Pregnancy complicated with PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome: a case report.

BACKGROUND: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome has been considered as a childhood syndrome. The underlying etiology of PFAPA syndrome is unclear however, currently considered as auto-immune inflammatory disease. Recently, a few cases of adult-onset of PFAPA syndrome have been reported. However, there is no report about the successful management of pregnancy complicated with PFAPA syndrome. CASE PRESENTATION: The patient was a 31-year-old woman who developed recurrent episodes of high fever associated with cervical adenitis, pharyngitis and vomiting started 9 months after a delivery. She was diagnosed with PFAPA syndrome and cimetidine 800 mg/day was initiated. Since then, these symptoms got better. Cimetidine treatment was discontinued since she became pregnant (6 weeks of pregnancy). Except one febrile episode at 8 weeks gestation, she did not develop a febrile episode during pregnancy. Peripheral blood Th1/Th2 ratio was decreased from the first trimester to the second trimester of pregnancy. Then again, the ratio was steadily elevated during the third trimester. At 38 weeks, she delivered a live born infant without any complication. Two months after delivery, she developed PFAPA syndrome again and cimetidine treatment was re-initiated. However, febrile episodes were not controlled well, and Th1/Th2 ratio was further elevated compared to pregnancy status. Colchicine 0.5 mg once a day was initiated. Symptoms were diminished and Th1/Th2 ratio was gradually decreased. CONCLUSION: There was no case report of pregnancy complicated with PFAPA syndrome, though there were several reports of adult-onset PFAPA cases without pregnancy. The current case may be the first case report of a successful pregnancy complicated with PFAPA. In this case, PFAPA symptoms were ameliorated during pregnancy, but reappeared after delivery. We speculate that PFAPA syndrome, a Th1 type immune disorder, might be improved due to the Th1 to Th2 shifting, which was induced by pregnancy. It is necessary to investigate further about PFAPA syndrome with pregnancy and Th1/Th2 immune responses in the future.

Laparoscopic Uterovaginal Anastomosis for Cervical Agenesis: A Case Report.

STUDY OBJECTIVE: Isolated cervical agenesis occurs in 1 in 80 000 to 100 000 births. According to the American Fertility Society, cervical agenesis should be classified as a type Ib müllerian anomaly. According to ESHRE/ESGE classification, it is classified in class C4 category. Here we demonstrate the possibility of an innovative surgery for the management of cervical agenesis. DESIGN: Stepwise description of laparoscopic uterovaginal anastomosis (Canadian Task Force classification II-3). SETTING: Video. PATIENT: A 13-year-old girl. INTERVENTION: Laparoscopic uterovaginal anastomosis was performed. Informed consent was taken from the patient for use of video and images. Institutional review board has ruled that approval was not required for this study. MEASUREMENTS AND MAIN RESULTS: This video demonstrates the management of a case of a 13-year-old girl with primary amenorrhea and cyclical lower abdominal pain for 5 months. After complete examination and investigation, a diagnosis of isolated cervical agenesis with hematomata and blind-ending vagina was made. An innovative technique was used to perform laparoscopic uterovaginal anastomosis. Later, a hysteroscopy was done that revealed patency of anastomoses. As a result, the patient is experiencing spontaneous regular menstruation for 48 months. The main steps of the procedure were as follows: A follow-up hysteroscopy was performed at 9 weeks after surgery. It showed patent anastomosis and normal uterine cavity. After 48 months, a repeat hysteroscopy was done and a partial fibrotic septum noted. It was resected using electric energy. CONCLUSION: Uterovaginal anastomosis for isolated cervical agenesis is possible by a minimally invasive approach. It can be offered as a first-line management for such cases over hysterectomy and cervical canalization, which have high complication rates. The surgery should only be performed by a specialized team with required expertise in minimally invasive surgery.

Long-Term Follow-Up after Laparoscopic Uterovestibular Anastomosis in Patients with Cervical Atresia and Complete Absence of the Vagina.

BACKGROUND: The surgical treatment of girls with cervical atresia and complete absence of the vagina remains a problem because of the rarity of cases and the controversial study results. OBJECTIVE: To describe the surgical technique and long-term results of laparoscopically assisted uterovestibular anastomosis in patients with cervical atresia and complete absence of the vagina STUDY DESIGN: Sixteen consecutive patients with cervical atresia and complete absence of the vagina were conservatively treated with laparoscopically assisted uterovestibular anastomosis in 2 tertiary care referral centers. The follow-up assessments included clinical examination, determination of the presence and quality of sexual intercourse, and vaginoscopy. RESULTS: All patients underwent laparoscopically assisted uterovestibular anastomosis. No perioperative complications occurred. The mean follow-up period was 8 ± 3.2 years. In all patients, the length of the neovagina was greater than 4 cm at 1 year after the surgery and approximately 6 cm after 2 years. After the start of sexual intercourse, the neovagina exceeded 7 cm in length in 2 of the 11 sexually active patients. At 12 months after the surgery, iodine-positive epithelium was present in all patients and was maintained over time. The continuity of the neovagina, neocervix, and uterine body was maintained without further interventions in 15 of the 16 patients. During the follow-up, 11 patients were sexually active, 5 were married, 4 were seeking conception, and 2 had spontaneous pregnancy. CONCLUSIONS: Laparoscopically assisted uterovestibular anastomosis seems to be a safe and effective treatment for patients with cervical atresia and complete absence of the vagina, at least in terms of the recovery of menstrual function and sexual activity.

Spontaneous Cervicovaginal Fistula in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report.

BACKGROUND: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare diagnosis; patients classically present with dysmenorrhea and increasing pelvic pain. Unusual manifestations of OHVIRA syndrome might occur as a result of patient anatomy and the rupture or relief of the obstructed hemivagina. CASE: We present a 15-year-old patient with OHVIRA syndrome who developed a spontaneous cervicovaginal fistula and subsequently underwent hemihysterectomy.

Plicae palmatae in neonates: tumor mimic on sonography.

Plicae palmatae are uterine cervical folds that can be misdiagnosed as a uterine septum on magnetic resonance imaging (MRI). Previous studies in the literature generally tend to include only adults and are limited to MRI findings. Here, we present the case of a 6-day-old girl with a tumorous lesion in her uterine isthmus on sonography. Although we considered uterine tumor as a differential diagnosis, the smooth surface and continuity with a cervical fold on the caudal side suggested that the lesion was, in fact, the tip of plicae palmatae. MRI was subsequently performed, and no restricted diffusion was found, which was useful in ruling out a malignant tumor. Our case highlights that plicae palmatae in neonates can mimic a tumor on sonography. Meticulous observation of a continuity with a cervical fold is essential for correct diagnosis.

Piometra secundario a estenosis cervical en una adolescente / Pyometra secondary to cervical stenosis in an adolescent

INTRODUCCIÓN: El piometra es una afección infrecuente, pero grave, que en general se diagnostica en mujeres posmenopáusicas. En adolescentes es sumamente raro, y si se acompaña de amenorrea primaria hay que tener en mente las anomalías congénitas. CASO CLÍNICO: Adolescente de 13 años, sin antecedentes personales de interés salvo amenorrea primaria, que acude con abdomen agudo y es intervenida por una peritonitis difusa causada por un piometra secundario a disgenesia (estenosis) cervical congénita. Se realizó dilatación cervical y se dejó una sonda vesical intrauterina para prevenir la reestenosis. CONCLUSIONES: Un diagnóstico precoz y un tratamiento conservador con dilatación cervical y colocación temporal de un catéter urinario son esenciales para un manejo seguro y efectivo de la estenosis cervical en adolescentes.

INTRODUCTION: Pyometra is an uncommon but serious condition that is generally diagnosed in postmenopausal women. In adolescents it is extremely rare; if accompanied by primary amenorrhea, consider congenital abnormalities. CASE REPORT: A 13-year-old adolescent, with no relevant personal history except primary amenorrhea, who presented with an acute abdomen and was operated on for diffuse peritonitis caused by pyometra secondary to congenital cervical dysgenesis (stenosis). Cervical dilation was performed and a urinary catheter was temporarily placed inside the uterus to prevent restenosis. CONLUSIONS: An early diagnosis and conservative treatment with cervical dilation and temporary placement of a urinary catheter are essential for the safe and effective management of cervical stenosis in adolescents.

Management of neonatal genital prolapse: case reports and historic review.

Neonatal genital prolapse is a rare condition. It usually occurs during the first few days of life and presents as a tumor mass protruding from the vulva. Most cases occur in association with meningomyelocele or other anomalies of the central nervous system. Two cases are presented to illustrate the usefulness of conservative management of this unusual problem. A historical review of the literature is presented to acquaint the reader with the variety of treatments that have been used in the past.

Persistent incompetence of the cervix in female infants with congenital abnormalities of the pelvic viscera and perineum.

Vaginal reflux during voiding cystourethrography (VCUG) is a common finding in young females. Reflux of urine into the uterus, fallopian tubes, and peritoneal cavity documented by contrast studies is rare and considered abnormal. Chronic retrograde flow of urine and its contaminants through an incompetent cervical os mechanism into the peritoneal cavity may lead to peritonitis, pelvic inflammatory disease, and sterility. Five females with congenital abnormalities of the pelvic viscera and perineum had reflux into the uterus, fallopian tubes, and/or peritoneal cavity during voiding studies done before or months to years after successful corrective surgery. The findings were compatible with a persistent incompetent cervical os. It appears that this group of patients is at risk for the development of chronic pelvic inflammatory disease and its consequences.

Congenital and acquired pathology of ovary and tubular genital organs in ewes: a review.

Advances in our understanding of ovarian cyclicity, pathogenesis of subfertility and/or infertility and reproductive pathology in food animals have frequently entailed examination of abattoir material. Despite the fact that most lesions in ewes are likely to be of relatively minor significance to fertility, results of previous studies suggest that lesions of the female reproductive system may represent a significant source of loss to sheep husbandry. The objective of this paper is to review the pathophysiology, the effects on reproductive efficiency and the key gross and histological diagnostic features of congenital and acquired pathology of ovary and tubular genital organs in ewes.