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.

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.

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.

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.

Tubularized incised plate urethroplasty for hypospadias reoperations in 100 patients.

OBJECTIVES: To determine the key points for a successful redo hypospadias procedure using tubularized incised plate urethroplasty operation. METHODS: A retrospective chart review of a cohort of 100 patients (Mean age: 4.5 years, range: 2-12) who had undergone a redo tubularized incised plate urethroplasty operation was performed. Fischer exact and Chi square tests were used for statistical analysis. RESULTS: The incidence of complications of tubularized incised plate urethroplasty reoperation after failed repairs of meatal advancement and glanuloplasty procedure (n: 14), meatal based flap (n: 36), and tubularized incised plate urethroplasty (n: 50) were 29% (n: 4), 22% (n: 8) and, 28% (n: 14) respectively (P > 0.05). The overall complication rate was 26% (n: 26). Eighteen patients (18%) had fistula, five had meatal stenosis (5%), two had dehiscence (2%) and one had neourethral stenosis (1%). Postoperatively, fistula was closed in 18 patients and 5 underwent meatoplasty. Two patients with dehiscence and one with neourethral stenosis underwent an unsuccessful third redo tubularized incised plate urethroplasty reoperation and they were candidates for a complex hypospadias repair (3%). The ultimate success rate of tubularized incised plate urethroplasty reoperation after treatment of complications was 97%. CONCLUSION: Tubularized incised plate urethroplasty is a safe and efficacious alternative procedure for hypospadias reoperations if the urethral plate has no scars and outcome is favourable if the first failed hypospadias repair is a meatal based flap procedure. The complication rate increases if the urethral plate has been previously incised in the midline and a redo third redo must be avoided.

An unusual complication of familial Mediterranean fever: intestinal volvulus and necrosis.

Familial Mediterranean fever is an autosomal recessive disease characterized by recurring inflammatory attacks of synovial membranes. More than 95% of patients show peritoneal involvement which mimics acute abdomen and can sometimes cause unnecessary surgical intervention. The authors present two patients with the diagnosis of familial Mediterranean fever who underwent surgery because of rare abdominal complication of the disease. Two patients with the diagnosis of familial Mediterranean fever underwent laparotomy, and segmental small bowel resection was done because of the necrosis. Adhesive intestinal obstruction with associated bowel strangulation and volvulus is a rare complication of familial Mediterranean fever, and this life-threatening emergency must be kept in mind.

Testicular volume before and after hydrocelectomy in children.

OBJECTIVE: The purpose of this study was to investigate whether there is an association between communicating congenital hydroceles and testicular volume in children. METHODS: Seventeen children with unilateral communicating congenital hydroceles who underwent hydrocelectomies were included in the study. Testicular volumes were measured before and after hydrocelectomy on both the affected and unaffected sides. RESULTS: We found statistically significant differences in testicular volumes between the normal sides (mean +/- SD, 0.62 +/- 0.24 mL) and the sides with hydroceles (0.72 +/- 0.26 mL) before surgery (P < .001) and in testicular volumes of the sides with hydroceles before (0.72 +/- 0.26 mL) and after (0.60 +/- 0.19 mL) surgery (P < .001). There was no significant difference in testicular volumes before (0.62 +/- 0.24 mL) and after (0.62 +/- 0.21) surgery on the normal sides (P = .978). The mean decrease in volume of the testes with hydroceles after hydrocelectomy was approximately 15%. CONCLUSIONS: This study shows that there is an association between congenital communicating hydroceles and testicular volume in children. Clinicians should be aware that there is a decrease in testicular volume after hydrocelectomy so they do not misdiagnose this change as postoperative trauma or atrophy.

An audit on pediatric hydatid disease of uncommon localization: incidence, diagnosis, surgical approach, and outcome.

BACKGROUND/PURPOSE: Infestation of sites other than the liver and lungs by the parasite Echinococcusgranulosus is rarely encountered in clinical practice. The present study aims to determine the incidence of hydatid disease of uncommon localization in children and to document the clinical and radiologic findings, the types of the operations performed, and the postoperative course of the disease. METHODS: A retrospective review of the demographical data as well as preoperative and postoperative clinical findings of children who underwent surgical treatment of hydatid disease was done. RESULTS: Hydatid disease of uncommon localization was encountered in 15 (7%) of 207 children reviewed. The sites in which the cysts were located were as follows: spleen (n = 4, 1.9%), kidney (n = 4, 1.9%), retroperitoneum (n = 3, 1.4%), omentum (n = 2, 1%), anterior abdominal wall (n = 1, 0.5%), and anterior thigh (n = 1, 0.5%). Radiodiagnostic tools such as ultrasonography were used in all. Partial pericystectomy was the surgical procedure of choice in most cases. There was 1 recurrence in a patient with splenic disease, with no mortality. CONCLUSIONS: Pediatric hydatid disease can involve any body part. Radiologic imaging techniques are helpful in diagnosis. Surgery should aim to preserve as much tissue as possible while precautions to prevent recurrences are undertaken.

Urinary tract involvement in a child with epidermolysis bullosa simplex.

Epidermolysis bullosa is a rare disorder which may present various systemic involvement. We present a 15-month-old boy with epidermolysis bullosa simplex who was admitted with symptoms of hematuria and dysuria and discuss the pathology from a urological standpoint.

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst.

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.

Tubularized incised plate urethroplasty: 5 years' experience.

OBJECTIVE: To evaluate the results of tubularized incised plate (TIP) urethroplasty in a series of primary hypospadias repairs. PATIENTS AND METHOD: The medical records of 360 children (Mean age: 4.3 years, range: 2-14 years) with primary hypospadias undergoing TIP urethroplasty repair and treatment of complications were reviewed. A good result is a direct urinary steam through a slit and vertically oriented meatus and mild angled urinary stream through a slightly regressed meatus is a satisfactory result. RESULTS: The sum of good (74%) and satisfactory results (3%) was the overall success rate (77%) which was increased to 95% after a second surgical procedure for the treatment of complications. In 30 patients with single fistula (8%), fistula healed spontaneously in 2 patients and 20 patients had successful fistula closure. Redo TIP urethroplasty (n = 30) was performed for dehiscence (n = 15, 3%), neourethral stricture (n = 3, 1%) and multiple fistula with meatal stenosis (n = 12, 3%). The complication rate after redo TIP urethroplasty is 30%. Twenty-four (7%) patients underwent meatoplasty for meatal stenosis. CONCLUSION: TIP urethroplasty can be used to repair primary hypospadias. After a learning curve and attention to surgical details, cosmetic and functional outcome become excellent. Redo TIP urethroplasty can be performed in the treatment of complications.

Bronchogenic cysts: a rare congenital cystic malformation of the lung.

PURPOSE: Bronchogenic cysts are rare congenital cystic malformations of the lung. We retrospectively analyzed ten cases of bronchogenic cyst (BC) to reinforce the importance of recognizing this malformation. METHODS: Between 1985 and 2000, ten pediatric patients with BC were treated surgically in our department. Their clinical presentation, radiological, operative, and pathological findings were analyzed retrospectively. RESULTS: There were five boys and five girls, ranging in age from 16 days to 6 years (mean 6.5 months). The clinical signs and symptoms included respiratory distress in seven patients (70%), cyanosis in four (40%), chronic cough and fever in five (50%), and dysphasia in two (20%). Routine chest X-ray revealed a pulmonary air-filled cyst in six patients (60%) and a pulmonary nodular opacity in four (40%). The diagnosis was supported by computed tomography in four patients and by ultrasonography in two. Eight of the patients were treated by cyst excision and two by lobectomy. The pathological diagnosis made from all specimens was bronchogenic cyst, with squamous metaplasia in two. CONCLUSION: In newborns, infants, and even children, the development of dyspnea, cyanosis, chronic cough, and fever should alert us to the suspicion of a cystic malformation in the lung, such as a bronchogenic cyst, especially if an air-filled cyst is seen on a plain chest X-ray.

The effect of pentoxifylline on intestinal anastomotic healing after ischemia.

The negative effects of ischemia-reperfusion injury (IRI) on intestinal anastomotic healing have been described in previous studies. To determine the effect of pentoxifylline (PTF) on small-bowel anastomoses performed after IRI, 20 male Wistar albino rats were studied. Under ketamine anesthesia, the superior mesenteric artery was occluded for 30 min and ileal resection-anastomosis was performed after 10 min of reperfusion. In group I, no medication was given. In group II, PTF 50 mg/kg was administered intraperitoneally before reperfusion and i.m. on the 1st, 2nd, 3rd and 4th postoperative days (POD). All rats were killed and anastomotic segments were removed on the 7th POD. Anastomotic bursting pressure (ABP) and hydroxyproline (HP) content were measured in the anastomotic segments; parameters were compared statistically with Student's t-test. In group I, the mean ABP was 225.5 +/- 38.18 mmHg the and mean HP level was 16.03 +/- 1.91 micro mol/g tissue. In group II, these levels were 277 +/- 41.1 mmHg and 19.85 +/- 1.81 micro mol/g tissue, respectively. The differences between the groups were significant (P < 0.005). Thus, PTF can improve anastomotic healing in an experimental model after IRI by promotion of collagen synthesis.