Cistični limfangiom mezenterija jejunuma koji oponaša akutni apendicitis: prikaz slučaja

Cystic lymphangiomas of the small bowel mesentery are rare manifestations of intra-abdominal tumors. Usually, they are discovered incidentally during examination for an unrelated abdominal illness. We present a case of a 4-year-old boy who was admitted to our hospital because of the right lower quadrant acute abdominal pain suspect of acute appendicitis. At laparotomy, a giant, cystic, encapsulated and lipomatous mesenterial mass was found, 15x15x10 cm in size, infiltrating the jejunum. The tumor was located 70 cm from Treitz’s ligament. Extirpation of tumor mass with intestinal resection of the involved loops was necessary. Pathologic examination confirmed the diagnosis of mesenteric cystic lymphangioma. Although they are rare, cystic mesenteric lymphangiomas should be considered as a possible cause of acute abdomen and treated with surgical resection. Prognosis after surgical removal is excellent.Cistični limfangiomi mezenterija tankog crijeva pripadaju rijetkim intraabdominalnim tumorima. Obično se nađu slučajno tijekom obrade zbog neodređene trbušne boli. Prikazuje se slučaj četverogodišnjeg dječaka hospitaliziranog zbog bolova u donjem desnom abdominalnom kvadrantu sumnjivih na akutnu upalu crvuljka. Nakon učinjene laparotomije našla se velika, cistična, inkapsulirana, lipomatozna mezenterijska masa veličine 15x15x10 cm koja je infiltrirala jejunum. Tumor je bio smješten oko 70 cm aboralno od Treitzova ligamenta. Učinjena je ekstirpacija tumora s infiltriranom jejunalnom vijugom. Patohistološki nalaz je potvrdio dijagnozu mezenterijskog cističnog limfangioma. Iako rijetki, cistični mezenterijski limfangiomi se mogu smatrati mogućim uzrokom akutne abdominalne boli i liječiti kirurškom resekcijom. Prognoza je nakon kirurškog odstranjenja odlična

A 3-Year Experience of a Minimally Invasive Technique for Correction of Pectus Excavatum in Croatia

The aim of this study was to assess the early results of a three-year experience with the minimally invasive correction of pectum excavatum, which reguires no cartilage incision or excision, and no sternal osteotomy. Since 2001 we have performed 35 minimally invasive pectus excavatum procedures at our hospital. A convex steel bar is inserted under the sternum through small bilateral incisions, and removed after 2 years when permanent remolding had occurs, the bar is removed. Complications were pneumothorax in 5 patients (only 1 required a thoracostomy tube, the other 4 resolved spontaneously), pneumonia in 3 patients, and bar displacement in 1 patient. The mean follow-up was 3 months to 3 years. Initial excellent results were maintained in 28 patients (normal postoperative chest), good results in 5 patients (mild residual pectus) and poor in 2 patients (severe recurrence requiring further treatment). Poor results occurred because the steel bar was too soft in 1 patient, and the sternum too soft in 1 patient with Marfan’s syndrome. Our early results with the minimally invesive technique without cartilage incision and resection or sternal osteotomy showed that the procedure is effective with excellent preliminary results

Coloanal anastomosis in the treatment of congenital megacolon in children: case report

Kongenitalni megakolon ili Hirschprungovu bolest prvi put je opisao Harold Hirschprung. To je urođena crijevna aganglioza kao rezultat poremećaja u fetalnom razvoju mienteričkog živčanog sustava. Normalan motilitet crijeva ovisi o koordiniranim segmentalnim kontrakcijskim valovima koji slijede neposredno nakon opuštanja glatkih mišića. Bolesnicima s Hirschprungovom bolešću nedostaje funkcionalni mienterički živčani sustav u zahvaćenom distalnom crijevu pa imaju nedjelotvornu distalnu peristaltiku. Klinički rezultat je neuspješno pasiranje mekonija ubrzo nakon rođenja, zatvor, distendiran trbuh, palpabilne vijuge crijeva, povraćanje, proljevaste vodenaste stolice, slabo napredovanje tjelesne mase, spor rast i malapsorpcija. Aganglionarni distalni segment crijeva je razlog dilatacije proksimalnog dijela debelog crijeva ili nesposobnosti otvaranja analnog sfi nkternog sustava. Hirschprungova bolest zahvaća između 1:5 000 do 1:8 000 živorođene djece. Većina slučajeva dijagnosticira se prije 10. godine života. Bolesnici se povremeno prezentiraju ovim tegobama u kasnijoj dobi. Nedavno smo operirali 12-godišnjeg bolesnika s ovim stanjem. Najprije smo izveli kiruršku resekciju rektosigmoidnog kolona sa zatvaranjem bataljka rektuma i formiranjem kolostome, Hartmannov zahvat. Tri mjeseca nakon toga obavili smo ekstirpaciju rektuma i formirali koloanalnu anastomozu. Postoperativni tijek, stanje bolesnika i klinički testovi pokazali su da je ovakav način liječenja kongenitalnog megakolona bio uspješan, što je bila i svrha ovog prikaza bolesnika.Congenital megacolon or Hirschsprung’s disease was fi rst described by Harold Hirschsprung. It is a congenital intestinal aganglionosis as a result of arrested fetal development of the myenteric nervous system. Normal intestinal motility depends on a coordinated segmental contraction waves followed immediately by smooth muscle relaxation as it propagates caudally. Patients with Hirschsprung’s disease lack functional myenteric nervous system in the aff ected distal intestine and have ineff ective distal peristalsis. The clinical outcomes are failure to pass meconium shortly after birth, constipation, abdominal distension, palpable loops of bowel, vomiting, watery diarrhea in the newborn, poor weight gain, slow growth and malabsorption. The aganglionic distal segment of the bowel is the reason for dilatation of the proximal part of the colon or opening debility of the anal sphincter system. Hirschsprung’s disease is aff ecting 1:5000 to 1:8000 live births. Most cases are diagnosed before the patient is 10 years of age. Occasionally, patients present with this problem at a later age. Recently we operated on a 12-year-old patient with this condition. First we performed surgical resection of the rectosigmoid colon with closure of the rectal stump and formation of an end colostomy, also known as Hartmann’s procedure. Three months later, the rectum was extirpated and coloanal anastomosis formed. Based on the uneventful postoperative course, good patient’s condition and clinical tests, the treatment of congenital megacolon was successful

A RARE TUMOR OF THE LUNG IN CHILDHOOD – INFLAMMATORY MYOFIBROBLASTIC TUMOR

Inflamatorni miofibroblastični tumor (IMT) benigna je novotvorina koja uglavnom zahvaća pluća kod djece. Od svih tumora pluća javlja se u 0,7%.1 Brunn je tumor prvi opisao 1939. godine.2 Postavljanje sigurne dijagnoze veoma je teško i često moguće tek nakon resekcije tumora. Mi želimo prikazati 13-godišnju bolesnicu s plućnim IMT-om koja je duže vrijeme kašljala, imala zaduhu i osjećaj nelagode u prsnom košu. Rendgenska snimka, kao i kompjutorizirana tomografija pokazale su prisutnost tumorske mase u donjem plućnom režnju s desne strane. Njezini klinički i radiološki nalazi nisu bili specifični da bi se mogla utvrditi dijagnoza pa se indicirao kirurški zahvat. Sam zahvat i postoperativni tijek protekli su bez komplikacija. Tumor je odstranjen u cijelosti, histološki je potvrđen upalni miofibroblastični tumor pluća. Bolesnica je 2 godine nakon operacije bila bez kliničkih znakova recidiva bolesti.Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm, mainly involving the lungs of the children. It represents 0.7% of all lung tumors.1 It was first described by Brunn in 1939.2 Diagnosis is very difficult and often only possible after resection of the tumor. We would like to present a case of pulmonary IMT in a 13-year-old girl who presented with symptoms like cough, shortness of breath , and chest discomfort. Chest X ray and computed tomography revealed the presence of a right lower lobe lung mass. Its clinical and radiological findings were diverse and non specific. The mass was removed in toto, histopathology confirmed the inflammatory myofibroblastic tumor of the lung. Intraoperative and postoperative courses were uneventful. The patient has been without any signs of relapse 2 years after the surgery

Haemorrhagic pseudocyst of the adrenal gland: case report and literature review

Adrenalne pseudociste su relativno rijetke patološke tvorbe u pedijatrijskoj populaciji. Obično su asimptomatske te su sporadičan nalaz na radiološkim pretragama. No kad promjerom prelaze veličinu od 5 cm, mogu uzrokovati simptome kompresije susjednih organa te zahtijevati kirurško liječenje. U dijagnostici takvih lezija u pedijatrijskoj populaciji izrazito je bitno razlikovati benignu od maligne lezije. Prikazujemo pacijenticu u dobi od 12 godina koja se prezentirala slikom difuznih bolova u abdomenu te febriliteta. Kod pacijentice je ultrazvučno praćena cista desne nadbubrežne žlijezde kroz prethodne dvije godine. Pacijentica je kirurški zbrinuta ekscizijom te drenažom cistične tvorbe otvorenom medijalnom laparotomijom. Patohistološki nalaz verifi cira tumorsku tvorbu kao hemoragičnu pseudocistu nadbubrežne žlijezde. Praćenje šest mjeseci postoperativno pokazalo je dobre rezultate.Adrenal pseudocyst is a rare pathologic fi nding in paediatric population. They tend to be asymptomatic lesions and are usually found sporadically in radiologic fi ndings. However, cysts larger than 5 cm in diameter can cause symptoms of compression on adjacent organs and therefore surgical removal is needed. Regarding diff erential diagnosis, it is crucial to diff er a benign from malignant lesion in paediatric population. We report a case of a 12-year-old girl who presented with acute abdominal pain and fever. She had been diagnosed with a right abdominal cyst two years before and was routinely followed up through our outpatient clinic. Medial laparotomy, surgical excision and drainage of the cystic lesion were performed. Histopathology examination reported a haemorrhagic adrenal pseudocyst. Six-month follow up was uneventful

Has Ombrëdanne’s Method of Hypospadic Urethra Reconstruction Been Ignored with Reason?

A B S T R A C T From January 1970 to December 1979 inclusive, 193 boys (aged 2 to 16) underwent surgery for distal hypospadia using Ombredanne’s method at the Department of Pediatric Surgery University Hospital Center Rijeka and at the Department of Pediatric Surgery Zagreb. Follow-up period was 7 to 20 years (mean 13.4). 20 (10.36%) subjects had post-operative organic complications and 15 (7.77%) of them required surgical correction. According to these findings, the success rate using Ombredanne’s method of reconstruction of the hypospadic urethra in no way lags behind the success rate using MAGPI and Mathieu’s methods as well as »Preputial island flap urethroplasty« for analogous cases. Out of 193 subjects who underwent surgery, 80 (41.45%) of those who were sexually mature and had normal psychosexual development were questioned. In this sample, 75 (93.75%) were satisfied with the post-operative appearance of the penis while only 5 (6.25%) were dissatisfied, 3 of which had hypoplastic penis. In 78 (97.50%) subjects questioned, the post-operative urinary squirt was normal and two of them had weak urinary squirt (2.50%), due to meatal stenosis. In conclusion, Ombredanne’s method of reconstruction of the urethra in boys with distal hypospadia is equally successful as other methods used for this purpose

Central Venous Catheters for Chemotherapy of Solid Tumors – Our Results in the Last 5 Years

Central venous catheters provide an easy access for intravenous medications. Having a central line in place will relieve a child from the discomfort and danger of multiple regular intravenous lines for chemotherapy. The use of indwelling central venous catheters has become commonplace in the management of children undergoing oncological treatment. There are two types of central lines commonly used. There are Broviac catheters and Port-A-Cath (PAC) catheters. In the last 5 years we inserted 194 catheters in 175 children. We inserted 121 Broviac catheters and 73 PAC catheters. During the follow up of 39382 catheter days 44 complications were observed. In Broviac group the median follow up was 155 days and in PAC group was 230 days. We observed differences in the incidence between two devices. In Broviac group infections were more frequent and in PAC group other complications were more frequent than infections

EWING SARCOMA OF THE RIB – CASE REPORT

Ewingov sarkom je jako maligni koštani tumor koji je prvi put u literaturi opisao J a m e s E w i n g 1921. godine (1). Ewingov sarkom se obično javlja u dječjoj ili ranoj adolescentnoj dobi, s najvećom učestalošću između 10. i 20. godine života, premda se može javiti i kod mlađe i starije životne dobi. Najučestalije se javlje u području zdjelice, natkoljenične i nadlaktične kosti te rebara. Ewingov sarkom je po učestalosti pojavljivanja drugi tumor koštanog tkiva kod djece i najmaligniji koštani tumor dječje dobi. Učestalost obolijevanja je 0.3 bolesnika na 1.000.000 djece mlađe od 3 godine i 4.6 djece na 1.000.000 mladih ljudi između 15 i 19 godina života. Prosječna učestalost obolijevanja je ispod 2 bolesnika na 1.000.000 djece na godinu. Odnos muške i ženske djece koja obolijevaju je 1.5:1 (2-6). Preživljavanje kod lokaliziranog tumora je 60-70% oboljelih, 30% preživljavanje je s metastazama po plućima te preživljavanje manje od 10% je s metastazama na drugim mjestima (7, 8). Prikazujemo osmogodišnju djevojčicu s Ewingovim sarkomom na rebru.Ewing sarcoma, a highly malignant primary bone tumor, was first described by James Ewing in 1921 (1). Ewing sarcoma usually presents in childhood or early adulthood, with its peak between 10 and 20 years of age, although it can occur in younger children and older adults. The most common areas in which it occurs are the pelvis, the femur, the humerus and the ribs. Ewing sarcoma is the second most common malignant bone tumor in young patients and it is the most lethal bone tumor. Tumor occurrence ranges from 0.3 cases per 1 000 000 children younger than 3 years to as high as 4.6 cases per 1 000 000 adolescents aged 15-19 years. The annual incidence rate averages less than 2 cases per 1 000 000 children. The male-to-female ratio is 1.5:1 (2-6). Survival rate is 60-70% with low-risk, in a localized tumor, intermediate risk (30%) when the tumor has spread to lungs and advanced risk (less than 10%) when the tumor has spread further to bone, bone marrow and/or other tissue (7, 8). We present an 8 year-old girl with Ewing sarcoma of the rib

Abernethy malformation as a rare indication for liver transplantation: Case report

Abernethyjeva malformacija ili kongenitalna ageneza portalne vene (KAPV) rijetka je malformacija abdominalnoga splanhničkog venskog sustava. Dijagnoza se najčešće postavlja u dječjoj dobi i često je povezana s drugim malformacijama poput srčanih grešaka i poremećaja skeletnog sustava, kao i s tumorima jetre. Sama malformacija može se manifestirati u dva oblika. Kod tipa I portalna krv potpuno zaobilazi jetru te se gornja mezenterijska vena i lijenalna vena odvojeno (tip Ia) ili zajedno (tip Ib) dreniraju u donju šuplju venu, a u jetri ne postoje intrahepatalni portalni venski ogranci. Kod tipa II prisutan je parcijalni portokavalni shunt i portalna krv djelomično opskrbljuje jetru. Nema jedinstvenoga terapijskog pristupa za sve bolesnike s Abernethyjevom malformacijom, međutim, kod bolesnika sa simptomima jetrene bolesti (encefalopatija, loša funkcija jetre) te onih s tumorima jetre preporučuje se transplantacija jetre. U ovom radu prikazujemo Abernethyjevu malformaciju tipa Ib u 17-godišnje bolesnice kod koje se obradom kronične malaksalosti praćene bolima pod desnim rebrenim lukom pronašao neresektabilni tumor jetre te joj je uspješno obavljena ortotopna transplantacija jetre.Abernethy malformation or congenital agenesis of the portal vein (CAPV) is a rare malformation of the abdominal splanchnic venous system. This malformation is commonly found in children and is often associated with other malformations such as congenital cardiac anomalies and skeletal system disorders, as well as liver tumors. There are two types of Abernethy malformation. In type I, portal blood bypasses the liver completely, with the superior mesenteric vein and the splenic vein draining into the inferior vena cava separately (type Ia), or together (type Ib). There are no intrahepatic portal vein branches in the liver. Type II is a partial portocaval shunt in which portal blood partially supplies the liver. There is no unified therapeutic approach for all patients with Abernethy malformation, however, liver transplantation is recommended in patients with liver disease (encephalopathy, poor liver function) and those with liver tumors. In this case report we present a case of Abernethy type Ib malformation in a 17-year-old patient with chronic malaise and uper abdominal pain. During diagnostic work-up, an unresectable liver tumor was found and the patient was successfully treated with orthotopic liver transplantation

Knowledge and attitudes about undescended testicles – the need for an educational approach

Cilj: S obzirom na loše pokazatelje dobi orhidopeksije za kongenitalnu skupinu nespuštenih testisa, cilj ovoga rada bio je istražiti znanja i stavove specijalizanata/mladih specijalista pedijatrije, obiteljske i školske medicine o navedenoj problematici jer upravo su oni, u većini slučajeva prvi kojima se roditelji obraćaju za pomoć i mišljenje. Metode: Znanje i stavovi specijalizanata i mladih specijalista ispitani su pomoću anonimnog online upitnika, temeljenog na najno- vijim spoznajama i smjernicama. Prikupljanje podataka provedeno je putem besplatnog programa Google Forms®. Kako bismo utvrdili odstupaju li dobiveni odgovori od odgovora koje bismo očekivali pod određenom hipotezom, odgovori su uspoređivani korištenjem hi-kvadrat testa uz razinu statističke značajnosti od 5%. Rezultati: Upitnik je ispunilo 129 specijalizanata/mladih specijalista, od kojih većina smatra kako za vrijeme studiranja i specijalistič- kog usavršavanja nisu dovoljno čuli i naučili o nespuštenim testisima, kako svoje znanje o problematici nespuštenih testisa smatraju nezadovoljavajućim te kako se u dosadašnjoj karijeri nisu susreli sa smjernicama koje govore o dijagnostici i liječenju nespuštenih testisa. Odgovori na većinu pitanja, koja su se temeljila na najnovijim smjernicama, bili su nezadovoljavajući. Gotovo 2/3 ispitanika smatra kako se o dječacima s nespuštenim testisima u Republici Hrvatskoj ne vodi pravodobna briga i liječenje, a 1/3 ispitanika ključni problem vidi u primarnim pedijatrima, obiteljskim liječnicima te u timovima školske medicine. Zaključci: S obzirom na dobro poznate posljedice nepravodobnog liječenja nespuštenih testisa u dječaka, rezultatima analize anke- te kojima ne možemo biti zadovoljni potrebno je ozbiljno pristupiti. Stoga potičemo buduće edukativne intervencije, utemeljene na dokazima, koje će za cilj imati bolju informiranost o problematici nespuštenih testisa i u konačnici vremenski pomak samih orhido- peksija k ranijoj dobi.Objective: Considering the poor indicators relating to the age group undergoing orchidopexy for the congenital group with undescended testicles, the objective of this article is to investigate the knowledge and attitudes of residents and young specialists in the fi eld of pediatrics, family, and school medicine concerning the above-mentioned issue, given that these specialists are, in most cases, the fi rst from whom parents contact seek help and opinion. Methods: The knowledge and attitudes of residents and young specialists were examined using an anonymous online questionnaire, based on the latest knowledge and guidelines. Data collection was carried out using the free Google Forms® program. To determine whether the obtained answers deviate from the answers expected under a certain hypothesis, the answers were compared using the chi-square test with a statistical signifi cance level of 5%. Results: The questionnaire was fi lled out by 129 residents and young specialists, most of whom believe that, during their studies and specialist training, they had not heard and learned enough about undescended testicles, and they considered their knowledge about the problem of undescended testicles to be unsatisfactory, and they had not encountered guidelines in their career detailing the diagnosis and treatment of undescended testicles. The answers to most of the questions were unsatisfactory based on the latest guidelines. Almost two-thirds of the respondents believe that boys with undescended testicles in the Republic of Croatia are not cared for and treated on time, and one-third of the respondents see the key problem in primary pediatricians, family doctors, and school-age medical teams. Conclusions: Considering the well-known consequences of untimely treatment of undescended testicles in boys, the results of the survey analysis, which we cannot be satisfi ed with, must be approached seriously. Therefore, we encourage future evidence-based educational interventions to provide better information about the problem of undescended testicles and ultimately shift the timing of orchidopexies to an earlier age