Outcome of children with multicystic dysplastic kidney: Does involved side matter?

BACKGROUND: Multicystic dysplastic kidney (MCDK) is a common anomaly detected on antenatal ultrasound. We aimed to assess the profile of children with MCDK and to investigate whether the involved side has any effect on outcome. METHODS: Thirty-nine patients with MCDK and 20 controls were enrolled. Patients who estimated glomerular filtration rate (eGFR) values over 90 mL/min/1.73 m2 were compared with controls. Comparison was made between the involved sides. RESULTS: MKDB was right-sided in 20 (51.3%) and left-sided in 19 (48.7%) patients. 33.3% had additional urinary tract abnormality, 10.2% had systemic abnormality. 82% showed contralateral kidney enlargement. 48.7% involuted, 17.9% underwent nephrectomy. 35.8% suffered from urinary tract infection (UTI). 5.1% had renal scarring (RS). 30% developed microalbuminuria. 12.8% complicated with hypertension. 17.9% progressed to chronic kidney disease (CKD). Hypertension was independent risk factor for developing CKD. Blood pressure, cystatin C and urine microalbumin/creatinine levels were increased, and eGFR values were decreased in patients compared to controls. No significant difference was found between the two sides for rates of involution, UTI, RS, nephrectomy, and additional abnormality. Cystatin C levels were higher on the right than left sides (p = .033). CONCLUSION: Children with MCDK predispose to renal deterioration even at normal eGFR values. Although cystatin C levels tended to increase in right-sided patients, the involved side seemed to have no significant effect on renal outcome. Hypertension was main determinant for progression to CKD in MCDK.

Penetrating injury caused by a long iron bar: A case report.

Foreign bodies that strike the body with their long edges can cause severe problems. From the hospitalization of the patients to the removal of the foreign body and from surgery to follow-up, working as a team in a well-organized manner is necessary. In the present research, we present our experience, including the hospitalization, initial assessment by the emergency team, examination, and treatment plan, of a pediatric patient who had a 12-m long iron bar that traversed from the right side of the anus to the loin; this impalement happened while the patient was sliding through a snow-covered street, and the patient had to wait on the snow until the fire department arrived and cut the iron bar.

Acquired Chiari I Malformation Secondary to Spontaneous Intracranial Hypotension Syndrome and Persistent Hypoglycemia: A Case Report

Spontaneous intracranial hypotension (SIH) is a rare and potentially serious condition in childhood. Cerebrospinal fluid (CSF) volume depletion is thought to be the main causative feature for intracranial hypotension and results from a spontaneous CSF leak, often at the spine level. SIH is increasingly diagnosed in clinical practice, although it manifests a varied symptomatology. The downward displacement of the brain, sometimes mimicking a Chiari I malformation, has rarely been reported. We present a case of a SIH with Chiari I malformation accompanied by an unusual clinical presentation of persistent hypoglycemia.

Neonatal gastric perforations in very low birth weight infants: a single center experience and review of the literature.

PURPOSE: Gastric perforation is a rare condition with high mortality rates in preterm infants. The aim of this retrospective study was to define the risk factors and prognosis in very low birth weight (VLBW) infants with gastric perforations. METHODS: VLBW infants with a diagnosis of gastric perforation between 2012 and 2016 were included. The data including birth weight, gestational age, gender, risk factors, time and location of the perforation and prognosis were recorded. RESULTS: A total of eight infants were identified. The median gestational age and birth weight of the infants were 26 weeks and 860 g, respectively. Five were male and 6 (75%) had a diagnosis of hemodynamically significant patent ductus arteriosus (PDA), early sepsis, persistent hypotension, and drug administration (paracetamol, ibuprofen). The main clinical finding was abdominal distension and pneumoperitoneum was detected in all infants. The median diagnosis was 6 days of life. The median perforation size was 2.5 cm and curvature major and anterior wall were the most common locations. The mortality rate was 62.5%. CONCLUSION: Male gender, chorioamnionitis, early sepsis, asphyxia, hemodynamic PDA, persistent hypotension, ibuprofen and paracetamol usage, and orogastric catheter administration were the main risk factors for gastric perforations in VLBW infants.

Vitelline duct pathologies in neonates.

OBJECTIVE: The aim of this study was to review the management of pediatric cases of vitelline duct pathology (VDP) detected surgically or incidentally during the neonatal period and the outcomes. METHODS: The data of newborns who were symptomatic and underwent VDP resection or who were incidentally diagnosed with VDP at a single institution between 1985 and 2015 were retrospectively analyzed in terms of age, sex, clinical features, treatment, perioperative findings, ectopic tissue pathology, and postoperative follow-up information. RESULTS: Among the 36 newborns enrolled in this study, 26 were male and 10 were female (2.6:1). The median weight was 2400 g (range: 800-3090 g). In 16 cases (14 males and 2 females; 7:1) the VDP was surgically repaired. Pathological evaluation indicated that 43% (n=7) of the cases had ectopic gastric mucosa. VDP was incidentally discovered in 12 males and 8 females (1.5:1). VDP was removed in 10 cases (50%) and left intact in others. Ectopic gastric mucosa was observed in 10% of the VDP removal cases. Ectopic gastric tissue was more prevalent in the surgical VDP cases than in the incidentally discovered and VDP removal cases (p<0.05). Male predominance was greater in the surgically repaired cases than in the incidentally discovered cases (p<0.05). One patient whose VDP was discovered incidentally was admitted 3 years later with obstruction due to intussusception caused by Meckel's diverticulum, and 1 patient was admitted with rectal bleeding at 11 years of age. CONCLUSION: Symptomatic VDP in the newborn demonstrates a significant gender difference. Symptomatic cases are more likely to have ectopic gastric tissue than non-symptomatic cases. Incidentally detected cases without removal should be followed closely for future complications.

Unusual Presentation of Duplex Kidneys: Ureteropelvic Junction Obstruction.

Aim. Ureteropelvic junction obstruction (UPJO) is rarely associated with a duplex collecting system. We review this unusual anomaly in terms of presentation, diagnostic evaluation, and surgical management. Method. We retrospectively reviewed the medical records of patients diagnosed with a duplex system with UPJO. Result. Sixteen patients (6 girls, 10 boys) with 18 moieties were treated surgically and four patients were treated conservatively. The median age at surgery was two years (range, 2 months to 7 years). The lower pole and upper moiety were affected in 12 and two kidneys, respectively, and both were affected in two patients. The anomaly was right-sided in 12 moieties and left-sided in six. The duplication was incomplete in seven patients and complete in nine. The mean renal pelvis diameter at the time of surgery was 25.6 (range 11-48 mm) mm by USG. The mean renal function of the involved moiety was 28.3% before surgery. Management included pyelopyelostomy or ureteropyelostomy in six moieties, dismembered pyeloplasty in eight moieties, heminephrectomy in four cases, and simultaneous upper heminephrectomy and lower pole ureteropyelostomy in one patient. Conclusion. There is no standard approach for these patients and treatment should be individualized according to physical presentation, detailed anatomy, and severity of obstruction.

Increasing the Reliability of the Grading System for Voiding Cystourethrograms Using Ultrasonography: An Inter-Rater Comparison.

BACKGROUND: To assess the effectiveness of the current vesicoureteral reflux (VUR) grading system according to the international classification of VUR (ICVUR) and to evaluate whether VUR grading accuracy could be improved by renal ultrasonography (RU) according to the Society for Fetal Urology (SFU) grading system. OBJECTIVES: Therefore, this study assessed the accuracy of the current VCUG staging system by assessing inter-rater reliability among pediatric radiologists and urologists; it also evaluated whether accuracy is increased by RU without consensus (with respect to VCUG grading). METHODS: Four pediatric urologists and four pediatric radiologists independently graded 120 voiding cystourethrograms (VCUGs). Middle VUR grades were divided into the following three groups: VUR consensus grade III (group 1), VUR consensus grade IV (group 3), and VUR non-consensus grades III and IV (group 2). All groups were compared with respect to hydronephrosis grade using RU. RESULTS: The intraclass correlation coefficient (ICC) values ranging from 0.86 to 0.89 reflected good reliability. The lowest agreement was associated with middle grades (III and IV). A marked difference in sensitivity was observed between groups 1 and 3 (35% and 95%, respectively, P < 0.05), indexed by SFU hydronephrosis grade, suggesting that VCUG cases in group 2 (n = 16 at SFU 0 or 1) could be accepted as grade III, and SFU scores of 2, 3, or 4 could be considered grade IV. CONCLUSIONS: Inter-rater accuracy could be improved at middle grades using renal ultrasonography (USG), which could promote communication between different specialists.

Therapeutic approaches and long-term follow-up for prenatal hydronephrosis.

OBJECTIVE: This study summarises the outcomes of 149 patients who underwent surgery for antenatally diagnosed unilateral hydronephrosis. METHODS: The medical records of such patients over a 23-year period were reviewed retrospectively. Age at the time of operation, preoperative and postoperative mean pelvic diameter on ultrasound, split renal function, washout patterns on scintigraphy, and early and late complications were recorded. RESULTS: The mean preoperative follow-up period was five months (range: 1-66 months). One patient was operated on after 12 months and two patients after five years of follow-up. Mean preoperative pelvic diameter and renal function were 30.8 mm and 38.6%, respectively; all patients had an obstructive wash-out pattern. In the postoperative period, the corresponding measurements were 11.7 mm and 39.2%, with 111 non-obstructive, 24 partially obstructive, and 14 obstructive wash-out patterns. Three patients with severe caliectasis and low renal function underwent surgery despite mild hydronephrosis. The mean postoperative follow-up period was six (range 4-11) years. Complications developed in 14 (9.3%) patients. CONCLUSION: Patients with antenatal hydronephrosis may need surgery even after a follow-up period of six years. Because of the potential late development of complications, postoperative follow-up should be continued for 10 years.

A bladder diverticulum model in rabbits.

PURPOSE: Shuttling of some of the bladder volume into the bladder diverticulum (BD) can cause urinary retention, lower urinary tract dysfunction, infection, and stone formation. This experimental study is the first to create a rabbit BD to study micturition physiology (urodynamics and pathology) that mimics clinical findings. MATERIALS AND METHODS: The study included 16 New Zealand adult male rabbits in the BD group and 16 sham-operated controls. BD creation consisted of a lower midline laparotomy and bladder entry via the spacing between the detrusor muscle fibers and the mucosa, posterolaterally from the bladder wall. The detrusor was excised to provide a mucosal prolapsus, creating a narrow BD neck (Figure). The sham group underwent bladder exposure with a midline incision. All rabbits underwent urodynamic study preoperatively and postoperatively, consisting of postmicturition residue (PMR), maximum bladder capacity (MBC), voiding detrusor pressure (VPdet), filling detrusor pressure (FPdet), compliance, and urine flow (Qflow). The animals were then sacrificed and their bladders assessed for pathology and stone formation. RESULTS: Preoperative MBC, Pdet, and Qmax were within reference ranges. No animals had PMR or urinary tract infections (UTIs). The BD group showed urodynamic and pathologic bladder changes, including decreased (28%) cystometric bladder capacity and compliance (Sham: 26.8 ± 0.4; BD: 4.46 ± 1.08, p = 0.0001) and increased post-void residual PMR (8.3 ± 2.4 mL). Pathology revealed increased bladder detrusor thickness correlated with urodynamic findings of increased filling detrusor pressures (Sham: 1.58 ± 0.2; BD: 4.89 ± 0.93, p = 0.0001). Urodynamics revealed intermittent BD bladder contraction during the filling phases. Eight BD group rabbits had UTIs; five had stone formation (4-9 mm). DISCUSSION: In the literature, it has not been determined whether lower urinary tract disorders (LUTD) could cause diverticula, or if a congenital diverticula could be reason for LUTD. Anatomical or neurological reasons for a low-compliance bladder can cause diverticulosis. As demonstrated in our study with rabbits, the congenital diverticulum could further reduce the compliance of the bladder. Further, a decrease in compliance logically correlates with the progressive decompensation of the bladder. CONCLUSIONS: The nature of diverticula remains undetermined. All the information presented for this model is relevant to our clinical observations. We conclude that the rabbit bladder can be used for research into experimental diverticulum-induced changes in the activity of the bladder and for experimental detrusor research.

The effect of bladder diverticula on bladder function: An experimental study in rabbits.

OBJECTIVE: This study aims to evaluate the effect of bladder diverticula (BD) on bladder function using a rabbit model, based on urodynamic findings. METHODS: A total of 32 New Zealand white rabbits were randomly assigned to four groups: (1) sham surgery; (2) a single, 1-cm-diameter BD; (3) a single, large, 3-cm-diameter BD; and (4) four (multi) 1-cm-diameter BD. Urodynamic evaluations were performed preoperatively, and 1week and 1month postoperatively, to measure the postmicturition residual (PMR), maximum bladder capacity (MBC), filling and voiding detrusor pressure (Pdet), compliance, and unstable detrusor contractions. At the end of the study, the animals were sacrificed and assessed for pathologic evaluation and stone formation. RESULTS: In groups 3 and 4, the rabbits all had PMR. At 30days postsurgery, the MBC was found to be 28% and 31% lower than the reference range in groups 3 and 4 and compliance was decreased (p<0.05). Further, the filling Pdet was significantly higher for large and multiple BD than in the 1-cm diverticula or sham groups (6.33±1.73, 4±1.26, p=0.0001). Groups 3 (62%) and 4 (50%) had unstable detrusor contractions. There was also a muscular/collagen ratio increase in the large and multiple BD groups. Four rabbits in the large group and one rabbit in the multiple BD group exhibited stone formation. CONCLUSIONS: Large or multiple BD can alter bladder storage and emptying, and can decrease the capacity of the bladder and reduce its elasticity. Large or multiple bladder diverticula can lead to involuntary contractions, causing dysfunctional voiding.

Reliability of the Grading System for Voiding Cystourethrograms in the Management of Vesicoureteral Reflux: An Interrater Comparison.

Aim. Vesicoureteral reflux (VUR) is one of the most common conditions seen in pediatric urology. Fortunately, there are many treatment options for this disorder. The grading system for VUR varies among doctors, and the literature on its reliability is sparse. Here, we assessed the effectiveness of the current VUR grading system. Methods. A series of 40 voiding cystourethrogram (VCUG) studies were selected. Four pediatric urologists (PU) and four pediatric radiologists (PR) independently graded each VCUG and then agreed on a uniform interpretation. For statistical analysis the intraclass correlation coefficient (ICC) was applied to assess interrater agreement. Results. ICC values ranging from 0.82 to 0.88 reflected the strong reliability of VCUG for grading cases of VUR among pediatric urologists and radiologists as separate groups, and the reliability between the two groups was also good, as indicated by an ICC of 0.89. Despite the high ICC, disagreement existed between raters; the lowest agreement was associated with middle grades (III and IV). Conclusions. The interrater reliability of the international grading system for VUR was high but imperfect. Thus, grading differences at middle grades can profoundly influence the type of treatment pursued.

Congenital Prepubic Sinus with Remnant Tissue Mimics Corpus Spongiosum: A Rare Case.

A congenital prepubic sinus is a tract that originates in the skin and overlays the base of the penis; however, its embryologic basis is still debated. We herein present a case involving a 3-year-old boy who was admitted for examination of overlying tissue located a few centimeters distal to the dorsal base of the penis. Examination revealed a tiny sinus in the prepubic area, and 3 cm of tissue was attached to the sinus. Pathologic examination showed that the tissue was lined with squamous epithelium and continued along the sinus tract, which was lined with urothelial epithelium. According to Stephens' classification, the sinus appeared to be a variant of type 2 dorsal urethral duplication, and the remnant tissue mimicked the corpus spongiosum of the penis.

Metabolic disorders in Turkish children with urolithiasis.

OBJECTIVE: To review metabolic disorders in Turkish children with urinary tract stone disease. MATERIALS AND METHODS: The medical records of 308 pediatric patients with the diagnosis of urolithiasis between 1996 and 2008, whose disease progression was followed in a single tertiary-care center, were reviewed retrospectively. Two hundred forty-eight patients whose metabolic analyses were performed were included in the study. RESULTS: Of the 248 patients participating in the study, 142 (57%) were men and 106 (43%) were women. The median age of the patients was 48 months (minimum-maximum, 2-180 months). Seventy-six percent of the patients had metabolic disorders. Of all patients, 44% had 1, 23% had 2, and 7% had 3 metabolic disorders. Hypercalciuria, hypocitraturia, hyperoxaluria, hyperuricosuria, and cystinuria were detected in 41%, 39%, 22%, 9%, and 4% of the patients, respectively. The rate of multiple stone formation, infection, and recurrence was significantly higher in the 0-2 years age group (P = .030, P = .001, P = .019, respectively). The median age of patients was greater (P = .001) in patients with hyperoxaluria in comparison with other metabolic disorders. Compared with other metabolic disorders, multiple stones and recurrence were more frequent in patients with cystinuria (P = .022 and P = .008, respectively). The size of the stones was greater in patients with hyperuricosuria in comparison with other metabolic disorders (P = .009). CONCLUSION: The majority of children with urinary tract stone disease exhibited ≥1 metabolic risk factors. Metabolic risk factors should be evaluated in all children with urinary stone disease to provide appropriate treatment.

Treatment of spontaneous rupture of lung hydatid cysts into a bronchus in children.

PURPOSE: While several publications have reported the treatment of ruptured lung hydatid cysts (HC) in adults, there is limited information on the treatment of children. This study summarizes the treatment approach and outcomes of 11 children with spontaneous rupture of lung HCs into a bronchus. METHODS: The complete medical records of 11 patients with spontaneous lung HC bronchus rupture between March 1993 and April 2012 were examined retrospectively. Gender, age at the time of diagnosis, patient symptoms, lung localization, dimensions of the cyst, medical treatment duration, and associated morbidities were evaluated. Routine chest x-rays, ultrasonography, computed tomography and serological tests were used. RESULT: The study included 11 patients [4 males, 7 females; average age 7.45 (range 5-11) years] presenting to the Emergency Department. The symptoms at the time of presentation were coughing in 81.8% (n=9), hemoptysis in 54.5% (n=6), and chest pain in 54.5% (n=6). The average cyst diameter was 8.71(35-15) cm. The rupture involved the left lung in 7 (63.6%) patients and the right in 4 (36.4%). Serological tests were positive in seven patients and all patients had eosinophilia. The treatment of one patient was discontinued, while all of the other patients were treated medically. Pneumonia developed in four patients and lung abscesses in two. Both patients who developed lung abscesses had cysts with diameters greater than 10 cm. The average follow-up period was 48.4 (range 15-85) months; no mortality occurred. CONCLUSION: In our experience, medical treatment is adequate for patients with lung HCs progressing to spontaneous bronchus rupture. There was a high rate of abscess development within the thoracic cavity in patients with cysts over 10 cm in diameter; these patients can be treated effectively with a medical approach.

Misdiagnoses caused by use of indwelling urethral catheters in children with ureterovesical junction anomalies.

PURPOSE: Children commonly undergo vesicograms for diagnosing vesicoureteral reflux (VUR). This requires urethral catheterization with transurethral replacement. We report misdiagnosed or related complications due to indwelling urethral catheters unintentionally placed in the ureter. METHODS: From our computerized urology records over an 18-year period from January 1995 to May 2013, we retrospectively identified nine cases of 1850 vesicograms that had misdirection of a urethral catheter placed in a ureter. Foley catheters with inflating balloons were used to obtain the vesicograms. RESULTS: In all, 1850 vesicograms were performed (746 males, 1104 females; age 1 week to 14 years, mean age 3.8 years) using standard radiological techniques. Size 6-10 Fr indwelling urethral catheters were used, depending on the patient's age and gender. In nine cases (five females, four males), a misdirected urethral catheter was discovered in one of the ureters. The urethral catheter was in the left ureter in four patients and in the right ureter in five patients. Cystoscopic examination found ectopic ureteral openings in six patients: at the bladder neck in four and just below the bladder trigone in two. Three patients in this group with ectopic ureters were followed due a misdiagnosis of VUR. The remaining three patients had grade 3 or 4 VUR. In this group, the catheter passed into the ureter because of the enlarged ureterovesical junction. In one patient with VUR, intraparenchymal fluid leakage and transient hematuria occurred due to the rapid tension increase following the fast injection of contrast with liquid to one ureter. CONCLUSION: Although placing an indwelling urethral catheter is a relatively safe procedure, complications can occur, particularly in patients with ureterovesical anomalies, such as high-grade VUR or an ectopic ureter. Using catheters with inflating balloons can cause rapid increases in tension in the ureter, and related complications.

Renal outcome of children with horseshoe kidney: a single-center experience.

OBJECTIVE: To assess the clinical features, additional abnormalities, and renal consequences of children with horseshoe kidney (HSK). METHODS: The medical records of 41 children with HSK followed in our pediatric nephrology clinic between the years of 2004 and 2014 were retrospectively reviewed. RESULTS: There were 22 girls (53.6%) and 19 boys (46.4%) aged 60 (2-192) months. The median follow-up time was 48 (12-120) months. HSK was incidentally found in 12 patients (29.3%), and 5 patients (12.2%) were prenatally diagnosed. Urinary tract abnormalities including vesicoureteral reflux (n = 8), ureteropelvic junction obstruction (n = 7), and duplex system (n = 3) were identified in 18 patients (43.9%). Eight children (19.5%) had bladder dysfunction, and 9 patients (21.9%) had additional systemic abnormalities. Urinary tract infection had been detected in 17 patients (41.4%). Seven patients (17%) required surgical intervention including pyeloplasty (n = 3), ureteroneocystostomy (n = 3), and upper pole heminephrectomy (n = 1). Renal scarring (RS) occurred in 10 patients (24.4%). Six patients (14.6%) developed proteinuria and 4 (9.8%) complicated with hypertension. Three patients (7.3%) progressed to chronic kidney disease (CKD). Multivariate logistic regression analysis showed that proteinuria, hypertension, and RS are independently associated with CKD. CONCLUSION: Children with HSK might be examined for additional urologic and nonurologic abnormalities. Patients might be closely followed up for the increased risk of urinary tract infection and RS. Proteinuria, hypertension, and presence of RS seem to be the major determinants for progression to CKD in children with HSK.

Current diagnosis and management of simple hepatic cysts detected prenatally and postnatally.

PURPOSE: The optimal management of simple hepatic cysts (SHCs) remains controversial. A retrospective review was carried out to evaluate the clinical presentation and surgical consideration of children with SHCs and outcome of management at our institution. METHODS: We performed a retrospective review of all patients diagnosed with SHCs between January 1993 and May 2013. Patient variables included prenatal findings, age, gender, presenting symptoms, signs and diagnostic modality. Operative management and nonoperative follow-up were abstracted. RESULTS: The 67 patients comprised 42 females and 25 males: 16 had prenatally detected, and 51 had postnatally detected hepatic cysts with a mean follow-up of 3.2 years (10 months-12 years). The average age was 5.9 years (0-17 years). Ten patients (six females and four males) were treated surgically. Fifty-seven asymptomatic patients were observed. Of those 57 patients, 9 (17%) had enlarged cysts, and 1 (2%) had clinical signs. The average enlargement time was 36 months (6-67 months). The cysts occurred in the right lobe in 47 patients, in the left lobe in 17 patients and in both lobes in 3 patients. The median cyst size for the asymptomatic cases was 2.1 cm (1.5-6 cm) and 13.7 cm (8-25 cm) for the surgery group. A statistically significant difference in cyst size was found between these two groups (p < 0.05). Prenatally detected cysts (n = 5) were subjected to surgery postnatally at an average age of 1.5 months (1 week-6 months). The main indication was the compressive effect of the cyst on the surrounding area. There was one case of hepatomegaly found to be causing pressure symptoms, two cases of respiratory distress, one case of hydroureteronephrosis, and one case of jaundice due to compression of the biliary system. Five patients underwent surgery in the postnatally detected group, and the main indication was a complication of the cyst or progressive enlargement. Symptoms included abdominal discomfort and pain in all five patients: one case exhibited a hemorrhage within the cyst, one case displayed a rupture with possible peritonitis, one case had a 25-cm cyst leading to secondary infection, and two cases were admitted with progressive enlargement with clinical symptoms. CONCLUSION: Conservative management of SHCs is practical. Surgical excision is recommended in patients with larger cysts, causing pressure symptoms, or if the cyst size does not seem to involute with intractable abdominal pain or becomes complicated in the follow-up period.

Surgical interventions in childhood rare factor deficiencies: a single-center experience from Turkey.

Congenital rare factor deficiencies may present in infancy by life-threatening bleedings or may not show any symptoms until adulthood. It is reported more commonly in countries having consanguineous marriages. Data regarding surgical interventions of rare congenital factor deficiencies are based on case reports and records of guidelines. There are no well documented and separately prepared directories related to pre-surgical and prophylactic approaches of surgical interventions of these deficiencies. Our retrospective study consisted of 171 rare factor deficiencies that were followed up in our clinic, and of whom 61 had 88 surgical interventions between 1990 and 2012. Of these patients, 45 were having factor VII deficiency, and factor V, X, XI, XIII and fibrinogen deficiencies were present in five, four, three, two and two patients, respectively. In 23 patients, factor coagulant activities were under 5% (37.7%), in 15 it was between 5 and 30% (24.6%), and in 23 between 30 and 50% (37.7%). Twenty-eight were symptomatic and 33 were asymptomatic. Information of 51 (83.6%) male and 10 (16.4%) female patients with an age range of 5-25 years (13 ±â€Š5.27), whose age at presentation ranged between 3 weeks and 18 years (7 ±â€Š4.66), were retrieved from patient records and from the records contained in the data-processing environment introduced in 2005. The rate of familial consanguinity was 49.2%. Of the surgical interventions, 24 (27.3%) were major, 24 (27.3%) were minor and 40 (45.4%) were circumcision. We used fresh frozen plasma in 32, recombinant factor (rF)VIIa in 20, prothrombin complex concentrate in five and fibrinogen in three patients during surgical interventions. In 18 patients, antifibrinolytic agents were also used. In 27 patients, surgical interventions were applied without any replacement therapy. No additional doses were required after surgical prophylaxis doses. Thrombotic events were not observed. Antibody occurrence was not detected in these patients. In our study, we evaluated preparation for surgical procedures, factor replacement therapy before surgical intervention and postoperative follow-up in patients with rare coagulation factor deficiency.

Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Fetal-neonatal ovarian cysts--their monitoring and management: retrospective evaluation of 20 cases and review of the literature.

OBJECTIVE: Neonatal ovarian cysts (NOC) are usually self-limiting structures. However, large or complex cysts may lead to severe complications. A standard guide to management, treatment and follow-up of NOC is not yet available. The aim of this study was to evaluate retrospectively the records of NOC patients from two medical centers. METHODS: A total of 20 newborns with NOC were included in the study. The size and localization of the cyst, the age, the signs and symptoms at presentation, and the possible maternal and fetal-neonatal etiologic factors were recorded. Follow-up procedures and treatment modalities were evaluated. RESULTS: The mean age at diagnosis was 34 gestational weeks. The cysts (mean size 53±15 mm) were predominantly in the right ovary (75%) and were evaluated as large cysts in 16 (80%) of the patients. In 5 of the patients with large cysts and in 1 of the 4 patients with small cysts, the cysts were evaluated as complex cysts. Torsion of the ovary was detected in five (25%) cases and these cases were treated surgically. Patients with simple cysts were closely followed by ultrasonography until the cysts disappeared. CONCLUSION: To date, there is no precise guide for the monitoring and treatment of NOCs. Surgical treatment should always be performed in a way to protect the ovaries and to ensure future fertility. In our NOC series, it has been possible to apply a non-invasive follow-up program and minimally invasive surgical procedures.