrent and massive bleeding. Diagnosis should be evoked in these cases, to avoid haemostatic curettage which will be useless and injurious. UAVM is often suspected by Doppler ultrasound, but pelvic MRI seems to be also relevant. Angiography confirms the diagnosis and allows concomitant embolization. Uterine embolization seems to be currently the best treatment, however surgery should still be performed in case of failure or hemodynamic instability. In this work, we aim to evaluate diagnosis and therapeutic modalities for UAVM.     

Surgical treatment of malformations of the uterus.

The reproductive history in 13 patients with uterine malformations was reviewed. Following plastic repair, there was a high incidence of pregnancy salvage. Of 19 pregnancies, 14 resulted in term deliveries, four in premature deliveries and one in abortion; there are 14 living children. The surgical repair in two patients with primary sterility was the contributing factor in achieving pregnancies with full term deliveries.     

Surgical treatment of adnexal masses in pregnancy: indications, surgical approach and pregnancy outcome

OBJECTIVE: To analyze the indications, surgical approach and pregnancy outcome in women undergoing surgical treatment of adnexal masses in pregnancy. STUDY DESIGN: In this retrospective study, 51 women were enrolled. Preoperative sonographic appearance, the size of the tumor, patient's age, gestational age at the time of surgery and pregnancy outcome following a laparoscopic and/or laparotomy approach were evaluated. The data were obtained from the National Perinatal Informational System, National Cancer Registry, inpatient records, operative reports and pathology records, and responses to a questionnaire on pregnancy outcome mailed to all the patients. RESULTS: Of the 51 pregnant women with adnexal masses, 27 were treated laparoscopically and 24 through laparotomy. Acute symptoms were the indication in 14 (27.4%) and tumor > 5 cm or sonographic appearance in 37 (72.6%). There were 44 (86.4%) benign and 7 (13.6%) malignant masses (4 borderline and 3 cancers). There were no differences in pregnancy outcome between emergency and planned surgery or between laparoscopy and laparotomy. CONCLUSION: Surgical treatment of persistent adnexal masses in pregnancy, particularly those with a sonographic appearance of a complex tumor, is justified because of the high risk of torsion, rupture and malignancy. Immediate treatment of symptomatic masses permits conservative, fertility-preserving surgery and has no adverse effect on pregnancy outcome. Laparoscopic surgery and surgery in the first trimester do not impair pregnancy outcome.     

Surgical treatment of uterine malformations]

Clinical data of 29 patients with uterine malformation treated at the Fertility Unit, Service and Department of Obstetrics and Gynecology, Dr. G. Grant B. Clinical Hospital, Concepción Chile from January 1978 to May 1990 are presented. Uterine septa (19 cases, 65.5%) were the most common uterine malformation found. Less frequent were: uterus bicornuous 7 cases (24.1%) and uterus didelphis 3 cases (10.4%). The high frequency (92.8%) of pregnancy wastage is detached. Surgical treatment was practiced in 24 patients. Thirteen Tompkins and 5 Jones techniques were performed in 18 uterine septa. Five Strassman techniques and one extirpation of a rudimentary not communicated horn were practised in the 6 cases with uterus bicornuous. In patients with adequate follow-up, the overall term birth rate after metroplasty is 84.6% compared with only 2.4% before surgical treatment. This study concludes that in despite of the lack of hysteroscopy as a new method of uterine septa treatment our patient's infertility was benefited with surgical treatment of uterine anomalies.     

Uterosacral ligament vaginal vault suspension: anatomy, outcome and surgical considerations

PURPOSE OF REVIEW: With aging populations, primary pelvic organ and recurrent pelvic organ prolapse have become a large-scale public health concern. Surgical options for patients include both abdominal and vaginal approaches, each with its own safety and efficacy profiles. This review summarizes the most recent anatomic, surgical and outcome data for uterosacral ligament vault suspension. It offers data on methods to avoid complications and difficult surgical scenarios. RECENT FINDINGS: Uterosacral ligament suspension allows reattachment of the vaginal vault high within the pelvis. New modifications in technique including the extraperitoneal and laparoscopic approaches allow surgeons more freedom when planning surgery. Five-year data on the durability of the procedure make it a viable surgical option. SUMMARY: As a technique widely used by many pelvic reconstructive surgeons, uterosacral ligament vault suspension provides a safe, anatomically correct and durable approach to uterine and vault prolapse. It requires advanced surgical training and an intimate understanding of pelvic anatomy to avoid and identify ureteral injury.     

Reversal of twin-twin transfusion syndrome: frequency, vascular anatomy, associated anomalies and outcome

OBJECTIVE: To determine the frequency of reversal of transfusional gradient and phenotype in a large cohort of prospectively studied cases of twin-twin transfusion syndrome (TTTS) and seek evidence of clinical or placental anastomotic associations. METHODS: Consecutive cases of TTTS seen over an eight-year period with serial documentation of ultrasonic growth, liquor volume and fetal and placental Doppler studies were reviewed. Postnatal injection studies were inspected. RESULTS: Reversal of TTTS occurred in 5 of 96 affected pregnancies (5%). Two of the five cases had underlying aneuploidy or genetic syndrome, higher than the 2% frequency found in cases without reversal of TTTS (p < 0.05). Placental anastomotic configurations provided no consistent explanation for reversal of phenotype. CONCLUSION: This study documents the frequency of reversal of the direction of TTTS, and suggests that it is a heterogeneous condition. Reversal of donor-recipient phenotype may be explained by haemodynamic changes secondary to underlying aneuploidy/genetic syndromes, to the presence of multiple anastomoses in either direction or following laser ablation. This series together with previous case reports argues for a high level of suspicion for underlying aneuploidy, genetic syndrome or structural defects where there is reversal of the donor-recipient phenotype.     

A syndromal and an isolated form of uterine arteriovenous malformations: two case-reports.

Uterine arteriovenous malformations are rare lesions with a considerable risk potential. Clinical presentation varies from no signs over various degrees of menorrhagia to massive life threatening vaginal bleeding. This is the first report of congenital uterine arteriovenous malformations in two patients with primary infertility. In one case, the uterine lesions were found in conjunction with other congenital malformations suggesting the diagnosis of hemihyperplasia/lipomatosis syndrome. Etiology, symptoms, diagnostic and therapeutic work-up are discussed; pathological findings are illustrated.     

Factors affecting the outcome of surgical treatment of acromegaly.

PURPOSE: To assess the results of and factors associated with the outcome of surgery for acromegaly. METHODS: We retrospectively examined the medical records of acromegalic patients who underwent trans-sphenoidal adenomectomy at our hospital during the period of January 1991 through August 1997. Preoperative evaluations included measurement of basal serum growth hormone (GH), insulin-like growth factor-I (IGF-I), prolactin (PRL), GH response to oral glucose, and GH and PRL response to bromocriptine, as well as pituitary magnetic resonance (MR) imaging. Postoperative evaluations included measurement of basal serum GH and IGF-I concentrations, and pituitary MR imaging. RESULTS: Thirty patients (14 men) with a mean age of 38 years were included. The mean follow-up period was 50 months (range, 15-90 mo). Ten of the 30 patients (33%) had early postoperative (1 mo after surgery) GH levels of less than 5 ng/mL. Twenty patients (67%) had final postoperative (last follow-up, 15-90 mo after surgery) GH levels of less than 5 ng/mL. Preoperative GH levels were positively correlated with early postoperative GH levels (r = 0.458, p = 0.011) and final postoperative GH levels (r = 0.479, p = 0.007). Early postoperative GH levels were also positively correlated with final postoperative GH levels (r = 0.595, p = 0.001). Tumor grade and stage were not significantly correlated with early or final postoperative GH levels. Thirteen of 21 patients (62%) who had postoperative MR imaging follow-up had residual tumor. There was no surgical mortality. CONCLUSIONS: These results highlight that acromegaly is not easily treated with surgery alone. The preoperative GH level was associated with the surgical outcome.     

Embolization of cerebral arteriovenous malformations with n-butyl-2-cyanoacrylate.

PURPOSE: To determine the safety and effectiveness of arteriovenous malformation (AVM) embolization using liquid adhesives in a series of 103 patients with cerebral AVMs who underwent embolization with n-butyl-2-cyanoacrylate (NBCA). METHODS: All embolization procedures were performed using NBCA and a lipiodol mixture delivered by a flow-directed and/or guide-wire directed microcatheter. RESULTS: Using the Spetzler-Martin grading system, there were two cases of grade I AVM, 23 of grade II AVM, 31 of grade III AVM, 37 of grade IV AVM, and 10 of grade V AVM. Eleven patients underwent embolization only; these patients either had evidence of morphologic cure on follow-up angiogram, or showed evidence of a stable condition at 2-years follow-up. Seven patients were not treated owing to technical problems or a positive functional test. All other patients underwent preoperative embolization; 75%-99% obliteration was noted in 39 of these patients, 50%-74% obliteration in 33, and less than 50% obliteration in 13. Embolization-related complications occurred in nine patients, and two patients died as a result of the procedure. Severe neurologic complications occurred in two patients and five patients developed mild or transient neurologic deficit. CONCLUSIONS: Endovascular treatment of brain AVMs with an NBCA mixture has a lower complication rate than shown in previous studies using other materials. The percentage of obliteration of the nidus is increased because NBCA penetrates AVMs better than polyvinyl alcohol particles. NBCA can be used at a lower concentration than other acrylates, and therefore causes almost no catheter gluing. The embolic mass formed by NBCA is more biocompatable than that formed by other acrylates.     

Uterine arteriovenous malformations: a review of the current literature

Uterine arteriovenous malformation (AVM) is a rare but potentially life-threatening condition. AVMs often present with intractable bleeding and commonly are seen in association with pregnancy and uterine trauma. Ultrasound is the most common form of initial investigation, and computed tomography and magnetic resonance imaging are being used with greater frequency. Despite this, angiography remains the gold standard for diagnostic evaluation. Embolization has become a more acceptable form of treatment and allows more invasive forms of treatment, particularly hysterectomy, to be avoided. Numerous medical therapies have also been used in the management of patients with uterine AVM. Reports of successful pregnancies after diagnosis and treatment of a uterine AVM are still uncommon, but increasingly good outcomes are being reported after successful treatment of a confirmed uterine AVM. Target Audience: Obstetricians & Gynecologists, Family Physicians Learning Objectives: After completion of this article, the reader should be able to describe the many and varied clinical manifestations of a uterine arteriovenous malformation (AVM), summarize the best ways to manage an acute hemorrhage from an AVM, and identify the current best way to diagnose an AVM.     

Reproductive outcome after surgical treatment of ectopic pregnancy

OBJECTIVES: Reproductive outcome after laparoscopy or laparotomy performed for treatment of ectopic pregnancy. METHODS: Data of 104 women were collected by a questionnaire and from medical records. RESULTS: No differences in pregnancy rate were found comparing the two different operative procedures. Women with a normal contralateral fallopian tube had a better pregnancy rate compared with those with a damaged contralateral tube. CONCLUSIONS: The crucial factor for future reproductive outcome after surgical treatment of ectopic pregnancy is the status of the contralateral tube.     

Pregnancy outcome after female infertility in Kuwait. Comparison of medical and surgical treatment

During a 4-year period, 163 patients conceived after treatment of their infertility due solely to a female factor. They were divided into two groups, 108 patients treated medically (Group 1) and 55 treated surgically (Group 2). At the time of conception, the mean age in Group (1) was 24.9 +/- 5.7 years while in Group (2) it was 31.2 +/- 6.1 years. The difference was statistically significant (p less than 0.01). The duration of infertility prior to pregnancy and the treatment-to-pregnancy interval were both significantly longer in Group (2) (p less than 0.05). The rates of miscarriage, multiple pregnancy, and preterm labor were higher in Group (1), though the differences were statistically not significant. In contrast, the rate of ectopic pregnancy was significantly higher in Group (2) (p less than 0.05), as was the need for elective cesarean section (p less than 0.01). Despite these differences, both groups had nearly the same perinatal outcome.