[Cystic mesenchimal hamartoma of the liver in two-year-old patient--case report].

Mesenchymal hamartoma is an uncommon benign hepatic tumor arising from the mesenchyme of the portal triad. This lesion is relatively uncommon, representing 5% of all primary hepatic pediatric tumors. This form of hamartoma usually presents before the age of 2 years, typically with abdominal swelling as the initial symptom. The classic management of these lesions has been excision either by hepatic lobectomy or wedge resection. We present a case of 2-year-old girl with a right hepatic lobe tumor, 66 x 57 x 71 in diameter that was completely removed by right hepatic lobectomy.

Gastroesophageal reflux in infants and children.

Gastroesophageal reflux disease (GERD) is the most common esophageal disorder and the most frequent reason why infants are referred to the pediatric gastroenterologist, affecting as much as 30% of the pediatric population. Presenting features of GERD in infants and children are quite variable and follow patterns of gastrointestinal and extra-esophageal manifestations that vary between individual patients and may change according to age. Patients may be minimally symptomatic, or may exhibit severe esophagitis, bleeding, nutritional failure, or severe respiratory problems. GERD is also complex for the diagnostic techniques required to assess its repercussions or explain its origin. Although different abnormalities in motility variables, such as lower eso-phageal sphincter (LES) function, esophageal peristalsis and gastric motor activity can contribute to the development of GERD, the degree of esophageal acid exposure represents the key factor in its pathogenesis. Esophageal pH monitoring, based on both the detection of acid reflux episodes and the measurement of their frequency and duration, has been regarded as the most sensitive and specific diagnostic tool for diagnosing reflux disease. The aim of this paper is to give a concise review for the clinicians encountering this specific disease in infants and children.

Continent appendicostomy in the treatment of fecal incontinence.

BACKGROUND: Fecal incontinence is common in children with anorectal anomalies or spina bifida. If it is possible to achieve fecal control, patients are given a large volume of enema once a day. Retrograde enemas are often unpleasant in children, particularly in adolescents. Malone procedure of antegrade appendicostomy achieves antegrade colonic irrigation. MATERIAL AND METHODS: From 1996 to 2003 Malone antegrade appendicostomy was performed in 10 patients with fecal incontinence. The patients were aged from 5 to 24 years. In 4 patients fecal incontinence was due to an anorectal anomaly, and in 6 patients spina bifida. Preoperatively, all patients were given a clysma to control fecal incontinence. The patients, who remained clean with regular usage of the clysma for 24 hours or longer, fulfilled the criterion for the formation of continent appendicostomy. In 9 patients the cecal appendix was used to create a stoma, while in another one a lateral tubularized cecal flap was applied. In 3 patients a continent conduit was also done due to urinary incontinence. The follow-up period was from 1 to 8 years. A patient was reoperated due to stenosis of the stomal aperture, while another one has not been using the stoma because of social reasons. CONCLUSION: Continent appendicostomy is a simple surgery, which is effective in the control of fecal incontinence in most children. It is indicated only in patients in whom the rerograde clysma successfully cleans the colon and if patient and parents are motivated to use it. Possible complications, among which stenosis is the most frequent, can be solved with a reoperation.

Surgical treatment of cloacal anomalies.

From 1989-1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1-8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry. PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Surgical treatment of the Müllerian duct remnants.

BACKGROUND: Persistent Müllerian duct tissue in male individuals may result in an enlarged prostatic utricle (utricular cysts and utricle) or a Müllerian duct cysts, either distinctively or synonymously. In intersex patients Müllerian duct remnants (MDR) are an usual occurrence. Surgical excision is the definitive treatment of symptomatic remnants, as well as during the reconstruction of intersexual genitalia. Many approaches have been described. The authors review their experience in intersex patients. METHODS: From 1986 to 1999, the authors treated 111 patients with intersex disorders. The records of 47 patients raised as boys with MDR were reviewed. Based on the symptoms and the size of the remnants, in 32 patients the structures were removed. In 13 patients extirpation was done by perineal approach, in 9 by transperitoneal approach, and in the remaining 9 patients the combined abdominal and perineal approach were undertaken. In one patient the large prostatic utricle was extirpated by a posterior sagittal pararectal approach. Perineal approach was mainly used in younger asymptomatic children, with the prostatic utricle disclosed incidentally during genitography because of intersex disorders. Operation was performed only in cases in which the prostatic utricle was observed by cystoscopy or identified by Fogarty balloon catheter introduction into the prostatic utricle. In older patients these structures were discovered frequently after failed urethroplasty, or after symptoms of urinary infection, urinary retention, or epididymitis. We elected to use the transperitoneal approach based on the extension of these structures into the pelvis. The average age of patients at the time of surgery was 8.6 years, with a range of 1 to 30 years. RESULTS: There were no rectal or bladder injuries during surgery. An older patient had temporary impotence after abdomino-perineal extirpation. The lack of ejaculation, seen in 5 patients, was related to frequent intra-utricular termination of the vas deferens. Posterior sagittal pararectal approach certainly enabled complete exposure and exact visualization of all structures, with considerably decreased bleeding. If gonadal biopsy or gonadectomy were necessary, the transperitoneal approach could not be avoided. CONCLUSIONS: Surgical treatment of MDR in intersex patients varies according to the size of the utricle, and a double approach is often necessary. A high degree of success may be achieved with minimal morbidity. J Pediatr Surg 36:870-876.

[Ultrasound in the diagnosis of hypertrophic pyloric stenosis]. / Ultrasvuk u dijagnostici hipertroficne stenoze pilorusa.

The paper deals with the advantages of ultrasound diagnosis of hypertrophic pyloric stenosis in newborns. Ultrasound decreases the number of radiological examinations of gastroduodenum and reduces diagnostic exposure of children to X-rays. In the last three years 107 children had manifested clinical signs of hypertrophic pyloric stenosis. The ultrasound studies revealed hypertrophic pyloric stenosis in 55 patients (51.4%). There were 48 boys (87.3%) and 7 girls (12.7%). Patients were aged 17 to 75 days (average about 40 days). The sonogram finding was typical for hypertrophic pylorus, which made the diagnosis easier. There were no false positive or false negative ultrasound findings. All patients with hypertrophic pyloric stenosis were operated on and diagnosis was confirmed. Ultrasound is reliable in diagnosis of hypertrophic pyloric stenosis. Ultrasound examination is technically easy and fast, what is important for urgent surgical therapy. In children with persistent projectile vomiting, suspected of hypertrophic pyloric stenosis, ultrasound is the method of choice and should replace X-ray examinations.

[Intestinal intussusception in children. Ultrasonic diagnosis]. / Invaginacija intestinuma kod dece. Dijagnostika ultrazvukom.

The aim of the paper is to demonstrate a successful use of ultrasound in the diagnosis of intestinal intussusception in children. Ultrasound decreases the number of irrigographic examinations and reduces diagnostic exposure of children to X-rays. In the last three years 35 children, aged from 3 months to 15 years (average 2 years), had a suspected clinical diagnosis of intussusception. The ultrasound studies revealed intestinal intussusception in 26 patients (74%). There were no false positive or false negative ultrasound findings. In four patients with secondary intussusception the main symptoms were identified (three solid lesions and two Meckel's diverticula). Intraluminal lesions at the apex of intussusception were confirmed by surgery. In 22 patients intussusception was idiopathic. In 15 of these patients (68%), hydrostatic desinvaginations, under combined ultrasound and radioscopic control, were successful. High grade unsuccessful hydrostatic reductions were associated with long persistence of symptoms (2 to 9 days). Ultrasound is reliable in diagnosis of intestinal intussusception and useful in control of hydrostatic reduction. In patients with expected intestinal perforation ultrasound should be combined with fluoroscopy.

True hermaphroditism: 10 years' experience.

True hermaphroditism (TH) is the rarest variant of intersex malformations. By definition, these patients have both ovarian and testicular tissue. Most present due to ambiguous genitalia and/or gynecomastia. From 1986 to 1996, we treated 97 patients with intersex disorders; there were 4 with TH. In all cases testicular and ovarian tissue was separate. In 3 patients the external genitalia were ambiguous and 1 had hypoplastic male genitalia. Three patients had a 46, XY and 1 a 46, XX karyotype. Three patients had been listed as males and 1 as a female. The number of operations required varied from 3 to 9 (mean 5.7 per patient). Two patients who had been raised as males finished puberty with hypogenitalism and hypoplastic testes. One married and could have erections, but no ejaculation. Two male patients have been on supplementary androgen therapy. The only patient who had been raised as a female after clitorovaginoplasty had a 6-cm-deep, blind-ending vagina that was deepened to 12 cm with bougienage, so that she was capable of having normal intercourse. It is felt at present that most patients with TH should be raised as females. This was confirmed in our patients as well, which underlines the significance of early diagnosis.

[Adrenal gland hemorrhage in neonates--radiologic aspects]. / Hemoragija u nadbubreznoj zlezdi kod novorodjenceta--radioloski aspekt.

INTRODUCTION: The relatively large adrenal glands of the newborn are vulnerable to mechanical trauma during delivery. Great birth weight, difficult labor, perinatal hypoxia and prematurity are predisposing factors of adrenal haemorrhage. Minor adrenal haemorrhage may not cause symptoms. Massive adrenal haemorrhage is uncommon. Symptoms include anaemia and jaundice associated with a suprarenal mass. In cases with severe blood loss acute shock may develop. In 5 to 10 per cent of cases the haemorrhage is bilateral. Ultrasonography has replaced urography in the diagnosis of this condition demonstrating the site and size of the lesion and allowing an accurate follow-up. Within a month after haemorrhage the blood and necrotic adrenal tissue are resorbed and thin calcification appears at the periphery of the gland. Surgery is necessary if haemorrhagic pseudocyst is large and does not resorb spontaneously. MATERIAL AND METHODS: From 1992 to 1996, five patients with neonatal adrenal haemorrhage were treated at the University Children's Hospital in Belgrade. Two of them were females. All patients were born at term by vaginal delivery. Their birth weights ranged between 3200 and 4050 g. At hospitalization infants were aged from 6 hours to 18 days. The first symptom of adrenal haemorrhage was an abdominal mass in three patients. One of them had laparoschisis with guts and stomach protruding out; the surgeon discovered a mass in the right retroperitoneum during operation. Two patients had jaundice associated with anaemia, and sepsis another two. Ultrasonography was done in all patients. We punctured the haemorrhagic pseudocyst (diameter above 5 cm) in three patients and made cystography. Liquid components of pseudocysts were aspirated and sent to bacteriological and cytological analyses. RESULTS: The diagnosis of adrenal haemorrhage was confirmed by ultrasonography in all patients, demonstrating a right adrenal mass (unilateral in all patients), mostly hypoechoic, which displaced the right kidney. Calcification at the periphery of the pseudocyst appeared in one patient. The adrenal haemorrhage disappeared spontaneously in two patients after two months. An attempt to support the adrenal hemorrhagic pseudocystic regression by puncturing and aspirating its content in three patients was successful in one infant. The patient with laparoschisis died because of sepsis and thrombocytopenia. In a patient the haemorrhagic pseudocyst persisted (6 cm in diameter) and was surgically removed. DISCUSSION: Ultrasound is the method of choice in the diagnosis of adrenal haemorrhage, antenatally and neonatally. It also allows diagnosis of coexisting complications such as renal vein or inferior vena cava thrombosis and a proper follow-up. Puncture of pseudocyst and aspiration of liquid components may support involution of large haemorrhagic pseudocysts. If it is unsuccessful, surgery is necessary.

[Surgical treatment of vesicoureteral reflux by tunnelling of the detrusor muscles]. / Hirursko lecenje vezikureterskog refluksa metodom tunelisanja misica detruzora.

Detrusor tunnelling is an extravesical surgical procedure of ureteral reimplantation. It is surely the least traumatic method of surgical correction of vesico-ureteral reflux because antireflux mechanism is achieved with minimal detrusor muscle dissection. This procedure simplifies implantation of dilated ureters, double ureters as well as the ureters shortened by previous antireflux surgical procedures. Ureteral tapering is unnecessary. One hundred and forty detrusor tunnelling uretero-cysto-neostomies in 102 patients have been performed in the University Children's Hospital between January 1990 and September 1994. The youngest patient was 1 month old and the eldest was 15 years (mean age 6.8 years). Indications for surgical treatment were primary (45.0%) and secondary (26.4%) megaureter as well as grade IV & V (international classification) vesico-ureteral reflux (28.6%). Follow up was 1-40 months (mean 26 months). Excellent postoperative results were achieved in 130 detrusor tunnelling uretero-cysto-neostomies, i.e. more than 90% of patients. Complication rate was 7.1% (6 patients-10 ureteral units) and about one third were classified as major-postoperative relapses or stenosis or minor-intraoperative mucosal perforation, hematuria, parahiatal diverticulum, spontaneous proximal migration of ureteral double J stent-about two thirds.

Surgical treatment of intersex disorders.

Despite the progress made in understanding the factors regulating sexual differentiation, infants born with ambiguous genitalia face significant problems. The authors reviewed a group of 84 children with ambiguous genitalia managed surgically between 1986 and 1993. The most frequent condition was male pseudohermaphroditism (PM) (58%); 31% had female pseudohermaphroditism. Fifty-seven percent of patients were raised as males and 43% as females. In each group of patients, feminine and masculine reconstructive operations were performed. In only 31% of PM and 60% of PF cases was the diagnosis made within the first 2 months of life. In 41% of PF and 40% of PM patients, treatment was begun before the second year of life, which we consider an acceptable time. The timing and type of vaginoplasty were determined by the point of entry of the vagina into the urogenital sinus. Of the 29 patients reared as females, 22 required perineal vaginoplasty, had pull-through vaginoplasty, and 2 had colovaginoplasty. Since 1986, we have applied Mollard's clitoroplasty, which preserves the neurovascular bundle and is important for experiencing orgasm. Seventeen percent of patients with feminization procedures experienced complications. The optimal time for masculinization procedures is 2 years of age, after obligatory testosterone treatment. If there is utriculus prostaticus (UP) type II or III, it is removed before urethroplasty. This is not done for UP types 0 and 1. In PM cases, the number of feminization and masculinization operations was 2.1 and 4.05 per patient, respectively. It is easier to make a vagina than a phallus, not taking into consideration dimensions, aesthetics, or capability of erection of the phallus.(ABSTRACT TRUNCATED AT 250 WORDS)

Modified orchiopexy: a "real dartos pocket".

A modified technique of surgical treatment of undescended testes, termed the "real dartos pocket", is presented. The method may be briefly described as transinguinal orchiofuniculolysis, utilizing internal fixation of the tunica vaginalis and/or the gubernaculum to the everted dartos pocket (the tunica dartos of the septum being also grasped), with application of an additional inguinoscrotal incision, and cordopexy, if necessary. The narrowing of the opening on the Colles' fascia cranially, and on the tunica dartos at the scrotal entrance caudally, through the inguinoscrotal incision, are additional safeguards against retraction of the testis. Eighty-two patients underwent surgery, using the new technique in its present form. The patients were under follow-up from 5 to 41 months, and results have been anatomically and cosmetically excellent. In our experience, all undescended testes with an adequate length of the cord and mild to moderate tension, may be treated using our modified technique, with excellent anatomic and cosmetic results.

[Central venous access: our experience with Hickman and Broviac catheter in children]. / Centralni venski prisup--nasa iskustva s primenom katetera tipa Hikman-Brovijak kod dece.

We report the results of a 4-years-experience with Hickman-Broviac double lumen silicone catheters. The catheters were inserted in 51 dialysed and nondialysed children. Forty three catheters were placed for temporary or permanent dialysis access, 2 in patients for plasmapheresis, 4 in severely burnt patients, and 2 in neonates with the "short gut" syndrome. The youngest patient was 7 days old and the eldest 14.5 years (mean age 5.4 years). The catheters were implanted by open surgery into the right atrium through the right (92%) or left internal jugular vein (8%) in more than 80% of patients. Ten catheters were implanted in the femoral vein (three through the saphenous vein: in a patient with extensive burns in the thorax and neck area, and in seven patients with acute renal failure and high risk for anaesthesia). The complications, except the insufficient flow, were fewer and less dangerous than those reported in literature. Therefore, we strongly recommend insertion of these catheters as the method of choice for immediate vascular access in children in whom the creation of conventional vascular access is difficult or impossible, as well as in patients in whom provision of long- term intravenous nutrition or medication is essential.

[Personal experience with the use of percutaneous nephrostomy in children]. / Nasa iskustva sa primenom perkutane nefrostome kod dece.

Over a 3-year period 52 percutaneous nephrostomies (PCN) were performed in 37 children. The youngest patient was 2 months old, the oldest was 16 years. The primary indication was the obstruction of the upper urinary tract; infection in 25 patients and renal failure in 14 patients were evident. By posterolateral approach a sonographically guided antegrade pyelography was first performed with Chiba 22-gauge needle. Under fluoroscopic control a lower polar calix was punctured with 18 G sheathed needle; a guide wire was introduced through the sheet. After dilatation of the tract a pigtail catheter (7-8 French) was placed into the renal pelvis. Catheter placement was performed with local anaesthesia (2% Cystocain). Complications were uncommon. Surgery was needed in all patients for permanent solution of obstruction. PCN is the method of choice for temporary relief of obstruction of the upper urinary tract in children when immediate surgery is hazardous.

[Diagnostic and therapeutic significance of percutaneous nephrostomy in children]. / Dijagnosticki i terapijski znacaj perkutane nefrostomije u dece.

During the period from April 1986 to March 1988 on the Pediatric Clinic in Belgrade a total of 12 percutaneous nephrotomies were performed to relieve obstruction of the upper urinary tract. The youngest patient was a two-month old with giant hydronephrosis, and the oldest was a 12-year old girl with an acute kidney obstruction caused by impaction of wedging the poured off stone. The most common reasons for obstruction of the upper urinary tract were congenital stenoses and postoperative scars, urethrocystoneostomia and pyeloplasty. The most frequent clinical manifestations in patients on whom nephrotomy was carried out as an emergency operation were infection, palpable tumor (cyst) and acute kidney insufficiency. Percutaneous nephrostomy was always performed with basal sedation and local infiltrative anesthesia under X-ray control. Surgery was necessary in all cases, but no complications needing surgical intervention occurred. Mild hematuria can be expected at almost every punction, so it should not be treated as a complication. Dislocation of the nephrostoma catheter immediately after surgery can be a complication which necessitates reintervention, but if this happens after more than ten days, the catheter can easily be replaced through the formed fistulous channel. In one case percutaneous placing of the catheter was not possible, so it was placed surgically in the kidney and skin to enable drainage of the infected urinome a month after rupture of the ureteropyelic segment. Percutaneous placing of a catheter through the nephrostoma is the method of choice for urgent and temporary relief of obstructions of the upper urinary system regardless of etiology, and this procedure achieves immediate therapeutic and, if necessary, diagnostic effect which enables recovery of the patient and preparation for definitive surgery which in such a situation could be hazardous.

[Stenosis of the external urethral opening as one of the causes of recurrent urinary infections in girls--surgical treatment]. / Stenoza spoljasnjeg otvora uretre kao jedan od uzroka recidivantnih urinarnih infekcija u zenske dece--hirurska leenje.

105 middleaged girls (8.4 years) were operated upon at this Clinic because of distal meatal stenosis (DMS) as one of the causes of recurrent urinary infections and/or tenacious dysuria. Meatoplasty, one small surgical intervention, does treat about 78.1% infections of the urinary tract, as well as about 36.8% vesicoureteral reflux (VUR) of the I and II grade. The fibrous ring which is an anatomical alteration of DMS, is primarily obstructive lesion, which is appearing most frequently associated with urinary infection. In one quarter of cases in which a meatoplasty did not show a clinical improvement, one should think over the more radical surgery either at the urinary neck or at the vesico-ureteral junction. Spontaneous improvement or/and regression of VUR, in one third of cases following meatoplasty is an encouraging datum; but this intervention by itself is not a method for its treatment. A small number of complications, one stress incontinence only, demonstrate a reliability of this procedure in selected cases. Meatoplasty and dilatation of stenosed urethral meatus are alternative methods, which give similar results and their use mainly depends on the urologist's affinity to this method.

[Successful autotransplantation of intra-abdominal testes into the scrotum using microvascular technics]. / Uspesna autotransplantacija intrabdominalnih testisa u skrotum mikrovaskularnom tehnikom.

The surgical treatment of undescended and nonpalpable testes still remains controversial. The shorter the vascular stem, the greater difficulties are during orchidopexy. The results of the formerly used "stage" and "Loop" techniques are unsure and frequently lead to postoperative atrophy of the testes. Thanks to the marked development of the microsurgical technique, over the past 10 years, besides the conventional methods for orchidopexy of the testes with a short vascular stem, microvascular revascularization on the autotransplanted testes from the abdomen into the scrotum is also used. During the last year two boys were successfully operated upon with this method with which this microvascular anastomosis technique is described and the problems openly discussed.