Dystrophic calcifications and Raynaud's phenomenon in an eight-year old girl.

INTRODUCTION: Dystrophic calcifications are the most common subtype of skin calcinosis. Tumorous soft tissue calcium deposits usually contain hydroxyapatite and amorphous calcium phosphate. Differential diagnosis of skin calcinosis encompasses Thibierge-Weissenbach syndrome, systemic sclerosis, scleroderma, CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), dermatomyositis, systemic lupus erythematosus, ad myositis ossificans progressiva. CASE OUTLINE: We present the case of an eight-year old girl with tumorous soft tissue calcium deposits and Raynaud's phenomenon. At the age of 3.5 years, our patient was admitted to Pediatric Surgery Clinic because of bilateral acrocyanosis localized at the fingertips area of hands, with the signs of vascular trauma. Therapy with vasodilators and hyperbaric oxygen treatment were completed. This therapy resulted in improvement. At the age of eight, the patient was admitted again due to intermittent, painful cramps localized in both hands. Punctiform deposits were present at the tips of fingers and toes, which looked like calcifications and were spontaneously eliminated, with the remnants of crater-shaped defects. A hard tumorous deformity localized in soft tissue was present in the extensor area of the right elbow. Laboratory indicators of inflammation were within the reference values, and antinuclear antibodies were positive. A nodus localized at the right elbow was extirpated. Pathohistological findings: connective and fat tissue with large deposits of calcium. CONCLUSION: Further follow-up of our patient is necessary due to possible development of complete picture of CREST syndrome or systemic sclerosis.

Severe diffuse hypoplasia of the aorta associated with multiple vascular abnormalities.

Hypoplasia of the thoracic and abdominal aorta is an extremely rare vascular pathology. The most common clinical manifestation is severe uncontrolled hypertension in adolescents and young adults. Medical treatment alone can decrease blood pressure, but often very high doses of antihypertensive drugs are needed. When hypertension is refractory to the antihypertensive medications, surgical revascularization is considered as the treatment of choice. We report the case of a severe and diffuse hypoplasia of the aorta, beginning with the aortic isthmus, to the aortic bifurcation, associated with an aberrant celiac trunk and superior mesenteric artery, and with other multiple vascular abnormalities. Unlikely, the only manifestation of this extensive vascular malformation was medicamentously controllable hypertension. To our knowledge, this severe vascular anomaly, with such a minimal clinical manifestation, has not been previously described in the English literature.

Cystic echinococcosis in children - the seventeen-year experience of two large medical centers in Serbia.

Cystic echinococcosis (CE) is a public health problem in countries having such endemic areas. Epidemiological studies of CE, especially pediatric, are rare. The aim of this study was to evaluate epidemiological and clinical characteristics of CE in children in Serbia. Data were obtained retrospectively from the case records of patients under the age of 18 years admitted for surgical treatment of CE at two large pediatric medical institutions in the period 1990-2006. Patients' age, number of cysts and their anatomic location were evaluated in relation to differences by patients' gender and socio-geographic status (urban or rural origin). The study included 149 children with 272 hydatid cysts. The mean age of patients was 10.1+/-3.8 years. There were no significant differences in the number of patients in relation to gender and urban:rural origin. There were no significant differences in patients' age at the time of surgery or the number of cysts per patient when patients' gender or socio-geographic status was evaluated. The anatomic location of cysts was as follows: liver (N=165; 60.7%), lungs (N=82; 30.1%), and other locations (N=25; 9.2%). Multiple cysts, and combined liver/lung involvement were identified in 34.2% (N=51), and 6.0% (N=9) of patients, respectively. Hepatic cysts were significantly more common in girls than in boys. There were no significant differences in anatomic location of cysts between socio-geographic groups. The large number of infected children during a long period of investigation indicates an active transmission of disease and a lack of program for control and prevention of CE in Serbia.

[Treatment of vesicouretheral reflux in pediatric patients].

BACKGROUND/AIM: Vesicouretheral reflux (VUR) is an inherited abnormality of the urinary tract caused by dysfunction of vesicouretheral junction frequently accompanied by recurrent urinary infection. The optimal treatment method of VUR is still controversial. The aim of the study was to analyze medical charts of children with VUR with special focus on the results of endoscopic treatment with Deflux. METHODS: This retrospective study analyzed the medical charts of all children diagnosed with VUR at the Institute for Children and Youth Health Care of Vojvodina, in five years period. The analyzed data were: age, gender, grade, bilateral or unilateral presence of reflux, distribution to the sides of the body, urinary tract infections, associated anomalies and complications of VUR, treatment methods and the success rate of endoscopic correction with Deflux. Data processing was performed using the standard statistical methods. RESULTS: The study included 167 patients (101 females and 66 males) with 231 refluxing ureters. The patients age at diagnosis was 1 month to 18 years (mean 4.6 years). Frequencies of different grades of VUR at initial investigation were: 17%, 27%, 22%, 21% and 13% for grades I to V, respectively. VUR was unilateral in 103 patients (left in 65 and right in 38), and bilateral in 64. Urinary tract infections were present in 78.4% of patients, reflux nephropathy in 38.3%, hypertension in 3.0%, chronic renal insufficiency in 4.8%, associated anomalies in 39.5% of patients. The treatment method was recorded in 154 cases. Medically were treated 80 patients, and surgically 74. Endoscopic injection of Deflux was carried out in 59 patients. From 79 ureters treated, five had grade I reflux, 11 grade II, 23 grade III, 27 grade IV, and 13 grade V. In 46 patients Deflux injection was carried out once. A second injection was required in eight, and third injection in five patients. Reflux was absent in 42 of the treated patients (71.2%) and 15 patients (25.4%) had a decreased grade of reflux after the treatment Overall success rate was 96.6%. CONCLUSION: Endoscopic subureteral injection of Deflux is a minimally invasive method for VUR treatment in pediatric patients.

[History of minimally invasive surgery].

This paper presents a historical review and development of minimally invasive surgery. The interest of physicians to "look into the internal organs" has existed since the ancient time. The first described endoscopy was by Hippocrates. He made reference to a rectal speculum. The credit for modern endoscopy belongs to Bozzini. He developed a light conductor which he called "Lichleiter" to avoid the problems of inadequate illumination. In 1853, Desormeaux first introduced the "Lichtleiter" of Bozzini to a patient. Many developments, which occurred independently but almost simultaneously, produced breakthroughs for endoscopy and laparoscopy that were bases for modern instruments. In 1901, Kelling coined the term "coelioskope" to describe the technique that used a cystoscope to examine the abdominal cavity of dogs. In 1910, Jacobaeus used the term "laparothorakoskopie" for the fist time. In 1938, Veress developed the spring-loaded needle for draining ascites and evacuating fluid and air from the chest. Its current modifications make the "Veress" needle a perfect tool to achieve pneumnoperitoneum during laparoscopic surgery. In 1970, Hasson developed a technique performing laparoscopy through a miniature leparotomy incision. The first solid state camera was introduced in 1982 that was the start of "video-laparoscopy". In 1981 Kurt Semm performed first laparoscopic appendectomy. Within a year, all standard surgical procedures were performed laparoscopically. The authors also analyzed the new surgical techniques, such as telesurgery, robotics and virtual reality in current surgical practice. They specially enmphasized the use of laparoscopic access in pediatric surgery which has become a new gold standard in surgical treatment of pediatric patients.

[History of surgical treatment of appendicitis].

Most of the history of appendicitis and appendectomy has been made during the past two centuries. Jacopo Berengario da Carpi gave the first description of this structure in 1522. Gabriele Fallopio, in 1561, appears to have been the first writer to compare the appendix to a worm. In1579 Caspar Bauhin proposed the ingenious theory that the appendix served in intrauterine life as a receptacle for the faexes. Many of anatomists added more or less insignificant ideas concerning the structure of the appendix and entered upon useless controversy concerning the name, function, position of the appendix vermiformis. The first successful appendectomy was performed in 1735 by Claudius Amyand. Geillaume Dupuytren considered that acute inflammation of the right side of the abdomen arose from disease of the caecum and not the appendix. As surgeons were wary of opening the abdomen for examination, early stages of appendicitis remained unknown. John Parkinson was able to give a good description of fatal appendicitis in 1812. Surgeons began draining localised abscesses which had already formed. In 1880 Robert Lawson Tait made the first diagnosis of appendicitis and surgically removed the appendix. In 1886 Reginald Heber Fitz published a study on appendicitis and named the procedure an appendectomy. In 1889, Tait split open and drained an inflamed appendix without removing it. Charles McBurney proposed his original muscle splitting operation in 1893 and this was modified by Robert Fulton Weir in 1900. Today we have a multiplicity of signs and symptoms, helping to diagnose appendicitis, and there are a lot of techniques for operation with little essential difference throughout. Kurt Semm performed the first laparoscopic appendectomy in 1981 which became a new gold standard in surgical treatment of acute and chronic appendicitis.

[Constipation--a common problem in childhood].

INTRODUCTION: Constipation in children is defined as the infrequent and difficult passage of hard stool, not necessarily associated with in-frequent stools. All healthy newborns have their first stool within the first 24 to 48 hours after birth. Intestinal transit time increases with age, therapy decreasing the frequency of stooling. ANATOMY AND PHYSIOLOGY OF ANUS AND RECTUM: Acquisition of fecal continence requires: normal internal and external anal sphincters, puborectal muscle as well as intact sensory input from both the rectal vault and anal canal. ETIOLOGY AND DIFFERENTIAL DIAGNOSIS: During the first year of life, failure to have bowel movement every other day warrants evaluation. During infancy, constipation is usually due to dietary manipulations, malnutrition or some other functional abnormalities. Anatomic causes are found only in 5% of patients. DIAGNOSIS AND THERAPY: Diagnosis relies on history and physical examination. Digital rectal examination usually reveals a shorter anal canal with decreased sphincter tone. The rectal ampulla is dilated and filled with stool. Anorectal manometry is helpful in differentiating functional constipation from aganglionosis or other neurologic problems. Treatment varies depending on the underlying cause. Bowel retraining, aimed at establishing regular daily bowel movement, is of utmost importance in children. The response to treatment is usually dramatic. CONCLUSION: Constipation in children causes anxiety in the family and successful treatment requires persistent reassurance and repeted reevaluation.

[Ureteral triplication--a case report].

INTRODUCTION: Of all children born with congenital anomalies, 30-40% have urogenital abnormalities. Most of them are asymptomatic, but if any symptoms occur, they are usually symptoms of urinary infections. CASE REPORT: A four-year old girl was admitted because of recurrent urinary infections and in order to perform a thorough clinical examination. Clinical examination showed an asymmetrical gluteus and presence of a fibroma in the same region. Detailed radiologic examinations (ultrasonography, plain X-ray, excretory urography, voiding urethrocystography) revealed a triplicate ureter on the right side with a vesicoureteral reflux of the fifth grade on the same side. Intraoperatively, type III ureteric triplication according to Smith's classification was established. Ureteral remodeling and reimplantation was done. The postoperative course was good, and follow-up voiding urethrocystography showed absence of reflux. DISCUSSION: Ureteral triplication is a consequence of either development of three buds on the mesonephric duct, or development of two buds, one of which further divided into two parts. Most common anomalies associated with ureteral triplication are: ureteral duplication on the other side (37%), ectopy of the ureteric orifice (28%) and kidney dysplasia (8%). Vesicoureteral reflux is very common on affected or on both sides. Symptomatology is the same as in ureteral duplication--dominated by signs of urinary infections. CONCLUSION: Diagnosis of ureteral duplication requires detailed imaging. Treatment of symptomatic abnormalities should be individual, regardless of the applied treatment (conservative or operative).

[External genital abnormalities in male schoolchildren: an epidemiological study]. / Ucestalost anomalija spolgasnjih genitalija kod decaka skolskog uzrasta.

INTRODUCTION: External genital abnormalities represent the most common congenital anomalies. Proper, not delayed diagnosis and treatment of certain abnormalities (undescended testis, hypospadia, varicocele) is of great importance for future fertility potential of children. MATERIAL AND METHODS: An epidemiological study, that consisted of an urological checkup, was performed among school-aged boys in two elementary schools. Prevalence of external genital, and inguinal region was examined. RESULTS: A total of 1229 elementary school boys were examined. The incidence of external genital abnomalities was 27.8%. Certain anomalies were already surgically treated in 7.8% of boys. Phimosis wasfound in 66 patients (5,5%), which represents 26.6% of all abnomalities. DISCUSSION: In this study the incidence of undescended testis was 2.0%. Although it is similar to literature data, the fact that undescended testis was found in 25 boys older than 7 years (two of them 15 years old!) suggests that the primary health care is not satisfactory. The incidence of inguinal hernia and hydrocele varies according to different authors from 0.8-4.4% to 13.44%. In this study the incidence was 2.4%. Varicocele represents a developmental anomaly and the incidence is increasing with age. It is rare in boys up to 10 years of age, but the incidence of varicocele in boys aged 15 years (15.8%) reaches the level of incidence in adults (19.82%). The incidence of phimosis in this study was 5.5%. CONCLUSION: High incidence of external genital abnormalities in school aged boys suggests a need for further education of physicians in primary health care services as well as of parents.