Different distribution of c-kit positive interstitial cells of Cajal-like in children's urinary bladders.

We describe the presence of c-kit positive interstitial cells of Cajal-like (ICCs-like) in the walls of the urinary bladders of children. An immunohistochemical study of specimens, obtained at autopsy from either the trigonum (Group A) or the corpus (Group B), was performed using antibodies against c-kit (CD 117). Histological morphometry of the immunoexpression of c-kit positive ICCs-like was performed by means of image analysis system. The c-kit positive ICCs-like were identified by their morphology and counted in the vesical muscle layer in ten adjacent high power fields, each of 0.0479 mm(2). The areas of the epithelial and subepithelial layers containing c-kit positive mast cells (rounded body with no dendritic processes) were neglected. The results were expressed as the number of ICCs-like cells per mm(2). Differences between groups were tested using unpaired Student's t-test preceded by evaluation of normality and Levene's test. Results were considered statistically significant if p 〈 0.05. In Group A, the mean number of ICCs-like cells was statistically significantly higher (41.5 cells/mm(2)) than in Group B (30.4 cells/mm(2)), p 〈 0.05. ICCs-like cells were found within the smooth muscle layer of the urinary bladder. There was a different distribution of these cells in particular parts of the bladder, which was probably due to the different roles of the trigonum and the corpus in the bladders of children.

Thoraco-omphalopagus twins: different perinatal circumstances, different outcome.

Two pairs of omphalopagus twins were encountered at the Polish Mother's Health Institution in Lodz, Poland during the past 15 years. In the first set the prenatal diagnosis was not established and the delivery of the twins in a regional hospital was a complete surprise. Both babies died. In the second case the conjoined twins were diagnosed prenatally, surgical separation was successful, and both twins survived. The prenatal identification of conjoined twins is of cardinal importance for the planning of delivery and possible separation.

[Fetal echocardiography in 83 fetuses with omphalocele from Dept. for Diagnoses and Prevention of Fetal Malformations, Research Institute Polish Mother's Memorial Hospital, and Medical University of Lodz, (1999-2006)]. / Znaczenie badania echokardiograficznego w przepuklinie pepowinowej (Pp) u 83 plodów w materiale Zakladu Diagnostyki i Profilaktyki Wad Wrodzonych ICZMP i Uniwersytetu Medycznego w Lodzi w latach 1999-2006.

MATERIAL AND METHODS: It was a retrospective analysis of fetal echocardiography results (analysis of congenital heart defect (CHD) and functional abnormalities in normal heart anatomy) in 83 fetuses diagnosed and treated in the same institution. RESULTS: In our group of fetuses, the most common structural defect, in addition to omphalocele, proved to be CHD. In the group of omphalocele, normal heart anatomy and functional abnormalities (tricuspid regurgitation, myocardial hypertrophy, arrhythmias), the hospital stay was longer comparing to the group of omphalocele, normal heart anatomy and normal heart study (t-test p = 0.005). CHD was a bad prognostic factor and most fetuses/newborns did not survive (Chi-square 0.0005, Fisher test 0.000032). In the group of omphalocele and normal heart anatomy based on fetal echocardiography, the survival rate was 70%. The omphalocele and CHD survivors (n=3) had been only those who did not require a cardiac surgery in first month of postnatal life. CONCLUSION: Echocardiography exam of fetus with omphalocele and CHD and functional abnormalities has diagnostic and prognostic value.

[Fetal echocardiography in fetal ovarian cysts]. / Badanie echokardiograficzne u plodów z torbiela jajnika.

OBJECTIVES: The aim of the study was to evaluate the outcome of fetuses with ovarian cysts in relation to fetal echocardiography. MATERIAL AND METHODS: In the Department for Diagnosis and Prophylaxis of Birth Defects at the Polish Mother's Memorial Hospital in Lodz fetal echocardiography was performed in 21 fetuses with ovarian cysts between the years 1995-2006. OUTCOMES: In 18 out of 21 (86%) fetuses we have found normal heart anatomy (NHA) and in 3 fetuses congenital heart defect (CHD). In 7 out of 18 (39%) fetuses with NHA there were functional anomalies, including 5 fetuses with hypertrophy Four neonates with hypertrophy required surgical procedures after the delivery. Prenatal hypertrophy was not observed in case of only one neonate which was operated after the delivery. CONCLUSIONS: 1. Fetal echocardiography studies were very often abnormal in fetus with ovarian cysts (mainly as functional anomalies). 2. Functional anomalies detected in fetal echocardiography more often resulted in surgical procedures after the birth, whereas normal heart study was more often connected with spontaneous regression of ovarian cyst (p=0.0265).

[Peritonitis caused by a giant trichobezoar ventriculi--a case report]. / Objawy ostrego brzucha wywolane bezoarem zoladka--opis przypadku.

Bezoars are the most common foreign bodies of gastrointestinal tract. They contain swallowed hair, different fibres, seeds, nut shells, sweets or drugs. Clinical manifestations vary, depending on the location and size of bezoar, from "no symptoms" to "acute abdomen" syndrome. In this case report, the authors describe a giant trichobezoar from the stomach of an 18-year-old girl, who had been admitted to the gynecological department with syndromes suggesting an ovarian cyst torsion. The preoperative diagnosis was not confirmed and a giant trichobezoar was found and removed during the surgery.

[Diaphragmatic hernia in reference hospital ICZMP--diagnostic problems and outcome]. / Problemy diagnostyczne i losy plodów z przepuklina przeponowa w osrodku referencyjnym ICZMP w latach 1994-2006.

OBJECTIVES: The aim of the study was to analyze US/ECHO examinations in fetuses with diaphragmatic hernia (DH) diagnosed and treated in our institution from 1994-2006, and their follow-up. MATERIAL AND METHODS: Retrospective analysis of the data base from Department for Diagnoses & Prevention of Fetal Malformations, Research Institute of the Polish Mother's Memorial Hospital: 14,481 fetal echo/ultrasound examinations in 10,077 fetuses have been analyzed to retrieve 115 fetuses with DH. RESULTS: The mean gestational age at the targeted US/ECHO examination was 30 wks. There were 8 terminations of pregnancies (at mean 21 wks), 6 intrauterine demises, 60 neonatal deaths after delivery (in 1-3rd day of postnatal life), 8 deaths after surgery, 19 neonates were discharged home and in 14 cases the follow-up could not be monitored. The most common anomalies accompanying DH have been central nervous system anomalies (20%), polyhydramnion (16%) and cong heart defects (10%). In this subgroup, there was 100% mortality. Isolated DH has been diagnosed in every third case. In this subgroup, 27 neonates had undergone surgery and the survival rate was 70%, however since 2004 there was not a single death on record. CONCLUSIONS: Late gestational age of US/ECHO examinations in our tertiary center suggests that DH has been relatively difficult to detect during ultrasound screening. DH and the other structural malformations have been a lethal disease in our series in 100%. Isolated DH was much less frequent and was present in every third case (29%), and in this group the survival rate was 70%, regardless of the way of the delivery (CS or Vaginal).

[Assessment of the usefulness of ultrasound screening in fetal ovarian cysts]. / Ocena przydatnosci ultrasonografii u plodów z torbiela jajnika.

OBJECTIVES: The aim of the study was to assess the usefulness of ultrasound in management and prognosis in fetal ovarian cysts. MATERIAL AND METHODS: The study included 38 fetuses with cyst in abdominal cavity, who, between 1995 and 2006, underwent an ultrasound examination in our unit at the Polish Mother's Memorial Hospital in Lodz, The Department for Diagnosis and Prevention of Birth Defects. RESULTS: In all 38 fetuses with cyst in abdominal cavity we have diagnosed 27 (74%) cases of ovarian cyst. In 14 (74%) fetuses cysts regressed spontaneously, including all cysts < or = 40mm (n = 7). In 3 cases with cysts > 40mm needle aspiration has been successfully performed, without any further complications. Surgical neonatal treatment has been performed in 5 cases in prenatal cysts > 40mm without prenatal aspiration. In 3 cases cysts > 40mm regressed spontaneously. Ovarian cysts in 22 (87%) cases were an isolated malformation; in 5 (19%) cases other malformations were present CONCLUSIONS: 1. Fetal ovarian cysts < or = 40mm required only ultrasound assessment and, in majority of cases, revealed the tendency to spontaneous regression. 2. Cysts > 40mm in maximal diameter have signaled complications more often and required surgical procedure after birth. 3. In utero, aspiration of fetal ovarian cyst > 40mm may lead to cyst regression, making the surgery after birth unnecessary.

[Natural history of fetal/newborn ovarian cyst]. / Historia naturalna plodowych/noworodkowych torbieli jajnika.

OBJECTIVE: Fetal and neonatal ovarian cysts are detected freguently by ultrasonography. Prenatal ovarian cysts have a follicular or luteal origin. Their natural history is a spontaneous involution. Pre- and postnatal changes in the sonographic aspects allow to choose the appropriate therapeutical indications: conservative approach, surgery, or percutaneous aspiration. MATERIALALS AND METHODS: In retrospective review from 1992-2004 authors present 26 patients with ovarian cysts. RESULTS: Fifteen patients were diagnosed by prenatal ultrasonography carried out between 33 and 39 weeks of gestation. Postnatally 14 were observed only and their cysts regressed, 10 with cysts larger than 4 cm, had surgical exploration, 2 patients with cysts between 2 to 4 cm were candidates for percutaneous aspiration. CONCLUSION: 1. The conservative treatment and clinical observation using ultrasonography is recommended when the ovari cystic is 40 mm in diameter. 2. When the ovari cystic is bigger then 40 mm in diameter it is recommended to perform puncture and fluid aspiration from the cystis under ultrasonography control. 3. The surgical intervention was planned when the ovari cystis was heterogenous and has been growing for a few months.

[Gastroschisis prenatally and postnatally]. / Wrodzone wytrzewienie prenatalnie i postnatalnie.

OBJECTIVE: The aims of the study were: 1) evaluation of progress in surgical treatment of gastroschisis between 1990 and 2004 including the impact of prenatal diagnosis; 2) evaluation of mortality and coexisting congenital abnormalities in fetuses and newborns with gastroschisis. MATERIAL AND METHODS: Between 1990 and 2004 in Polish Mother's Health Institute gastroschisis was diagnosed in 108 cases. Basing on information from the Department of Pathology, Department of Pediatric Surgery and Department of Fetal Therapy, the patients were divided into 2 groups: 1) miscarried fetuses, still born neonates and non-operated live newborns who died within several hours after delivery (31 cases); 2) live born operated on newborns (77 cases). RESULTS: Until 1999 gastroschisis was diagnosed prenatally only in 30% of patients, while after 1999 the defect was diagnosed in 70% of cases. From the year 2000, due to close cooperation with obstetricians, newborns with gastroschisis have been operated on directly after delivery at the same theatre. In this period there were only 2 deaths due to prematurity (both twin deliveries)--5,4%. Main causes of deaths were: in group I--congenital defects of the cardiovascular and respiratory tracts and multiple defects; in group II--peritonitis, ileus due to adhesions and multiorgan failure. CONCLUSIONS: 1. Prenatal diagnostics and close cooperation between gyneacologists, obstetricians, neonatologists and pediatric surgeons significantly decreased mortality of newborns operated for gastroschisis in our Institute from 30% in 1990-1999 to 5,4 % in 2000-2004. 2. If miscarried or premature deliveries and deaths prior to operation are included, the mortality is 41%. 3. Deaths in the group of non-operated patients were caused by serious congenital defects not connected with gastroschisis.

[Treatment of lymphangioma with OK432 in children]. / Leczenie naczyniaków chlonnych u dzieci preparatem OK-432.

From 1998 to 2003 6 children with lymphangioma of cervico-facial region were treated with OK-432 in Polish Mother Health Institute. Three of them had nonresectable leasion and two required tracheostomy during first months of life. Each patient received from 1 to 10 sessions of obliteration. In total 23 sessions were performed. The youngest patient treated with OK-432 was 2 weeks and the oldest 2 years old. The response to treatment was very good in 4, good in 1 and fairly good in 1 case. In three patients criodestruction was applied as an additional treatment. In two children residual lesions were subsequently removed surgically. There were no serious complications. After obliteration sessions only 2-3 day temperature increase and local inflammation occurred. We consider obliteration with OK-432 as a treatment of choice in lymphangiomas in children.

[Evaluation of surgical treatment in gastroschisis based on personal experience]. / Ocena postepowania chirurgicznego w wytrzewieniu wrodzonym na podstawie badan wlasnych.

UNLABELLED: The ultimate goal of surgical treatment is the reduction of eviscerated bowel and full fascial closure of the abdominal defect; the sooner it is done -- the better, otherwise the risk of sepsis is overwhelming. On the other hand, however, forcible, one-step reduction may cause the development of the abdominal compartment syndrome. AIM OF THE STUDY: To summarize the personal experience concerning the surgical treatment of gastroschisis and to suggest the optimal procedure in this anomaly. MATERIAL AND METHODS: The analysis of the treatment of 67 neonates with gastroschisis who were operated on in our Department from 1994 through 2004 has been presented. In 51 of them, who were born in this Department, the diagnosis of gastroschisis had been established prenatally, in the remaining 16 -- postnatally. RESULTS: Prenatal diagnosis allows to undertake prenatal intervention, if indicated, and monitor the progress of anomaly and possible decision of earlier delivery. We found such indications in 8 of our patients in whom earlier delivery by caesarean section was performed and 9 others were given amnioinfusion. In the presented series -- in the years 1994-2000 multistep procedures with silo formation were performed more often, whereas in the years 2001-2004 we shifted our preference towards primary reduction of gastroschisis. The feasibility of primary reduction depends on the condition of eviscerated bowel and intra-abdominal pressure (monitored indirectly in the urinary bladder). In 8 cases (12%) coinciding bowel atresia was diagnosed. CONCLUSIONS: Prenatal diagnosis is of practical importance -- it allows for in utero treatment and if indicated earlier delivery. One step reduction of eviscerated bowel, if feasible is the treatment of choice. Otherwise, gradual reduction, with the use of silo is recommended.

[Beneficial effect of angiotensin converting enzyme inhibitor treatment in severe cystine urolithiasis]. / Korzystny efekt leczenia inhibitorem enzymu konwertujacego angiotensyne w ciezkiej cystynowej kamicy nerkowej.

Cystinuria is an autosomal recessive defect in transepithelial transport of dibasic amino acids (e.g. cystine) which involves the proximal canaliculi, small intestine and central nervous system. It is the least common cause of nephrolithiasis, accounting for 1 to 3% of renal calculi. The natural course of the disease, characterised by recurrent stone formation, can frequently lead to renal failure, if left untreated. Until recently, treatment of cystinuria has been limited to symptomatic management including intensive hydration and urine alkalinisation. Different drugs that react with cystine to form soluble complexes have been used but their efficacy remains questionable. We present the case of a 6-year-old boy with severe, recurrent cystine urolithiasis treated with captopril. The diagnosis of cystine urolithiasis was established after a 3-year course of clinically apparent nephrolithiasis, characterised by stone passage. At the age of 5 years he underwent lithotripsy and nephrolithotomy for removal of staghorn calculi. Since then treatment with citrate and magnesium supplementation combined with captopril was introduced. After a follow-up of 12 months the patient remained stone-free. Urinary cystine decreased from 230 to 136 mg per gram creatinine. We conclude that captopril can be useful in the treatment of cystine urolithiasis in children.

[Rare coexistence of phlegmonous appendicitis and tumor of a twisted left ovary]. / Rzadkie wspólistnienie ropnego zapalenia wyrostka robaczkowego i skreconego guza lewego jajnika.

The authors present a diagnostically difficult case of a three year old girl with abdominal pain. The girl with abdominal pains, nausea, upper airways infarction and some urinary system symptoms was admitted to Children's Surgical Clinic for observation. She was given antibiotic therapy and i.v. infusions. WBC was 29.6 tys./ul and CRP 2.7 mg/dl. No other abnormalities were detected in biochemical or sonographic investigation. The girl was submitted to laparotomy because of unclear abdominal signs suggesting acute appendicitis. Phlegmonous appendicitis and twisted/rotated left ovary with multiple adhesions were found. Histopathological investigation showed teratoma of the left ovary. Postoperative course went without complication.