Heather Appelbaum

Key Clinical Predictors in the Early Diagnosis of Adnexal Torsion in Children

OBJECTIVE Adnexal torsion (AT) accounts for 2.7% of cases of acute abdominal pain in children. When AT is undiagnosed, ovarian blood supply is compromised, eventually leading to tissue necrosis. Because the clinical presentation of AT is nonspecific, preoperative diagnosis is challenging. The purpose of this study was to identify predictors that differentiate AT from other sources of acute abdominal pain. METHODS This study was an IRB-approved retrospective chart review of girls age 4-18 y/o with acute abdominal pain who then underwent surgical evaluation. Data collected included age, menarchal status, symptoms, physical exam findings, laboratory tests, imaging studies, operative procedures and postoperative diagnosis. Factors associated with AT were included in a logistic regression model. A receiver operator characteristic (ROC) curve based on this model was then constructed in order to determine its ability to predict AT. RESULTS 94 patients presented with acute abdominal pain; 45 were diagnosed with AT and 49 with other causes of abdominal pain. Presence of intermittent pain (P < .0217), non-radiating pain (P < .0229) and increased adnexal size (P < .0032) were significantly associated with AT in the final model. The area under the ROC curve was equal to 0.8601, suggesting excellent discrimination between AT and other causes of acute abdominal pain by using these 3 parameters. CONCLUSION Key clinical and imaging findings can aid in the early diagnosis of AT in children. Future prospective studies will focus on development of a clinical predictive model for the diagnosis of AT in the pediatric population.

Clinical Variability in Approaches to Polycystic Ovary Syndrome

STUDY OBJECTIVE The objective of this study was to evaluate methods of initial diagnosis and management of polycystic ovary syndrome (PCOS) among members of the North American Society for Pediatric and Adolescent Gynecology (NASPAG) to assess the degree of practice heterogeneity among specialist providers of adolescent care. DESIGN Cross-sectional, anonymous, internet survey PARTICIPANTS NASPAG membership (N = 326; Respondents = 127 (39%)) RESULTS Percentage of respondents who incorporated specific tests at initial diagnosis was highly variable ranging from 87% (thyroid stimulating hormone) to 17% (sex hormone binding globulin). Oral contraceptives and diet modification/exercise were the most common therapies recommended by 98% and 90% of respondents respectively. CONCLUSION Considerable practice heterogeneity was present with regards to diagnostic testing for suspected PCOS. Recommendations for first-line therapy were more consistent. Future studies should clarify the clinical utility of specific diagnostic tests for adolescents, such that selection of diagnostic testing is evidence based.

Simultaneous development of ulcerative colitis in the colon and sigmoid neovagina

Vaginoplasty using sigmoid colon is a common technique for creation of a neovagina. However, special consideration must be given to potential long term consequences of using a colonic conduit for vaginal replacement. We report on the youngest described case in which a patient developed ulcerative colitis refractory to medical therapy with simultaneous involvement of a sigmoid neovagina requiring total proctocolectomy and neovaginectomy. A 17 year old XY female with a history of gonadal dysgenesis and sigmoid graft vaginoplasty presented with a history of bloody, mucoid vaginal discharge, abdominal pain, bloody diarrhea and weight loss. Colonic and neovaginal biopsies demonstrated active colitis with diffuse ulcerations, consistent with ulcerative colitis. Despite aggressive immunosuppressive treatment she had persistent neovaginal and colonic bleeding requiring multiple transfusions, subtotal colectomy and ultimately completion proctectomy and neovaginectomy. It is imperative to recognize that colectomy alone may be an inadequate surgical intervention in patients with ulcerative colitis and a colonic neovaginal graft and that a concomitant neovaginectomy may be integral in providing appropriate treatment.

Management of Isolated Tubal Torsion in a Premenarchal Adolescent Female with Prior Oophoropexy: A Case Report and Review of the Literature

BACKGROUND Isolated tubal torsion in a premenarchal adolescent girl is a rare phenomenon. Preoperative diagnosis remains a challenge. CASE A 14-year-old premenarchal girl, with a history of bilateral ovarian torsion treated by laparoscopic detorsion and oophoropexy two years prior, presented to the emergency room with lower abdominal pain accompanied by nausea and vomiting. Pelvic ultrasound demonstrated an enlarged left adnexa. Diagnostic laparoscopy revealed an isolated left tubal torsion. Surgical evidence of previous bilateral plication of the utero-ovarian ligaments was confirmed. Untwisting of the left fallopian tube immediately restored the vascular supply. Subsequently, her symptoms resolved. SUMMARY AND CONCLUSION Clinicians should consider torsion of the fallopian tube in the differential diagnosis of lower abdominal pain in all female patients. Prompt laparoscopic intervention is essential. Oophoropexy, while usually efficacious, may not prevent recurrence.

Primary vaginal dilation for vaginal agenesis: strategies to anticipate challenges and optimize outcomes.

Purpose of review Primary vaginal dilation is patient controlled, safe, less painful, and much lower cost compared with operative vaginoplasty and is considered first-line treatment for vaginal agenesis for women with Mayer–Rokitansky–Küster–Hauser syndrome and androgen insensitivity syndrome. Recent findings This review will highlight studies that assess the optimal methods of primary vaginal dilation and clarify ideal counseling, frequency of dilation, management of side-effects, and long-term physical and psychological outcomes. Summary Providers who care for women with vaginal agenesis should be prepared to not only teach the technical skill of dilation, but also to assess readiness and troubleshoot symptoms associated with dilation.

Torsion of fallopian tube remnant associated with noncommunicating rudimentary horn in adolescent girl with unicornuate uterus.

BACKGROUND The prevalence of müllerian anomalies may be as high as 7% in the general population, yet there is scant published literature on adnexal torsion occurring in these patients. CASE A 14-year-old female presented with right lower quadrant pain. Pelvic ultrasonography demonstrated a 2-cm simple right adnexal cyst. Diagnostic laparoscopy revealed a unicornuate uterus with a normal left uterine horn and fallopian tube but atretic and cordlike müllerian structures on the right side. Torsion of the right tubal remnant and two paratubal cysts were noted and the structures were then excised. SUMMARY AND CONCLUSION Agenesis, hypoplasia, or maldevelopment of müllerian structures may predispose patients to an increased risk of adnexal torsion secondary to looser ligamentous attachments and consequent lack of fixation to the pelvic sidewall.

Wilms Tumor With Metastasis to the Vagina: A Case Report

A 12-year-old female presented with abdominal pain, night sweats, weight loss, constipation, dysmenorrhea, menorrhagia, and vaginal discharge. Examination revealed a palpable flank mass and a large tumor adherent to the anterior vaginal wall. Computed tomography scan demonstrated a 23 cm mass in the left kidney, a separate 10.8 cm pelvic mass, and metastatic disease. Biopsies were consistent with Wilms tumor. Neoadjuvant chemotherapy and a left radical nephrectomy were performed for her stage IV disease as the kidney was amiable to complete resection. The patient received radiation and resumed chemotherapy. She was doing well with improved symptoms at follow-up.

Diagnostic Approach to Müllerian Anomalies

The diagnosis of Mullerian duct anomalies can be challenging. Appropriate and timely diagnosis may have profound effects on long-term quality of life, future fertility, and obstetrical outcomes. While most congenital obstructive malformations are detected during adolescence as a result of amenorrhea, dysmenorrhea, or pelvic pain, diagnosis of nonobstructive uterine anomalies may be delayed until fertility is compromised or obstetrical complications occur. A detailed history provides important data regarding syndromes associated with aberrant Mullerian duct formation, the chronicity of pubertal milestones, sexual functioning, and reproductive outcomes, while the physical examination allows for anatomical identification of lower genital tract abnormalities. In girls with Mullerian defects, imaging studies augment the physical findings and allow for a clearer understanding of the internal reproductive organs. Additionally, pelvic imaging is specifically valuable in distinguishing idiopathic dysmenorrhea from functional or structural causes of pelvic pain. Furthermore, precise imaging can help guide accurate surgical intervention. Genetic testing may be useful as an adjunct to understanding structural defects associated with disorders of sex development and other genetic syndromes. This chapter will provide a comprehensive approach to diagnosis of obstructive and nonobstructive congenital anomalies of the Mullerian ducts and other related congenital anomalies of the reproductive tract.

Congenital Anomalies and Abnormal Pubertal Development

Primary amenorrhea in girls is a frequent cause for referrals to the endocrinologist or gynecologist. History and physical examination provide important data regarding the chronicity of other pubertal milestones. Laboratory determinations contribute to understanding the hormonal milieu of the individual. Imaging studies can augment the physical findings and offer a clearer understanding of the internal reproductive organs. Pelvic imaging is specifically valuable in distinguishing idiopathic or functional causes of primary amenorrhea from structural causes. Genetic testing may be useful as an adjunct to understanding structural defects associated with disorders of sex development. Congenital anomalies account for a significant number of cases of primary amenorrhea and must be considered in the differential diagnosis, particularly in girls presenting with otherwise normal pubertal progression. This chapter will provide a case-based approach to appreciate the complexities of primary amenorrhea associated with congenital anomalies of the reproductive tract.