Hirschsprung’s Disease and Rehbein’s Procedure – Our Results in the Last 30 Years

Hirschsprung’s disease is congenital anomaly of the intestine and Harald Hirschsprung gave the first description of this disease1. The aim of this follow-up study was to evaluate the results of Rehbein’s procedure in the treatment of Hirschsprung’s disease in the last 30 years in Children’s Hospital Zagreb. Hirschsprung’s disease is congenital intestinal aganglionosis as the results of arrested fetal development of the myenteric nervous system. Hirschsprung’s disease is affecting between 1:5000 to 1:8000 live births. A total of 124 children underwent Rehbein’s lower anterior resection at Children’s Hospital Zagreb. The principle of Rehbein’ procedure is to remove aganglionic narrow segment and dilated sigmoid colon and anastomosis between normal intestine with rectal stump. The postoperative outcome was analysed for early and late complications like wound infections, abscesses, anastomotic insufficiency, postoperative enterocolitis, constipation, fecal incontinence, need for reoperation, ileus and mortality. On the basis of our results and data from literature we concluded that Rehbein’s procedure is an excellent method for treatment Hirschsprung’s disease

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

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

LESS FREQUENT CAUSES OF ACUTE SURGICAL PAIN IN CHILDREN - OUR EXPERIENCE IN THE PAST 10 YEARS

Akutna bol se kod djece može pojaviti zbog različitih razloga. Pod pojmom akutni abdomen smatra se skup simptoma koji se javljaju kod iznenadne bolesti tjelesnih organa. Prema Međunarodnoj udruzi proučavanja boli, bol je definirana kao "neugodno osjetno i osjećajno iskustvo povezano s pravom ili potencijalnom ozljedom tkiva, ili uvjetovano tom štetom ili ozljedom". Bol se po intenzitetu može razlikovati od lagane, teške te naposljetku agonizirajuće, i to kao stalne ili nestalne. Bol se može doživjeti kao oštru, pulsirajuću, mučnu, goruću, strijeljajuću ili kao kombinaciju navedenih. Željeli smo prikazati nekoliko bolesnika s različitim problemima i različitim kliničkim slikama, koji nisu uobičajeni u svakodnevnom rutinskom liječenju. Svi su bolesnici imali akutnu ili kroničnu bol, a svi su u početku bili liječeni medikamentima. Kod svih je bolesnika bilo potrebno kirurško liječenje. Kod postojanja boli u trbuhu moramo uvijek razmišljati o svim mogućnostima koje bi mogle izazvati tegobe. Često postoje dijagnostičke poteškoće u tome kako dokazati uzrok i razlog akutnog abdomena.Acute pain in children can occur for different reasons. The term acute abdomen is considered to be a set of symptoms that occur with a sudden illness of the body\u27s organs. According to the International Association for the Study of Pain, pain is defined as "an unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage." Pain intensity can vary, starting from light, then severe, and finally agonizing and can occur as permanent or nonpermanent. It may be experienced as sharp, throbbing, agonizing, burning, stabbing or as a combination of these. We wanted to show a few patients with different problems and different clinical pictures that are not common in daily routine treatment. All patients had acute or chronic pain, and all were initially treated with medication. All of them required surgical treatment. When we have patients with abdominal pain we must always consider all the possibilities that could be causing the problems. There is often a diagnostic problem which is the cause of acute abdomen

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

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

A 10-Year Experience in the Treatment of Intraabdominal Cerebrospinal Fluid Pseudocysts

The aim of this retrospective study was to assess a ten-year experience in the treatment of rare complications of ventriculoperitoneal shunting – intraabdominal cerebrospinal fluid pseudocysts. At this time there are no data about incidence, clinical course and treatment of these complications in Croatia. Cerebrospinal fluid (CSF) abdominal pseudocyst is an uncommon but important complication of ventriculoperitoneal shunts. Retrospective data were obtained from 5 children with abdominal CSF pseudocysts, treated between 1996 and 2007. The incidence of intraabdominal CSF pseudocysts in our study is 2.9%. All patients were girls ranged in age from 4 to 12 years old (mean 8.8 years). In most cases etiology of hydrocephalus was congenital, idiopathic. Abdominal pain and distension were the most frequent clinical finding (4/5). Although infection has been reported as responsible for pseudocyst formation, we did not found it in our series. Laparotomy with cyst wall excision and catheter replacement was performed in 2/5 cases, and only cyst fluid aspiration with catheter replacement in 3/5 cases. Recurrence of the abdominal cyst was observed in one girl who was in terminal stadium of anaplastic ependymoma. It is our opinion that only catheter replacement and cyst fluid evacuation, as one of the treatment modalities, may be successful, even in large CSF intraperitoneal pseudocysts

LESS FREQUENT CAUSES OF ACUTE SURGICAL PAIN IN CHILDREN - OUR EXPERIENCE IN THE PAST 10 YEARS

Akutna bol se kod djece može pojaviti zbog različitih razloga. Pod pojmom akutni abdomen smatra se skup simptoma koji se javljaju kod iznenadne bolesti tjelesnih organa. Prema Međunarodnoj udruzi proučavanja boli, bol je definirana kao "neugodno osjetno i osjećajno iskustvo povezano s pravom ili potencijalnom ozljedom tkiva, ili uvjetovano tom štetom ili ozljedom". Bol se po intenzitetu može razlikovati od lagane, teške te naposljetku agonizirajuće, i to kao stalne ili nestalne. Bol se može doživjeti kao oštru, pulsirajuću, mučnu, goruću, strijeljajuću ili kao kombinaciju navedenih. Željeli smo prikazati nekoliko bolesnika s različitim problemima i različitim kliničkim slikama, koji nisu uobičajeni u svakodnevnom rutinskom liječenju. Svi su bolesnici imali akutnu ili kroničnu bol, a svi su u početku bili liječeni medikamentima. Kod svih je bolesnika bilo potrebno kirurško liječenje. Kod postojanja boli u trbuhu moramo uvijek razmišljati o svim mogućnostima koje bi mogle izazvati tegobe. Često postoje dijagnostičke poteškoće u tome kako dokazati uzrok i razlog akutnog abdomena.Acute pain in children can occur for different reasons. The term acute abdomen is considered to be a set of symptoms that occur with a sudden illness of the body\u27s organs. According to the International Association for the Study of Pain, pain is defined as "an unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage." Pain intensity can vary, starting from light, then severe, and finally agonizing and can occur as permanent or nonpermanent. It may be experienced as sharp, throbbing, agonizing, burning, stabbing or as a combination of these. We wanted to show a few patients with different problems and different clinical pictures that are not common in daily routine treatment. All patients had acute or chronic pain, and all were initially treated with medication. All of them required surgical treatment. When we have patients with abdominal pain we must always consider all the possibilities that could be causing the problems. There is often a diagnostic problem which is the cause of acute abdomen

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

Treatment of rectal prolapse in children with cow milk injection sclerotherapy: 30-year experience

AIM: To evaluate the role and our experience of injection sclerotherapy with cow milk in the treatment of rectal prolapse in children