Prevalence of T-shaped uterus among fertile women based on ESHRE/ESGE and Congenital Uterine Malformation by Experts (CUME) criteria.

RESEARCH QUESTION: What is the prevalence of T-shaped uteri among fertile women based on ESHRE/ESGE and Congenital Uterine Malformation by Experts (CUME) criteria? DESIGN: A prospective cohort study of 258 women of reproductive age with a history of at least one natural pregnancy resulting in live birth. Participants were recruited from the family planning clinic between January 2018 and March 2020. The ESHRE/ESGE classification of congenital anomalies of the female genital tract was used for describing abnormal findings. CUME criteria were also used for diagnosing T-shaped uterus. Uterine cavity volume was measured. RESULTS: Mean age of participants was 35.4 ± 6.2 years. Participants were diagnosed with the following: congenital uterine abnormality (n = 9 [3.6%]); partial septate uterus (n = 5 [2.0%]) and hemiuterus (n = 2 [0.8%]). Two women (0.8%) were diagnosed with T-shaped uterus and borderline T-shaped uterus based on the ESHRE/ESGE criteria and CUME. Mean lateral indentation angle, lateral indentation depth and T-angle were 156.2° ± 9.53°, 2.85 ± 0.93 mm and 73.3° ± 9.85° in patients with normal uterine cavity. In patients with T-shaped and borderline T-shaped uteri, respective figures were 115° versus 121°, 10 mm versus 7.6 mm and 27.5° versus 70°. Median volume of the uterine cavity in patients with normal uterine cavity and T-shaped uterus was 3.71 ml (minimum 2.0 to maximum 9.03 ml, interquartile range 1.93) and 3.2 ml (2.9 and 3.62 ml), respectively. CONCLUSIONS: The prevalence of T-shaped uteri in fertile women is low, which corresponds to previous reports of women with poor reproductive history.

Challenges of access to kidney care for children in low-resource settings.

Kidney disease is a global public health concern across the age spectrum, including in children. However, our understanding of the true burden of kidney disease in low-resource areas is often hampered by a lack of disease awareness and access to diagnosis. Chronic kidney disease (CKD) in low-resource settings poses multiple challenges, including late diagnosis, the need for ongoing access to care and the frequent unavailability of costly therapies such as dialysis and transplantation. Moreover, children in such settings are at particular risk of acute kidney injury (AKI) owing to preventable and/or reversible causes - many children likely die from potentially reversible kidney disease because they lack access to appropriate care. Acute peritoneal dialysis (PD) is an important low-cost treatment option. Initiatives, such as the Saving Young Lives programme, to train local medical staff from low-resource areas to provide care for AKI, including acute PD, have already saved hundreds of children. Future priorities include capacity building for both educational purposes and to provide further resources for AKI management. As local knowledge and confidence increase, CKD management strategies should also develop. Increased awareness and advocacy at both the local government and international levels will be required to continue to improve the diagnosis and treatment of AKI and CKD in children worldwide.

The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment.

INTRODUCTION: Most patients with a cloacal malformation have a Mullerian anomaly. We sought to examine our patients with a cloacal malformation to determine the proportion of them we felt we knew their Mullerian anatomy and which proportion we felt would most benefit from longitudinal assessment to define their anatomy, reproductive potential, and risk of outflow tract obstruction after puberty. We also compared the preoperative assessment of reproductive anatomy (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) and intra-operative abdominal findings (at the time of primary cloacal reconstruction or subsequent abdominal procedures) to see how these correlated with and which preoperative assessment tool was most predictive of intraoperative anatomy. We also sought to confirm what we expected to be a lack of ovarian pathology. METHODS: A single site retrospective chart review was performed on all patients with a cloacal anomaly seen between May 2014 and September 2017. Preoperative assessment (pelvic ultrasound, MRI, cloaca gram, and vaginoscopy) and operative reports (both primary reconstruction and later abdominal procedures) were reviewed to ascertain Mullerian and ovarian anatomy. RESULTS: 30 of 36 (83%) of patients had defined Mullerian anatomy after preoperative assessment (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) with or without the addition intraoperative assessment of Mullerian structures obtained during laparoscopy or laparotomy. 19/30 (63%) had duplication of their Mullerian structures. 25/36 (69%) had intraoperative assessment of Mullerian anatomy during laparoscopy or laparotomy. In this group, preoperative assessment with pelvic ultrasound correlated in 4/8 patients (50%), MRI correlated in 3/4 patients (75%), cloacagram in 10/15 patients (67%), and vaginoscopy in 23/25 patients (92%). 14/36 (39%) patients were found to require longitudinal assessment to define anatomy, reproductive potential or risk of outflow tract obstruction after puberty. Patients with ovarian findings described at the time of laparoscopy or laparotomy had no evidence of ovarian pathology. CONCLUSIONS: The majority of patients with cloaca in our series (83%) had their Mullerian anatomy defined by either preoperative assessment and/or findings at the time of laparoscopy or laparotomy. Duplication of the vagina and uterus was the most commonly described Mullerian anatomy (63%) in our series. Vaginoscopy appears to be superior to pelvic ultrasound, MRI, and cloacagram in predicting Mullerian anatomy. Fourteen of our 36 (39%) patients will require longitudinal assessment follow for reproductive potential and/or risk of outflow tract obstruction after puberty as their Mullerian anatomy is not known. There was no evidence of ovarian pathology in any cloaca patient. While we felt as though we could define Mullerian anatomy in most of our patients, any opportunity for intraoperative assessment of Mullerian anatomy should be utilized and therefore teams who are involved in the management of these patients must have a systematic and collaborative method established to ensure that Mullerian structures are thoroughly evaluated intra-operatively and documented in a standardized fashion. TYPE OF STUDY: Retrospective Chart review. LEVEL OF EVIDENCE: III.

Assessment of kidney function in children by enzymatic determination of 2- or 24-h creatinine clearance: comparison with inulin clearance.

BACKGROUND: Although renal inulin clearance (Cin) is the gold standard for evaluation of kidney function, it cannot be measured easily. Therefore, creatinine clearance (Ccr) is often used clinically to evaluate kidney function. Enzymatically measured Ccr was recently found to be much higher than Cin because of the tubular secretion of creatinine (Cr). This study compared three measures of renal clearance, inulin, 2-h Ccr, and 24-h Ccr, in children. METHODS: Kidney function was evaluated in 76 children (51 males and 25 females) aged 1 month to 18 years with chronic kidney disease (CKD) by three renal clearance methods at almost the same time. RESULTS: Correlations between each pair of three renal clearance measurements were determined. Approximate glomerular filtration rate (GFR) was equal to 62 % of 2-h Ccr or 76 % of 24-h Ccr. CONCLUSION: Cr secretion by renal tubules was approximately 50 % of the GFR. In this study, we indicate that the measurements of 2-h Ccr or 24-h Ccr do not show true GFR but we could infer approximate GFR from the values. The use of 2- or 24-h Ccr might contribute to the treatment of pediatric CKD patients.

Fetal serum α-1 microglobulin for renal function assessment: comparison with ß2-microglobulin and cystatin C.

OBJECTIVE: To compare the prognostic value of fetal serum α1-microglobulin with that of ß2-microglobulin and cystatin C for postnatal renal function. METHOD: Retrospective study of α1-microglobulin, ß2-microglobulin, and cystatin C in fetal serum from 126 fetuses with congenital abnormalities of the kidney and urinary tract (73 and 53, respectively). Two groups were defined: group with normal renal function and group with renal failure. For live born infants, renal function was assessed on the basis of serum creatinine (cutoff 50 µmol/L) or glomerular filtration rate (cutoff 75 mL/min/1.73 m2) or both. In case of infant or fetal death, histological kidney lesions were considered. RESULTS: Significant differences (p < 0.001) were observed for the three markers between fetuses with good renal prognosis and those with renal failure (34.4 mg/L vs 67.6 mg/L for α1-microglobulin, respectively; 3.9 mg/L vs 7.35 mg/L, for ß2-microglobulin, respectively; and 1.67 mg/L vs 2.12 mg/L for cystatin C, respectively). Areas under receiver operator curves were used to compare the three markers, 0.96, 0.90, and 0.74 for ß2-microglobulin, α1-microglobulin, and cystatin C, respectively. CONCLUSION: Although α1-microglobulin is significantly different in fetuses with good renal prognosis and those with renal failure, overall, it is a less reliable prognostic marker than fetal serum ß2-microglobulin.

What the radiologist needs to know about urolithiasis: part 1--pathogenesis, types, assessment, and variant anatomy.

OBJECTIVE: This article reviews types of urinary calculi and their imaging appearances, presents direct and secondary imaging findings of urolithiasis, and provides an overview of treatment methods. Pertinent imaging findings that impact clinical management are highlighted. The implications of complex or variant genitourinary anatomy are reviewed. We outline a standard format for the reporting of urolithiasis to facilitate informed clinical management decisions. CONCLUSION: Unenhanced CT is the preferred examination for evaluation of urolithiasis because of its availability, ease of performance, and high sensitivity. An awareness of the important imaging findings to report allows appropriate and efficient therapy.

Promoting major pediatric surgical care in a low-income country: a 4-year experience in Eritrea.

BACKGROUND: Access to pediatric surgical care in many sub-Saharan African countries is strongly limited by lack of medical facilities, adequate transport system, and trained medical and nursing manpower. The mortality rate for major congenital abnormalities remains extremely elevated in this area of the world. Strong efforts have been spent during the past decades to elevate the level of pediatric surgery standards in these countries through cooperation programs acting through periodical medical missions or supporting local medical teaching institutions. This is a report of a partnership between an Italian Medical Institution and the Eritrean Ministry of Health with the goal to improve pediatric surgical standard of care in the country. METHODS: During the past 4 years, teams composed of two pediatric surgeons, a pediatric anesthetist, and a pediatric nurse regularly visited the Orotta Medical and Surgical Referral Hospital of Asmara (Eritrea) to offer modern surgical treatment to children with major congenital abnormalities and to offer academic training to local medical and nursing staff. The team worked in local health structures. A total of 714 patients have been visited and 430 surgical procedures have been performed during 35 weeks of clinical work. Among them were 32 anorectal malformations, 11 Hirschsprung's disease cases, 8 bladder extrophies, and many other major surgical problems, such as congenital intestinal obstructions, obstructive uropathies, and solid tumors. RESULTS: The standard of care has been based on the principle of researching sustainable solutions. Surgical options and timing of each procedure have been decided to reduce hospitalization and the recourse to temporary stomas, indwelling catheterization, and prolonged intravenous feeding. Posterior sagittal anorectoplasties (PSARP) and posterior vagino-anorectoplasty (PSVARP) were performed for anorectal malformations, introducing minimal technical variants to reduce the need for postoperative nursing. Endorectal pull-through of functional stoma was the treatment of choice for patients with Hirschsprung's disease because frozen sections were not possible. Eight late-referred bladder extrophy cases were all managed by internal diversion (Mainz II pouch). Solid abdominal tumors always came to observation weeks or months after the first symptoms appeared. No CT scan was available and indications of surgery were based on clinical symptoms only. Only 11 of 18 cases were resectable, and only 5 of them with favorable histology survived, 2 after adjuvant therapy abroad. A large number of hypospadias were observed at the mean age of 4.5 years. Failures of previous attempts at correction were frequently found. The postoperative complications rate progressively decreased with the use of dripping stents to avoid the risk of accidental catheter removal or kinking. CONCLUSIONS: On the basis of our experience, major pediatric surgery in many under-resourced areas of sub-Saharan Africa can be developed, taking care to adapt surgical options to local conditions. Late referral of many congenital abnormalities, the impact of local culture, difficulties to establish regular follow-up, and shortage of facilities and medical devices must always be kept in mind before transferring modern protocols of management. Strong efforts have been devoted to train local medical and nursing staff to establish pediatric surgical manpower to cope with a still largely unanswered demand of care in this area of Africa.

MRI in the assessment of congenital vaginal anomalies.

AIM: To assess accuracy of magnetic resonance imaging (MRI) for the delineation of morphological abnormalities of the vagina in patients with congenital anomalies of the genito-urinary tract. MATERIALS AND METHODS: Fifty-one patients (median age 19 years; range 12-40 years) were studied. All were consecutively referred for MRI to assess genital tract anatomy, between 1996 and 2004, from a clinic specializing in congenital abnormalities of the urogenital tract. All patients were assessed clinically and underwent MRI. Images were reviewed retrospectively by an experienced radiologist. Where there was discordance between clinical and radiological findings a consensus diagnosis was achieved by the gynaecologists and radiologists reviewing all of the clinical and radiological evidence together, including assessment of vaginal length. RESULTS: The clinical data were incomplete for five women and the images non-diagnostic in two cases; consequently, 44 of 51 women had complete datasets and could be evaluated. Vaginas were abnormal in 30 of the 44 patients. There was discordance between the clinical and imaging findings at the initial review in three of the 44 cases (6.8%). After consensus review, and with the inclusion of measurement of the vaginal length on MRI, the MRI and clinical findings were concordant in all cases. The initial discordance was due to two vaginal dimples not being appreciated on MRI and one case in which presence of vaginal tissue proximal to a mid-segment obstruction was not appreciated clinically. CONCLUSION: MRI is an accurate method of imaging vaginal anomalies. However, to achieve reliable results the radiologist requires details of previous surgery and the vaginal length must be measured.

Genitourinary assessment: an integral part of a complete physical examination.

This article provides primary care providers, including pediatric nurse practitioners, with a framework for completing a genitourinary assessment. Many primary care providers are reluctant to examine the genitalia of their patients. Routine genital examinations increase diagnostic skills, provide a baseline for future examinations, may improve parent and child compliance with the examination, and may reveal previously undiscovered anomalies or trauma. An assessment of the reproductive and urologic systems should begin with obtaining a focused history from the parent from birth to present. Techniques for performing a focused genitourinary examination will be discussed.

[Prenatal hydronephrosis: incidence, management and final diagnoses in 2003]. / Ectasia piélica de diagnóstico prenatal. Incidencia y protocolo de estudio durante el año 2003.

BACKGROUND: Prenatal diagnosis of renal pyelectasis usually involves postnatal studies to determine whether treatment is necessary or not. OBJECTIVES: To determine the incidence of fetal pyelectasis in our environment, to review our postnatal management protocol, and to review definitive diagnoses. PATIENTS AND METHOD: We performed a retrospective review of newborns with a prenatal diagnosis of pyelectasis. The variables recorded included prenatal ultrasound examinations, gestational age, sex, anthropometric data, postnatal study (ultrasound, cystography, isotopic renogram) and indication for antibiotic prophylaxis. RESULTS: There were 21 newborns (nine boys and 12 girls). Pyelectasia were located in the right kidney in 10 patients, on the left in seven and were bilateral in four. Antibiotic prophylaxis was administered at birth in seven neonates. Postnatal ultrasound was performed at 17.19 +/- 12.7 days of life and revealed no abnormalities in seven patients, hydronephrosis grade I-II in nine, hydronephrosis grade III in three and suspected double excretion system in two. Cystourethrography and isotopic renogram were performed in six neonates. The definitive diagnoses in the 21 patients were: no abnormalities in 10, non-complicated renal dilatation in seven, double excretion system in two, vesicoureteral reflux grade IV in one and pyeloureteral stenosis in one. None of these newborns had urinary tract infection. CONCLUSIONS: The incidence of prenatal pyelectasis in our hospital is 2 %. Most pyelectasia resolve spontaneously in the first year of life and invasive investigations are not required. Adequate monitoring of these children can avoid urinary tract infections and their sequelae.

Congenital spinal deformity: a comprehensive assessment at presentation.

STUDY DESIGN: A series of 126 consecutive patients with congenital spinal deformity is presented. OBJECTIVE: To assess the incidence of intraspinal anomaly and other organic defects associated with different types of spine deformity at presentation. SUMMARY OF BACKGROUND DATA: A high incidence of intraspinal abnormalities and other organ defects is reported in relation to congenital spine deformity. The prevalence of these problems with different types of deformities is to be determined. METHODS: All patients had MRI, echocardiography, renal ultrasound, and a thorough clinical assessment. RESULTS: Intraspinal abnormalities were found in 47 patients (37%). These abnormalities were significantly more common in patients with congenital kyphosis ( = 0.0048), and in those with scoliosis resulting from mixed and segmentation defects. Scoliosis patients with cervical and thoracic hemivertebrae had significantly more intraspinal abnormalities ( = 0.0253) than those with lumbar hemivertebrae. In 64 (55%) patients other organic defects were found. These defects were more common in patients with congenital scoliosis resulting from mixed defects ( = 0.002). Cardiac defects were detected in 26% and urogenital anomalies in 21% of the patients. CONCLUSIONS: Magnetic resonance imaging and echocardiography should be an essential part in the evaluation of patients with congenital spinal deformity, and special attention should be paid to patients with segmentation abnormalities, mixed defects, and kyphosis.

Classification of anorectal malformations--initial approach, diagnostic tests, and colostomy.

The optimal surgical care of patients with imperforate anus begins with appropriate decision making in the critical newborn period. In most cases the decision to create a colostomy should be delayed until the infant is 18 to 24 hours old. Except in cases of a rectoperineal fistula, most neonates are best treated with a completely divided left-lower-quadrant colostomy between the descending and sigmoid colons. Female patients with cloacal anomalies must be recognized at birth so that all urgent urologic evaluations can be performed. Hydrocolpos and obstructive uropathy are common in these neonates and warrant urgent decompression of the urinary tract with a vaginostomy and/or vesicostomy as well as a colostomy. Renal ultrasonography and voiding cystourethrography are mandatory for all patients regardless of the height of the defect. It is critical to discover the important precursors to renal insufficiency including renal agenesis, renal dysplasia, and vesicoureteral reflux in the neonate. The presence of these anomalies mandates early consultation with a pediatric urologist because the morbidity and mortality of these lesions often exceed those of the imperforate anus. Spinal cord anomalies are common and can be found even in patients who have normal plain films and low defects. Spinal ultrasonography or magnetic resonance imaging should be performed in all neonates to rule out occult spinal pathology such as tethered cord or lipoma of the cord. Efficacious and cost-effective care of patients with imperforate anus begins with a carefully thought out plan in the neonate. Optimal execution of the evaluation and surgical treatment at this phase sets the stage for the best possible outcome later in life.