Pediculated Accessory Liver Lobe with Gallbladder in a Preterm with Umbilical Cord Hernia.

(1) Background: Accessory liver lobes are a rare finding and only a few case reports of accessory liver lobes in abdominal wall defects have been reported so far. In the case of a congenital wall defect including liver parenchyma, there is still an ongoing debate on the definition of the abdominal wall defect and best care practice. Even though congenital abdominal wall defects are frequently diagnosed in prenatal screenings, controversy on the underlying etiology, embryology and underlying anatomy remains. Prenatal distinction between omphalocele and hernia into the cord cannot always be obtained; however, due to its clinical relevance for postnatal management and counseling of parents, accurate diagnosis is essential. (2) Case Presentation: We describe the uncommon postnatal finding of a pediculated accessory liver lobe with gallbladder in a preterm with umbilical cord hernia, which was prenatally diagnosed as omphalocele. Postnatal examination revealed an amniotic sac with a diameter of six and a small abdominal wall defect of three centimeters in diameter. Postnatal management included resection of the accessory liver lobe and gallbladder and closure of the defect. (3) Results and (4) Conclusions: Throughout the literature, the distinction between umbilical cord hernia and omphalocele has been variable. This has led to confusion and difficulties regarding postnatal treatment options. In order to achieve an accurate prenatal and/or postnatal diagnosis, the morphological differences and clinical manifestation of umbilical cord hernia and omphalocele need to be assessed. Further embryological studies are warranted to understand the underlying embryological pathology of omphalocele and umbilical cord hernia and offer appropriate treatment. In consideration of possibly severe complications in the case of the torsion of a pedunculated accessory liver lobe, we strongly recommend primary removal once pre- or intraoperative identification has been made.

Ultrasound Markers in Fetal Hydronephrosis to Predict Postnatal Surgery.

PURPOSE: Parents confronted with the finding of antenatal hydronephrosis (ANH) are particularly interested in whether their baby will need postnatal surgery. The objective of this study was to predict ANH requiring surgery on the basis of the fetal anteroposterior renal pelvic diameter (APRPD) and the Society for Fetal Urology (SFU) grading system. MATERIALS AND METHODS: The medical records of 179 patients with the finding of ANH were reviewed retrospectively. ANH was graded according to the SFU grading system. Prenatal ultrasound examinations were correlated to postnatal outcome, which was divided into three groups: prenatal resolution, conservative management and surgical treatment. RESULTS: 58 (32.4 %) cases were classified as prenatal resolution, 89 (49.7 %) babies were assigned to the conservative outcome group and 32 (17.9 %) patients needed surgical repair. Postnatal surgery was best predicted in the second trimester (area under the receiver operating characteristics curve: 0.839) by an APRPD cut-off of 8.3 mm (sensitivity: 77.8 %; specificity: 85.7 %; PPV of 53.9 %, NPV of 94.7 %). The combination of the parameters "progression of SFU grade" and SFU grade 3 or 4 achieved a sensitivity of 84.4 % and a specificity of 80.3 % for the prediction of surgery. CONCLUSION: Second-trimester APRPD is a useful parameter for predicting the risk for postnatal surgery. The SFU grade should be assessed in every prenatal ultrasound examination as some further risk estimates can be made based on its dynamics over time.

Sacrococcygeal Teratoma Presenting with Vaginal Discharge and Polyp in an Infant.

BACKGROUND: Sacrococcygeal teratoma accounts for the most common solid tumor in neonates. Because of improved technology, 50%-70% of cases can be diagnosed antenatally during routine ultrasound screenings. If not diagnosed antenatally, clinical findings at birth are distinct in most cases including a palpable or visible mass. CASE: We report an unusual case of a 1-year-old girl who presented with persistent vaginal discharge leading to diagnosis of a mucosal polypoid lesion of the vagina, ultimately revealing a hidden sacrococcygeal teratoma. SUMMARY AND CONCLUSION: We suggest thorough investigation of all infants who present with purulent discharge and recurrent vaginal mass; sacrococcygeal teratoma should routinely be considered as a differential diagnosis.

Laparoscopic Management of Autoamputated Ovary in Newborns: A Report of 2 Cases.

Intrauterine autoamputation of the ovary is an extremely rare diagnosis in the pediatric population. The current literature is limited to contradictory recommendations, while a standard management protocol for autoamputated adnexa secondary to intrauterine ovarian torsion is yet to be established. We report 2 cases of auto-amputation of the ovary, leading to a free-floating intra-abdominal cyst in the newborn. Laparoscopic management was successful in both cases.

Reduced bacterial colonisation of the glans penis after male circumcision in children--a prospective study.

OBJECTIVE: The aim of this prospective study was to evaluate the effect of male circumcision on the bacterial colonisation of the glans penis in children. PATIENTS AND METHODS: 244 males were included in this study. The study group consisted of 143 boys admitted for circumcision between August 2009 and July 2010. Periurethral swabs were taken preoperatively and one week postoperatively. The control group included 101 boys without phimosis, in which only one swab was taken. Patients were subgrouped according to age below and above five years. Bacterial cultures were analysed, results were categorized by non-uropathogenic and uropathogenic bacteria, and compared within and between groups. RESULTS: Patients in both control group and study group before circumcision showed significant bacterial colonisation (>98%), involving known uropathogenic bacteria in over 86%. After circumcision, bacterial colonisation dropped from 100% to 86.3% (p < 0.005) in boys younger than five years and from 98.57% to 77.14% (p < 0.001) in those aged five or above, respectively. Moreover, the fraction of uropathogenic bacteria decreased significantly. CONCLUSION: Male circumcision significantly reduces the bacterial colonisation of the glans penis with regard to both non-uropathogenic and uropathogenic bacteria.

Severe pediatric blunt trauma--successful ROTEM-guided hemostatic therapy with fibrinogen concentrate and no administration of fresh frozen plasma or platelets.

Use of allogeneic blood products to treat pediatric trauma may be challenged, particularly in relation to safety. We report successful treatment of a child with severe abdominal and pelvic injuries with preemptive fibrinogen supplementation followed by rotational thromboelastometry (ROTEM)-guided, goal-directed hemostatic therapy. Fibrinogen concentrate was administered (total dose: 2 g), while transfusion of fresh frozen plasma and platelet concentrate was avoided. Activated partial thromboplastin time was prolonged and Quick values were low but ROTEM clotting time values remained normal, therefore, no thrombin-generating drugs were considered necessary. This case shows the potential for hemostatic treatment with coagulation factor concentrates to be applied to pediatric trauma.

Improvement of continence with reoperation in selected patients after surgery for anorectal malformation.

PURPOSE: Fecal incontinence is a serious complication after repair of anorectal malformations. We investigated whether reoperation can improve fecal continence. METHODS: Medical records of 41 patients (40 children and one adult; 26 male and 15 female) who underwent reoperation after previous reconstruction of an anorectal malformation were reviewed for outcomes of bowel function. Type of primary corrective surgery performed, therapeutic measures, results of physical examination and barium enema, and reoperation procedures were evaluated. A questionnaire was administered to assess stool behaviour and level of continence at follow-up three or more years after secondary operation. RESULTS: Secondary operations in males comprised posterior sagittal anorectoplasty (PSARP) in 16 patients, PSARP with antegrade continent enema in one patient, antegrade continent enema alone in 6, anoplasty in one, rectosigmoid resection in 1, and definitive colostomy in 1 patient. Secondary operations in females included PSARP alone in 4 patients, PSARP with total urogenital mobilization in 4, PSARP with vaginoplasty in 2, PSARP with vaginoplasty and antegrade continent enema in 2, and PSARP with vaginourethroplasty in 3. Of 41 patients 18 (44 percent) were continent at follow-up, 21 (51 percent) were clean with use of enemas, diet, or drug therapy. One patient had a definitive colostomy. One died after kidney transplantation. CONCLUSIONS: Surgery is a good option for improving incontinence in selected patients previously operated for anorectal malformations. Posterior sagittal anorectoplasty is advocated to improve bowel control. Antegrade continent enema is a reliable therapeutic option to maintain clean patients with fecal incontinence.

Dynamic magnetic resonance imaging of the pelvic floor in children and adolescents with vesical and anorectal malformations.

BACKGROUND: Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic floor and continence organs, it plays a major role in assessing urinary and fecal incontinence or constipation, especially when performed as a dynamic investigation such as MRI defecography. PATIENTS AND METHODS: Twenty-three patients (3-21 years old) with urinary and/or fecal incontinence or constipation attributable to congenital malformations of the pelvic region presented at our institution. The diagnoses were anorectal malformations (18), bladder exstrophy (2), and cloacal exstrophy (3). All patients underwent static and dynamic MRI of the pelvic floor with rest, squeeze, and evacuation in supine position. RESULTS: Morphology and function of the pelvic floor and pelvic organs could be demonstrated in each case. The reason for urinary incontinence, fecal incontinence, or constipation could be defined, and an individual therapeutic management concept was made based upon the data obtained by the investigation. CONCLUSIONS: The advantages of this method, in comparison to others, are direct visualization of the pelvic floor muscles and continence organs and their anatomical relationship during different functional actions. Pelvic floor dysfunction is often the reason for fecal and urinary incontinence and can be detected by MRI. Especially in children, minimizing radiation exposure is of major concern. Disadvantages are the costs and long investigation time.

Colpo-wrap: a new continence procedure.

OBJECTIVE: To present a new surgical method to increase bladder outlet resistance for the treatment of urinary incontinence in girls and women. PATIENTS AND METHODS: Six patients (mean age 9.6 years), with urinary incontinence were operated using the new technique within the last 3 years. The principle of the procedure is tightening of the bladder neck by mobilizing the anterior vaginal wall and wrapping it around the bladder neck and proximal urethra, in the sense of a vaginoplication (colpoplication). The underlying conditions and causes for urinary incontinence was neurogenic bladder-sphincter dysfunction caused by myelodysplasia in three girls and anorectal malformation combined with a tethered spinal cord in one. In one case incontinence was caused by a cloacal anomaly and one girl had intrinsic sphincter insufficiency after repetitive Otis urethrotomies. The colpo-wrap was combined with a bladder augmentation and Mitrofanoff in three patients, the three other girls undergoing isolated procedures. RESULTS: The result of the method is a constant increase in outlet resistance and coaptation of the urethra, comparable with the effect of a vaginal sling procedure. Five patients are completely dry after surgery, one girl with cloaca needed an additional bladder neck injection with hyaluranon/dextranomer copolymer. Transurethral catheterization was possible after surgery with no problems in all patients who required intermittent catheterization. CONCLUSION: Considering the feasibility of this technique the colpo-wrap is a reasonable alternative for treating urinary incontinence in females.

Rotundum psoas hitch: a new method for colpohysteropexy in girls with bladder exstrophy.

Female patients with bladder exstrophy frequently suffer from uterine prolapse, which is due to a defective pelvic floor anatomy. Different techniques for the correction of procidentia in this patient population have been described; however, since the numbers of patients are very small and no series with long-term follow-up exists, it is unclear which technique yields superior results. We describe here a new technique for uteropexy in girls and women with bladder exstrophy. In the rotundum psoas hitch procedure (RPH), the round ligaments are detached from the inner inguinal ring and fixed to the psoas muscle on both sides, thereby fixing the uterus in a more cranial and dorsal position. This procedure has been applied successfully in nine patients with a mean follow-up of 23.8 months. In six cases RPH was done prophylactically and in three cases therapeutically to correct an existing uterine prolapse. The mean age at operation was 9.6 years. In all but one case, RPH was combined with continent urinary reconstruction. No postoperative morbidity due to this technique has been described. RPH is an effective means of preventing or correcting procidentia in patients with bladder exstrophy; we emphasize that it should be combined with other abdominal operations, such as continent reconstruction, in girls or women with this condition.

Congenital obstructive distal urethral membrane.

We present a rare case of complete distal urethral obstruction caused by a distal meatal membrane in a newborn boy. A cystic lesion of the distal penis was detected and excised, revealing a hypospadiac urethra. Further examination showed bilateral vesico-ureteral reflux with a dilated posterior urethra and urethral lesion, suggesting posterior urethral valves (PUV); these were confirmed at 2 months and resected endoscopically. At 16 months a left nephrectomy was performed due to impaired renal function on that side; there was no longer reflux on the right side. The obstruction probably did not become severe until late in gestation, but still led to functional loss of one kidney. The PUV do not seem to have been the cause of renal deterioration. The cystic lesion may have been a remnant of the cloacal membrane, but this is difficult to prove histologically.

Strategies in urological reconstruction in myelomeningocele.

PURPOSE OF REVIEW: This paper reviews recent advances in the strategies for urinary tract reconstruction in children with spina bifida. The aims of reconstruction are the preservation of renal function and achievement of urinary continence. Considerable controversy exists on the subjects of bladder augmentation, procedures to increase outlet resistance, and continent stomas. The authors put the most recently published information in perspective in the light of their own personal experience. RECENT FINDINGS: Thirty-seven papers covering the above-mentioned subjects published in the past 5 years (25 of them published since 2000) have been selected. Seven relevant older references are included. There is persistent interest in developing methods to enlarge the urinary bladder that avoid bringing the urine in contact with the intestinal mucosa. The artificial urinary sphincter and fascial slings are the most frequently reported methods to increase outlet resistance. The Mitrofanoff principle continues to be regarded as an effective method to construct a continent catheterizable channel, either with the appendix or reconfigured intestinal segments. The achievement of fecal continence has to be pursued in parallel with urinary continence. Incontinent diversions continue to be best for a small group of patients. The role of continent urinary diversion requires reassessment. SUMMARY: Progress in this area continues to be made. Periodic, critical and objective reviews on the subject should help the practitioner to arrive at sound decisions.