Repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation - observations of a paediatric surgeon.

Introduction: Congenital diaphragmatic hernia (CDH) is a defect involving herniation of the abdominal organs into the pleural cavity through an incompletely closed diaphragm. This defect is also accompanied by various types of pulmonary and bronchial hypoplasia and serious anomalies in the pulmonary vascular bed and pulmonary hypertension. Extracorporeal membrane oxygenation (ECMO) is used in extreme cases resistant to standard treatment. There are no reports clearly indicating the optimal time to make a decision about the repair of CDH in paediatric patients on ECMO. In high-risk neonates (with severe hypoplasia and pulmonary hypertension), this repair procedure is postponed until the cardiopulmonary parameters are stabilized and pulmonary pressure reduced. Aim: To present the experience of paediatric surgeons regarding congenital diaphragmatic hernia repair in neonates on extracorporeal membrane oxygenation. Material and methods: CDH was surgically repaired in 17 neonates, and of these 7 were on ECMO. Results: Surgical problems were encountered intraoperatively: positioning of the patient forced by the cannulae inserted in the carotid vessels, significant generalized oedema, significant capillary bleeding from the surgical wound, difficulties with closing the abdominal cavity. The most common postoperative complications included bleeding from various areas. Conclusions: Each surgery in a neonate with CDH on ECMO is a challenge for the whole medical team involved in the procedure.

Dyssynergic Defecation and Anal Sphincter Disorders in Children in High-Resolution Anorectal Manometry Investigation.

OBJECTIVES: Dyssynergic defecation is a common disorder in children with functional constipation (FC) because of relaxation disorders of the sphincter apparatus and intra-rectal pressure during defecation. The aim of the study was to determine frequency and type of dyssynergic defecation and to assess pressure in the anal canal poles during simulated evacuation and function of puborectalis muscle in defecation in children with FC. METHODS: Three-dimensional (3D) high-resolution anorectal manometries (3D HRAM) were performed in 131 children with FC. In the manometric test, resting pressure measurements were assessed in 4 measuring poles of the anal canal. RESULTS: One hundred thirty-one children ages 5 to 17 years (mean age 10.2; SD ±â€Š3.8; median 10) were involved in the study (69 girls and 62 boys). Dyssynergic defecation was shown in 106/131 (80.9%) examined children. A statistically significant difference between the age of examined children (P < 0.02) and intrarectal pressures at the anal canal measuring points (left P < 0.009, right P < 0.005, anterior P < 0.01) was found. Correlation between the residual pressure values in lateral anal canal measurement poles and intrarectal pressure was demonstrated in all types of dyssynergy (left: r = 0.69, P < 0.0005; right: r = 0.74, P < 0.0005). In a group of 53/131 (40.5%) children, 3D HRAM showed a rectal pressure increase during simulated defecation, because of the dysfunction of the puborectalis muscle. CONCLUSION: The increase in sphincter pressure in lateral and posterior poles in I and II types of dyssynergia and in lateral poles in other types of dyssynergia may depend on relaxation disorders of the puborectalis muscle during defecation.

Percutaneous Intratumor Laser Ablation for Fetal Sacrococcygeal Teratoma.

OBJECTIVE: To evaluate the efficiency of percutaneous intratumor laser ablation for fetal solid sacrococcygeal teratoma (SCT). SUBJECTS AND METHODS: We carried out percutaneous ultrasound-guided intratumor laser ablation through a 17-gauge needle using an output of 40 W in 7 fetuses with large solid SCT and reviewed the literature for minimally invasive therapy for this condition. RESULTS: Laser ablation was carried out at a median gestational age of 20 (range 19-23) weeks, and in all cases there was elimination of obvious vascularization within the tumor and improvement in cardiac function. Three (43%) babies survived and had surgical excision of the tumor within 2 days of birth, 3 liveborn babies died within 5 days of birth and before surgery, and 1 fetus died within 2 weeks after the procedure. In previous series of various percutaneous interventions for predominantly solid SCT the survival rate was 33% (2/6) (95% CI 9.7-70%) for endoscopic laser to superficial vessels, 57% (4/7) (95% CI 25-84%) for intratumor laser, 67% (8/12) (95% CI 39-86%) for intratumor radiofrequency ablation, and 20% (1/5) (95% CI 3.6-62%) for intratumor injection of alcohol. CONCLUSIONS: In solid SCT, the reported survival from intratumor laser or radiofrequency ablation is about 50%, but survival does not mean success, and it remains uncertain whether such interventions are beneficial or not because the number of fetuses is small and there were no controls that were managed expectantly.

Congenital cystic adenomatoid malformation - diagnostic and therapeutic procedure: 8-year experience of one medical centre.

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a rare anomaly. The mechanisms and the time at which the abnormality develops are still unclear. The malformation is characterized by the presence of single large or multiple but smaller cysts. AIM: To present the experience of our medical centre, the Polish Mother's Memorial Hospital - Research Institute. MATERIAL AND METHODS: We analysed the medical records of 32 neonates hospitalized in 2008-2017 at the Department of Paediatric Surgery and Urology ICZMP due to pre- or postnatally diagnosed congenital cystic adenomatoid malformation. RESULTS: In 2008-2017 32 children were hospitalized at our department due to prenatally diagnosed CCAM. An intrauterine procedure of thoracoamniotic shunting was performed in 13 fetuses. All newborns had chest computed tomography. Surgery had been performed in 21 children. Histopathological analysis of specimens prepared from the resected lung tissue confirmed type 1 CCAM in 4 cases, type 2 CCAM in 11 cases and type 3 CCAM in 1 case. Four patients were diagnosed with a bronchogenic cyst and one with extralobar pulmonary sequestration. CONCLUSIONS: Postnatal management of CCAM depends on the clinical status of the newborn. Newborns without clinical symptoms require radiological diagnostics to confirm the presence of malformation. Surgical treatment is recommended before the age of 10 months. Resection of the malformed lung fragment in patients with CCAM is the first-line treatment. It should be emphasized that despite the tremendous advances in diagnostic imaging, the final diagnosis of CCAM is made based on histopathological findings.

Thoracoamniotic Shunts in Macrocystic Lung Lesions: Case Series and Review of the Literature.

OBJECTIVE: To evaluate the efficiency of thoracoamniotic shunts for drainage of macrocystic-type congenital cystic adenomatoid malformation (CCAM). SUBJECTS AND METHODS: This was a retrospective study of 12 fetuses with a large thoracic cyst treated with thoracoamniotic shunting between 2004 and 2014 in a tertiary fetal therapy center. Medline was searched to identify cases of CCAM treated with thoracoamniotic shunting. RESULTS: In all cases the thoracic cyst was associated with major mediastinal shift, the CCAM volume ratio (CVR) was >1.6, and in eight cases there was associated hydrops. Shunt insertion was successfully carried out in all cases at a median gestational age of 24 weeks (range 18-34). In 10 cases there was live birth at a median age of 38 weeks (range 35-41), but in two hydropic fetuses there was intrauterine death. A literature search identified a total of 98 fetuses with CCAM treated with thoracoamniotic shunting between 1987 and 2016. In the combined data from the previous and the current study, the survival rate was 77% (53 of 69) for hydropic and 90% (37 of 41) for nonhydropic fetuses. CONCLUSIONS: The role of thoracoamniotic shunting in macrocystic lung lesions associated with hydrops is well accepted. Intrauterine intervention is also likely to be beneficial in the subgroup of nonhydropic fetuses with a CVR >1.6.

Difficulties in prenatal diagnosis of tumour in the fetal sacrococcygeal area.

Prenatal ultrasound at the 20th week of gestation revealed a 3-cm tumour in the sacrococcygeal area. Initially, a sacrococcygeal teratoma was suspected on the basis of fetal ultrasonography, which revealed normal heart anatomy and an increasing tumour mass. The diagnosis was then changed to fetus in fetu or teratoma. Prenatal magnetic resonance imaging at the 34th week of pregnancy confirmed the ultrasound diagnosis. No other anomalies were found. Elective caesarean section was performed at term. The care team included a paediatric surgeon, obstetricians, neonatologists, midwives, and an anesthesiologist. A female newborn was delivered in good condition. The tumour was resected in the operating room and mature teratoma was established by histopathological evaluation. Surprisingly, agenesis of the right forearm was revealed which had not been detected prenatally, despite many examinations (both in our hospital and earlier at a primary care obstetrician office).

[Fetal therapy--evaluation of intrauterine therapy in congenital cystic adenomatoid malformation of the lung (CCAM)]. / Terapia plodu--ocena skutecznosci leczenia wewnatrzmacicznego wrodzonego zwyrodnienia gruczolakowato-torbielowatego pluc (CCAM).

OBJECTIVES: The aim of the study was to evaluate the efficiency of intrauterine treatment of large cysts in fetal lungs using thoracoamniotic shunts. MATERIAL AND METHODS: Our observational retrospective study was carried out on a series of 8 fetuses who under went thoracoamniotic shunting after sonographic statement of large macrocystic lesions in the lungs at the Department of Gynecology Fertility and Therapy of the Fetus, Polish Mother's Research Institute, between 2009-2014. RESULTS: Mean gestational age at shunt insertion was 26.6 (range 18-33) weeks. Marked mediastinal shift in the echocardiographic examination was observed in all of the investigated cases. Five fetuses had polyhydramion, with 4 hydropic cases. Out of the remaining 4 fetuses without impaired cardiac function, 3 had very large lesions at initial presentation and 1 had a lesion that was rapidly increasing in size. Shunt insertion was successful in all cases. Only one patient went into premature labor (at 36 weeks of gestation). Mean gestational age at delivery was 38.2 weeks. Cesarean section was necessary in the half of the patients due to obstetric complications. All newborns underwent resection of the lesions. Three of them were operated in the first month after birth. The rest of the operations were postponed. Prenatal diagnosis of congenital cystic adenomatoid malformations was confirmed by pathologists in all cases. CONCLUSION: Intrauterine therapy of macrocystic lesions in fetal lungs enables to achieve good perinatal outcome It needs to be considered in every case of a fetus with developing impaired cardiac function.

[Comparative analysis of interuterine therapy of unilateral and bilateral fetal obstructive uropathy]. / Analiza pórownawcza wyników terapii wewnatrzmacicznej obustronnej i jednostronnej uropatii zaporowej.

OBJECTIVE: The goal of the work was a comparative analysis of intrauterine therapy among pregnant women with diagnosed fetal uropathy and unilateral hydronephrosis. METHODS: The study was conducted on a group of 98 pregnant women hospitalized at the Department of Gynecology Fertility and Fetal Therapy Polish Mother's Memorial Hospital between 2006-2012. The study group included 77 cases of fetal obstructive uropathy (79%) and 21 cases of unilateral hydronephrosis (21%). Both groups have been analyzed in terms of prognosis. Follow-up has been conducted and results have been statistically analyzed. RESULTS: In 11% of cases of obstructive uropathy therapy early complications were observed. The remaining 89% of the patients delivered at a later date, of which 54% after 34 weeks of pregnancy The average weight of a newborn was 2645g and Apgar score - 7.45. 73% of newborns were discharged home in good condition, with preserved diuresis. CONCLUSIONS: 1. The use of intrauterine therapy has a positive effect on fetal condition and newborn postnatal state. 2. Unilateral hydronephrosis group was characterized by a significantly longer duration of pregnancy higher birth weight, Apgar score, and greater survival of newborns. 3. In the group of obstructive uropathy survival improvement was achieved and dieresis was preserved Intrauterine interventions were associated with a low risk of complications.

Long-term follow-up of children with congenital diaphragmatic hernia--observations from a single institution.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is a complex malformation. The majority of children after CDH repair lead normal life, with good exercise tolerance. However, in some patients, long-term sequelae are observed, resulting from the primary defect and implemented treatment. MATERIAL AND METHODS: We evaluated (basing on clinical examination and review of medical records) the incidence of the most common long-term complications after surgical repair of CDH and determined their risk factors. The study group comprised 50 children operated in our department for CDH. RESULTS: The following long-term complications were seen: recurrent respiratory system infections (34%), pathological gastrointestinal reflux (30%), body mass deficiency (20%), and chest deformations (40%). Parents reported most common symptoms from the gastrointestinal tract (48%) and recurring respiratory system infections (34%). Perfusion of the ipsilateral lung was significantly lower in the group of children in whom negative prognostic factors were present: patch repair, transposition of the liver up CDH, lung hypertension in echocardiography, high-frequency oscillatory ventilation (HFOV) use, prolonged artificial ventilation. In lung scintigraphy, in 70% of patients ipsilateral lung hypoplasia was found. There was a correlation between gastroesophageal reflux (GER) and early detection of CHD in prenatal studies and the presence of polyhydramnios. Patients with GER also required longer artificial ventilation and longer hospitalization. Significant body mass deficiency (below 3rd centile) was found in 20%, correlated with patch repair and HFOV. Chest deformations were more common in children who required synthetic patch repair and artificial ventilation for longer than 10 days. CONCLUSIONS: The most common long-term complications observed in children after CDH repair are recurrent infections of the respiratory tract, lung hypoplasia, pathological GER, somatic growth abnormalities, and skeletal deformations. Children operated for CDH should be followed up regularly for many years by a multispecialty medical team. Diagnostics and/or follow-up of the above complications require detailed clinical examination, lung perfusion scintigraphy, spirometry, and 24-hour pH-metry.

[Successful intrauterine therapy in a fetus with a large lung cyst in the third trimester of pregnancy--case report]. / Skuteczna interwencja wewnqtrzmaciczna u plodu z olbrzymiq torbiela pluca w trzecim trymestrze ciazy--opis przypadku.

Congenital cystic adenomatoid malformation is a rare disorder of the respiratory system which occurs with an incidence of 1/25,000-35,000. Depending on the size of the cysts, CCAM is classified into microcystic and macrocystic. Very large lesions carry a significant risk of causing both, pulmonary hypoplasia due to compression of the lung tissue and fetal hydrops, probably due to impaired cardiac function as a result of mediastinal shift and compression of the vena cava. In this report, we present a case of a fetus with prenatally diagnosed large cyst of the left lung. Despite advanced gestational age, the baby was treated by thoracoamniotic shunting owning to developing impaired cardiac function and polyhydramion. Spectacular effects of the therapy have been achieved.

Pulmonary torsion as an atypical complication of congenital esophageal atresia repair-a case report and review of literature.

Pulmonary torsion is a severe, life-threatening event, rarely occurring in children. We present a case of atypical postoperative complication of esophageal atresia repair in the form of pulmonary torsion comprising the middle lobe of the right lung. Clinical deterioration in the face of normal arterial blood gases should rise a high index of suspicion for pulmonary torsion. Early diagnosis is crucial, treatment is surgical, detorsion if possible, or if the lobe is not viable-resection.

[Bezoars - diagnostic problems based on own observations and literature review]. / Bezoary-Trudnosci Diagnostycznena Przykladzie Wlasnych Obserwacjii Przeglad Pismiennictwa.

INTRODUCTION: Bezoars are concretions of human or vegetable fibers that accumulate in the gastrointestinal tract of humans and animals. Patients may remain asymptomatic for many years, and symptoms develop as bezoars increase in size to the point of obstruction or perforation. THE AIM OF THE STUDY WAS TO PRESENT: 3 cases of bezoars treated in our centre and review of the current knowledge about this disorder, emphasizing the diagnostic difficulties. CONCLUSIONS: Physicians should consider bezoars as a differential diagnosis of acute abdomen. As bezoars may remain asymptomatic for many years, monitoring of pathogenetic and predisposing factors is essential, in particular in compulsive disorders.

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.

[Ileus due to adhesions as a consequence of abdominal surgery in childhood - analysis of 94 cases]. / Niedroznosc zrostowa jelit po operacjach brzusznych u dzieci - analiza 94 przypadków.

INTRODUCTION: Ileus due to adhesions is a common postoperative complication. However, there are only rare reports of this condition occuring in children. The aim of this study was to determinate the risk of incidence of intestinal adhesion ileus in children after abdominal operations. MATERIAL AND METHODS: Retrospective studies were carried out on a group of 94 children operated in the Department of Pediatric Surgery and Urology of the Polish Mother's Memorial Hospital - Research Institute in Lódz in the years 1996-2005, in whom adhesions were found on relaparotomy. The following parameters were analysed:indication for operation age at first laparotomy, timeintervalbetweenl aparotomyandrelaparotomy, localization and extent of adhesions, complications after adhesiolysis, indications for relaparotomy. RESULTS: Among 1987 children in whom laparotomy was performed, in 94 intestinal adhesions were found. The majority of relaparatomies were performed in the first 6 months after the primary operation. CONCLUSIONS: Adhesive bowel obstruction is a frequent and serious complication after laparotomy especially in the neonatal period. It occurs most frequently in the 6 months after the primary operation.

[Late manifestation of congenital diaphragmatic hernia - case report]. / Pózna manifestacja wrodzonej przepukliny przeponowej - opis przypadku.

Congenital diaphragmatic hernia (CDH) in most cases presents immediately or within hours after birth with signs of respiratory failure: dyspnea, tachypnea, and cyanosis. Late presentation of CDH (beyond the neonatal period) is less common and represents true diagnostic challenge. Diagnosis of the defect is difficult. Symptoms are non-specific and can be misleading. Late onset of this anomaly must be considered in the differential diagnosis of chronic gastrointestinal or respiratory symptoms. The aim of this report is a case of 17 year old boy with delayed presentation of congenital diaphragmatic hernia.

[Environmental conditions and family prevalence of obesity in children]. / Uwarunkowania srodowiskowe oraz rodzinne wystepowania otylosci u dzieci.

UNLABELLED: Obesity, which is a chronic systemic disease, is one the main health problems in contemporary society. Prevalence of obesity in childhood obesity and promotes the occurrence of many chronic diseases into adulthood. The aim of the study was to assess the relationship between obesity and child obesity, and lifestyle conditions in the family. MATERIAL AND METHODS: The study group included 100 children aged 4-18 years, with a body mass index (BMI) above 97 centile. Respondents (44 girls and 56 boys) were selected from among children treated in the period from April 2008 to May 2009, the Department of Pediatric Surgery and Urology with general diseases, scheduled treatments not associated with obesity. In each patient, based on a prepared questionnaire, has been interviewed for dietary habits, family history in the direction of obesity and other comorbidities. The measurements of height and weight and abdominal circumference and waist were also made. RESULTS: The studies show that obesity is most common in children aged 9-13 years. The majority of obese children has errors habits. In the study group found the relationship between the prevalence of obesity in children and obesity occurring in the immediate family. It was observed that, in the oldest patients (14-18 years) most attempts to treat obesity. CONCLUSIONS: The percentage of obesity in children and their parents were similar and remained tied with overweight and obesity in the family. Therapeutic interventions are undertaken mainly in older children. In addition, children aged 9-13 years usually reach for foods with a fast food, which can negatively associated with rarely undertaken interventions aimed at weight loss.

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.

Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood.

Interstitial cells of Cajal (ICCs) are pacemaker cells which are densely distributed throughout the whole gastrointestinal tract. ICCs have important functions in neurotransmission, generation of slow waves and regulation of mechanical activities in the gastrointestinal tract, especially for the coordinated gastrointestinal peristalsis. Therefore, a loss of ICCs could result in gastrointestinal motor dysfunction. In recent years c-kit labeling has been widely used to study pathological changes of ICCs in gastrointestinal motility disorders. Paediatric gastrointestinal motility disorders such as hypertrophic pyloric stenosis, Hirschsprung's disease, total colonic aganglionosis, hypoganglionosis, intestinal neuronal dysplasia, internal anal sphincter achalasia, megacystis microcolon intestinal hypoperistalsis syndrome have been reported to be associated with loss or deficiency of ICCs networks. This review describes the distribution of ICCs in the normal gastrointestinal tract and its altered distribution in intestinal motility disorders of childhood.

Interstitial cells of Cajal in the human normal urinary bladder and in the bladder of patients with megacystis-microcolon intestinal hypoperistalsis syndrome.

OBJECTIVE: To investigate the distribution of c-kit-positive interstitial cells of Cajal (ICCs) in normal bladder and bladders from patients with megacystis-microcolon-intestinal peristalsis syndrome (MMIHS, a rare congenital and generally fatal cause of functional intestinal obstruction in the newborn), the most characteristic feature of which is abdominal distension caused by a distended unobstructed urinary bladder. PATIENTS AND METHODS: Full-thickness bladder specimens were obtained from four infants with MMIHS and four controls, and processed as paraffin-wax and frozen sections. Sections were assessed using single immunohistochemistry with monoclonal and polyclonal anti-c-kit antibodies. Anti-alpha-smooth muscle actin (SMA) antibody was used to investigate the contractile apparatus in smooth muscle cells of the urinary bladder. Specimens were examined using light and confocal scanning microscopy. RESULTS: There were many c-kit positive ICCs in the normal urinary bladder, appearing as small, long, bipolar cells with only two long and several short processes. In contrast, ICCs were absent in the MMIHS bladder. alpha-SMA immunoreactivity was lower in MMIHS urinary bladder than in control sections. CONCLUSION: This study shows for the first time the presence of c-kit-positive ICCs in the normal human urinary bladder. The lack of ICCs in the MMIHS bladder may contribute to the voiding dysfunction in this disease.

Increased expression of mast cells in reflux nephropathy.

End-stage renal disease is characterized by interstitial infiltrate of inflammatory cells in association with tubular atrophy and interstitial fibrosis. Mast cells (MCs) secrete a large number of fibrogenic factors and have been implicated in chronic inflammatory processes with fibrous tissue deposition. The aim of this study was to investigate the distribution of MCs in kidneys with reflux nephropathy (RN) and to determine the relationship between MCs and the interstitial fibrotic process in RN. Kidney specimens from 12 patients (aged 2-13 years) with severe RN secondary to primary high-grade vesicoureteral reflux, obtained at the time of nephrectomy, and 5 controls were examined. Sections were investigated histochemically by acid toluidine blue (TB) and immunohistochemically with antibodies for anti MC-tryptase, MC-chymase, c- kit (CD117), and fibronectin. Double staining for fibronectin and MC-tryptase was performed and examined using confocal scanning microscopy. TB histochemistry showed a marked increase of MCs in RN specimens compared with controls. MC-tryptase, chymase, and c- kit immunopositive MC infiltration was significantly higher in RN samples (14.2+/-9.6) than controls (1.3+/-0.8), ( P<0.05). In all the sections there were more MC-tryptase-positive cells than MC-chymase-positive MCs. Double staining showed increased immunoreactivity of MCs and fibrosis in the renal interstitium of kidneys with RN. The number of infiltrating tryptase-positive MCs was correlated with the degree of interstitial renal scarring. This study demonstrates for the first time the increased expression of MCs in RN, suggesting that MCs may be involved in the development of scarring in RN.