A rare gynecologic presentation of proteus syndrome: A case report.

Proteus syndrome is a genetic condition with an estimated incidence of less than one in a million. This condition is sporadic and presents as progressive, mosaic overgrowth of different tissues. Clinical manifestations are diverse, with the reported involvement of lungs, skin, blood cells, the nervous system and bones. Gynecologic manifestations have rarely been reported in the literature. This case is the first to be reported in the literature of a woman with Proteus syndrome diagnosed in her prepubertal years and presenting at 34 years old with a cervical mass protruding from the vagina. The patient sought medical intervention only after the prolapse was advanced and symptomatic. Management of this case was surgical and consisted of vaginal hysterectomy, with vaginal suspension.

Perioperative outcomes of robotic hysterectomy with mini-laparotomy versus open hysterectomy for uterus weighing more than 250 g.

To compare perioperative outcomes in patients undergoing robotic hysterectomy and extraction of specimen via mini-laparotomy (RHML) versus open hysterectomy (OH) when uterus weighs more than 250 g. To study the factors determining the length of hospital stay in 2 groups. A retrospective analysis of all hysterectomies performed for uterus weighing more than 250 g from the year 2012 to 2015 was conducted. A total of 140 patients were divided into 2 groups based on the type of surgery; RHML (n = 82) and OH (n = 58). Mini-laparotomy consisted of a customised incision connecting 2 left lateral port sites for specimen extraction after completing the hysterectomy robotically. Patient factors and perioperative outcomes were compared using Student's t tests and Chi-square analysis. Mean length of stay (RHML = 1.4 days; OH = 3.4 days), estimated blood loss (EBL) (RHML = 119.9 ml; OH = 547.5 ml) and operative time (RHML = 191.5 min; OH = 162.8 min) were significantly different. No significant differences were noted for patient BMI, age, comorbidities, intraoperative complications, pathology of uterus and uterus weight. Postoperative complications were significantly different between two groups (RHML = 6.0%; OH = 15.5%; p = .021). None of the patients stayed less than 24 h in OH group compared to 59.8% patients in RHML group. Type of procedure (p = .004) and EBL (p = .002) significantly predicted the length of stay. Patients undergoing RHML have significantly shorter length of stay, EBL and postoperative complications than OH. The operative time for RHML was longer than OH, but the overall decreased length of stay overcomes this disadvantage. RHML approach retains the benefits of da Vinci, while simultaneously preserving the specimen.

Robotic-Assisted Laparoscopic Abdominal Cerclage Placement During Pregnancy.

STUDY OBJECTIVE: The objective of this study is to report our center's series of robotic-assisted laparoscopic abdominal cerclage (RALAC) placement during pregnancy. DESIGN: Descriptive study (Canadian Task Force classification III). SETTING: Single academic institution. PATIENTS: Patients undergoing RALAC placement during pregnancy. INTERVENTIONS: Eleven patients underwent RALAC. MEASUREMENTS AND MAIN RESULTS: Nine out of 11 (81.8%) primary RALAC procedures resulted in a viable live-born neonate; 8 (72.7%) were born at >34 weeks of gestation. Three patients (27.3%) had preterm premature rupture of membranes on postoperative day one, 2 of whom subsequently underwent a dilation and curettage, and 1 patient carried the pregnancy to 29 weeks and delivered a live-born neonate. Four patients had subsequent pregnancies after placement of a RALAC in the antepartum period, all of whom carried successfully beyond 36 weeks, for a total of 16 pregnancies. Fourteen pregnancies (87.5%) resulted in a live birth, and 13 pregnancies (81.3%) were delivered beyond 34 weeks. CONCLUSION: RALAC is a minimally invasive procedure with an acceptable risk profile and comparable efficacy to traditional open abdominal cerclage. RALAC may be considered an acceptable alternative to open abdominal cerclage in pregnancy, and may be a particularly favorable option in certain settings.

Nonobstructive Acute Renal Failure with a Large Solitary Fibroid.

A 38-year-old African American woman presenting with acute abdominal pain and nonobstructive renal failure was found to have an enlarged fibroid uterus. A differential for sepsis was considered. Lab evaluation revealed an elevated creatinine and myoglobin level at 3.9 mg/dL and 2140 ng/mL, respectively. Ongoing hemodynamic instability mandated surgery for acute abdomen. A 25 cm fibroid uterus was extirpated through a total abdominal hysterectomy. Immediate improvement of acute nephropathy mirrored the postoperative decline in serum myoglobin levels. Myoglobinemia from a massive degenerating fibroid is associated with nonobstructive acute renal failure.

Additional uses of a vaginal fornices delineator.

A vaginal fornices delineator was introduced in the mid-90s by a gynecologic surgeon named Charles Koh as a device that would facilitate total laparoscopic hysterectomy. It is also used in robot-assisted and traditional laparoscopic hysterectomies. The delineator delineates the vaginal fornices and provides improved visualization of vital structures during a hysterectomy. The determination of the anatomic level of the cervix is vital in the identification of the inferior borders of a total hysterectomy and to avoid injury to the ureters and uterine vessels. As such, we describe 3 gynecologic surgeries in which the identification of this level was suboptimal. Our solution was to utilize a vaginal fornices delineator as the cervical "guide" to enhance this visualization and to allow for a complete and safe surgical outcome. The following surgical scenarios and the surgical facilitation provided by the vaginal fornices delineator will be presented: (1) a postpartum cesarean hysterectomy complicated by a large lower uterine fibroid tumor that distorts the cervix, (2) a robotic-assisted placement of an abdominal cerclage in a first-trimester pregnancy, (3) abdominal hysterectomy complicated by a necrotizing uterine infection and the associated difficulty in the identification of the cervicouterine junction.

Gynecologic Malignancies Post-LeFort Colpocleisis.

Introduction. LeFort colpocleisis (LFC) is a safe and effective obliterative surgical option for older women with advanced pelvic organ prolapse who no longer desire coital activity. A major disadvantage is the limited ability to evaluate for post-LFC gynecologic malignancies. Methods. We present the first case of endometrioid ovarian cancer diagnosed after LFC and review all reported gynecologic malignancies post-LFC in the English medical literature. Results. This is the second reported ovarian cancer post-LFC and the first of the endometrioid subtype. A total of nine other gynecologic malignancies post-LFC have been reported in the English medical literature. Conclusions. Gynecologic malignancies post-LFC are rare. We propose a simple 3-step strategy in evaluating post-LFC malignancies.

Percutaneous tibial nerve stimulation as an off-label treatment of clitoral pain.

OBJECTIVE: Percutaneous tibial nerve stimulation (PTNS) is used to treat refractory urinary frequency, urgency, and urgency urinary incontinence. To date, it is not approved by the US Food and Drug Administration in the treatment of chronic pain syndromes, and its use in the treatment of chronic clitoral pain has not been reported. METHODS: We describe 2 cases of women who presented with symptoms of urgency urinary incontinence, urinary frequency, and clitoral pain. After inadequate response to conservative treatment of their urinary symptoms, they received PTNS. RESULTS: By the 12th session, significant improvement in urinary symptoms and resolution of clitoral pain were noted. CONCLUSIONS: The findings of this report suggest that PTNS may be a therapeutic option in patients with idiopathic clitoral pain.

Dystrophic epidermolysis bullosa in pregnancy: a case report of the autosomal dominant subtype and review of the literature.

Epidermolysis bullosa (EB) is a group of inherited blistering skin diseases that vary widely in their pathogenesis and severity. There are three main categories of EB: simplex, junctional, and dystrophic. This classification is based on the level of tissue separation within the basement membrane zone and this is attributed to abnormalities of individual or several anchoring proteins that form the interlocking network spanning from the epidermis to the dermis underneath. Dystrophic EB results from mutations in COL7A1 gene coding for type VII collagen leading to blister formation within the dermis. Diagnosis ultimately depends on the patient's specific genetic mutation, but initial diagnosis can be made from careful examination and history taking. We present a pregnant patient known to have autosomal dominant dystrophic EB and discuss the obstetrical and neonatal outcome. The paper also reviews the current English literature on this rare skin disorder.

Could ossification of the achilles tendon have a hereditary component?

Ossification of the Achilles tendon (OTA) is an unusual clinical condition. It is characterized by the presence of an ossified mass within the fibrocartilaginous substance of the Achilles tendon. The etiology of the ossification of the Achilles tendon is unknown. Review of the literature suggests that its etiology is multifactorial. The major contributing factors are trauma and surgery with other minor causes such as systemic diseases, metabolic conditions, and infections. To our knowledge, no previous reports suggest any genetic/hereditary predisposition in OAT. We report 3 siblings who have OAT with no history of any of the aforementioned predisposing factors. Could OAT have a hereditary component as one of its etiologies?

A suspected pelvic aneurysmal bone cyst in pregnancy.

Albeit rare, the majority of identified bone lesions in pregnancy spare the pelvis. Once encountered with a pelvic bone lesion in pregnancy, the obstetrician may face a challenging situation as it is difficult to determine and predict the effects that labor and parturition impart on the pelvic bones. Bone changes and pelvic bone fractures have been well documented during childbirth. The data regarding clinical outcomes and management of pregnancies complicated by pelvic ABCs is scant. Highly suspected to represent an aneurysmal bone cyst, the clinical evaluation of a pelvic lesion in the ilium of a pregnant individual is presented, and modes of delivery in such a scenario are discussed.

Value of sagittal fat-suppressed proton-density fast-spin-echo of the knee joint as a limited protocol in evaluating internal knee derangements.

PURPOSE: The aim of the study was to determine the accuracy and observer agreement in the assessment of internal knee derangement using sagittal fat-suppressed proton-density fast-spin-echo (FS PD-FSE) compared with combined sagittal T1-weighted spin-echo, dual-proton-density, and T2-weighted spin-echo sequences and with arthroscopy. METHODS: One hundred eighteen patients undergoing routine knee magnetic resonance (MR) imaging had additional imaging with sagittal FS PD-FSE sequences. Menisci, cruciate ligaments, extensor tendons (ETs), bone marrow, osteoarthritic changes, soft tissue edema, joint effusion, and incidental tumors were analyzed. Magnetic resonance images were independently reviewed by 2 radiologists. Fifty patients underwent knee arthroscopy. Statistical analysis compared both imaging protocols with each other and with arthroscopy. Intrareader and interreader agreements were evaluated using κ analysis. Both protocols were compared with arthroscopy. RESULTS: Intrareader agreement was very high except for readings of the posterior cruciate ligament, ETs, and cartilage. Intrareader agreement did not differ significantly between the 2 readers except for ETs, bone marrow, and cartilage. Interreader percent agreements were high using both protocols and were not significantly different between the 2 readers except for posterior cruciate ligament. Compared with arthroscopy, both methods showed almost identical results regarding sensitivity, specificity, positive predictive value, and negative predictive value, except for cartilage where FS PD-FSE had increased sensitivity, whereas the combined protocol had increased specificity. CONCLUSIONS: Sagittal FS PD-FSE is comparable to our regular MR protocol in assessing internal knee derangement with an overall agreement of at least 93% on all sites except cartilage. It was also comparable to arthroscopy in assessing the cruciate ligaments and menisci, but had a low specificity for cartilaginous derangements. It can replace our 3 sagittal series comprising T1- and T2-weighted and proton-density-spin-echo sequences, hence saving time and cost.

New trends in the management of hepatic Echinococcus granulosus.

The liver is the most commonly involved organ in the body by cystic echinococcosis (CE) secondary to infection with Echinococcus granulosus. In this article, the authors discuss the classification, recent advances in magnetic resonance (MR) imaging for the diagnosis of hepatic CE, and approaches for management of hepatic CE using five therapeutic options that include: antihelminthic chemotherapy, surgery, percutaneous treatment, endoscopic approach, and the "watch and wait" approach.

The correction of the relapsed club foot by closed distraction.

Correction of a relapsed clubfoot deformity by distraction with an external fixator is a recognized alternative to open surgery. Most published series report a good outcome but none are prospective observational studies using the scoring system of the International Clubfoot Study Group (ICFSG). We present a series of 9 relapsed club feet treated with closed gradual distraction using this scoring method.