Comparison of laparoscopic and open ileocecal resection for Crohn's disease in children.

PURPOSE: Ileocecal resection (ICR) is the most frequently performed surgery in paediatric Crohn's disease (CD) patients. The aim of the study was to compare laparoscopic-assisted and open ICR. METHODS: Retrospective review of consecutive CD patients undergoing ICR between March 2014 and December 2021 was performed. The patients were divided into open (OG) and laparoscopic (LG) groups. Compared parameters included patients' demographics, clinical characteristics, surgery, duration of hospitalisation and follow-up. Complications were classified according to the Clavien-Dindo classification (CDc). Risk factors were identified using multivariable analysis. RESULTS: Sixty-two patients (29 females, 46.7%) were included in the analysis, forty-two patients in OG. The median duration of surgery was 130 in OG versus 148 in LG (p = 0.065) minutes. Postoperative complications were reported in 4 patients (12.1%). There was no significant difference in postoperative complications according to CDc (OG 7.14 vs LG 5%, p = 1). The median length of hospitalisation was 8 in OG and 7 days in LG (p = 0.0005). The median length of follow-up was 21.5 months. CONCLUSION: The laparoscopic-assisted approach had shorter hospital stay and was not associated with increased risk of 30-day postoperative complications. Laparoscopic surgery should be considered the preferred surgical approach for primary ICR.

Surgical treatment of Crohns disease in children in the era of biological treatment.

INTRODUCTION: The incidence of Crohns disease in the paediatric population has been increasing and requires surgical treatment in addition to conservative therapy. While surgical treatment used to be the last step after the failure of all conservative therapies, nowadays it is a standard part of complex treatment. Surgery can enter the treatment process at any stage of the disease and, with a proper indication, timing and preoperative optimization, it can induce immediate remission in patients. On the other hand, with inadequate or improper preoperative preparation and indication, surgical treatment can cause serious or even life-threatening complications. The spectrum of patients undergoing surgery is changing in the era of biological therapy. The aim of this review was to summarize the current knowledge of the impact of biological (anti-TNF alpha) therapy on the development of postoperative complications in children and adolescents operated for Crohns disease. METHODS: We present a review based on literature available in MEDLINE-PubMed and Embase databases. CONCLUSION: According to current knowledge, no association was found between biological treatment in the preoperative period and the development of postoperative complications in paediatric patients. Surgical treatment of paediatric patients with Crohns disease is one of standard treatment modalities.

[Artificial bowel sphincter ten years later]. / Umelý sverac anu po deseti letech.

INTRODUCTION: Congenital anorectal malformation is the most common cause of fecal incontinence in children and young adults. Surgical treatment options are limited. One of the treatment methods is the implantation of an artificial bowel sphincter. The goal of this study was to investigate the efficacy of the artificial bowel sphincter in reaching long-term fecal continence in patients with anorectal malformation. METHODS: Young adults with fecal incontinence due to anorectal malformation were included in the study. Those who underwent an artificial bowel sphincter implantation were followed each year for a period of 10 years. Outcome measures included the level of continence and the need for reoperation including explantation. RESULTS: Out of the 20 patients with congenital anorectal malformation, nine underwent implantation. Three devices were explanted within the first year; two were permanently removed and one was replaced. Thus, after the first year, seven patients (78 %) had a functional artificial bowel sphincter. Four patients (44 %) had a fully functional artificial bowel sphincter at 10 years after implantation. Although the artificial bowel sphincter improved the level of continence in all patients, full continence was not achieved in any of them. In one patient the artificial bowel sphincter was explanted after 10 years upon her request and a colostomy was created. CONCLUSION: Artificial bowel sphincter is a potential treatment modality for fecal incontinence in patients with congenital anorectal malformation. The main reason for failure results from inability to accurately assess the extent of the anorectal malformation before device implantation. Changes in the perineal region consequent to previous operations are the main limiting factors for artificial bowel sphincter implantation.Key words: anorectal malformation - incontinence - artificial bowel sphincter - long-term results.

[Hirschsprungs disease in adults two case reports and review of the literature]. / Hirschsprungova choroba v dospelém veku dve kazuistiky a prehled literatury.

Hirschsprungs disease (HD) in adults is extremely rare, only three publications in Czech and Czechoslovak journals making reference to the condition after childhood. We present two cases of adult patients with HD. The first case is a 46-year-old male patient suffering from chronic constipation since childhood and diagnosed with megacolon at the age of 16; however, no further detailed diagnosis was done. At the age of 41, he developed a sigmoid perforation due to fecaloma and underwent urgent rectosigmoid resection and colostomy. 5 months later, Swensons coloanal anastomosis with diverting ileostomy was performed. Postoperative course was uneventful. He has two bowel movements a day. 7 years after the Swensons procedure, he also underwent adhesiolysis for acute bowel obstruction. His daughter was operated on due to HD at 16 days of age. The second case is a 57-year-old male patient. He suffered from chronic constipation and megacolon since 2 years of age and was diagnosed with congenital megacolon at the age of 19. However, no detailed diagnostics followed. He had a long interval between stools of up to 14 days. He underwent colonoscopy and, with a diagnosis of resistant Crohns disease, was referred to a surgical department where he was diagnosed with HD. Left hemicolectomy was first performed, followed by Swensons procedure with diverting ileostomy. All postoperative courses were uneventful. Currently he passes one or two soft stools a day. Adult HD is extremely rare. However, adult surgeons should consider it in case of refractory constipation since childhood associated with megacolon. Diagnosis is based on contrast radiography and rectal biopsy. Both Swensons and Duhamels procedures are suitable for surgical management. Left hemicolectomy with colonic rotation and coloanal anastomosis and/or proctocolectomy with J-pouch anastomosis are indicated in advanced forms of non-functional megacolon.Key words: adult Hirschsprungs disease - megacolon surgical therapy.

[Experimental tumor therapy using intratumoral injection of dendritic cells]. / Experimentální terapie nádoru pomocí intratumorózní aplikace dendritických bunek.

AIM OF STUDY: After the treatment of tumors, we often encounter a minimal residual sickness. However, the elimination of these leftover tumor cells is crucial for the patient. In the past years one of the most discussed options for this treatment is Imunotherapy, mainly by Dendritic cells. Dendritic cells are the most efficient cells out of the antigen presenting cell group. METHODS AND RESULTS: In the first part of the project, we perfected a technique of inducting a tumor on an experimental model. We inducted the tumor by the use of Carcinogenic substances or with the help of the Sarkom line imortalized fibroblasts. Another important part of the project was perfecting the method for the preparation of undeveloped dendritic cells from periphery blood monocytes. After these significant procedures were developed and perfected we moved onto the main part of the study. The Induction of a tumor by the carcenogenic substances Ethylennitrosamin and Phenobarbital was successful only in 20 % of the cases and therefore, was unusable for our experiment. We inducted the tumors with the Sarkom line method. After the application of dendritic cells into the tumor, a decrease in the development of the growth of the tumor was achieved. CONCLUSION: Imunotherapy using dendritic cells as a basis for treatment is a perspective method for treatment of tumors.

Dendritic cell-based immunotherapy induces transient clinical response in advanced rat fibrosarcoma - comparison with preventive anti-tumour vaccination.

In this study we present the models of preventive and therapeutic vaccination of sarcoma-bearing rats with dendritic cells that present tumour antigens from killed tumour cells. We present the characteristics of dendritic cell-based vaccine and its capacity to induce anti-tumour immune response both in vitro and in vivo. We show that preventive vaccination efficiently prevents tumour growth. On the other hand, vaccination of rats with established tumours did not lead to eradication of the tumours. Despite the induction of a vigorous immune response after administration of dendritic cell-based vaccine and transient decrease in tumour progression, tumours eventually resumed their growth and animals vaccinated with dendritic cells succumbed to cancer. In both settings, preventive and therapeutic, dendritic cell-based vaccination induced a vigorous tumour-specific T-cell response. These results argue for the timing of cancer immunotherapy to the stages of low tumour load. Immunotherapy initiated at the stage of minimal residual disease, after reduction of tumour load by other modalities, will have much better chance to offer a clinical benefit to cancer patients than the immunotherapy at the stage of metastatic disease.

Parameters of healing in approximative intestinal anastomosis.

INTRODUCTION: The restoration of bowel continuity using multiple classic anastomoses is mostly impossible in unstable critically ill extremely low birth weight neonates. The parameters of healing of approximative anastomoses in which integrity and continuity of bowel is achieved with limited number of stitches were evaluated in an experimental study. MATERIAL AND METHODS: Small bowel anastomoses were performed in twenty-two adult male rats. An approximative ileo-ileal anastomosis was performed with five seromuscular-interrupted sutures only; in the control group the anastomosis was performed with the conventional technique of interrupted sutures. The mechanical and biochemical parameters were compared. RESULTS: All anastomoses in both groups healed well without obstruction. The mean operating time needed for an approximative anastomosis was shorter (16 +/- 7.1 min versus 23.6 +/- 6.2 min, p = 0.016). The strength of the approximative anastomoses on the 1st day after surgery was 55 +/- 15 torr; the strength of the conventional anastomoses was 55 +/- 42 torr. The strength of the approximative anastomoses after 7 days was 249 +/- 39 torr; the strength of the conventional anastomoses was 218 +/- 23 torr (p = 0.118). The activity of the collagenolytic enzymes matrix metalloproteinase-2 and matrix metalloproteinase-9 in the anastomotic area was significantly increased compared with the activity in samples of non-operated bowel. There was no significant difference in collagenolytic activity between both types of anastomoses. CONCLUSION: The approximative anastomosis is a time-saving alternative to conventional anastomoses with a comparable course of anastomotic healing, anastomotic strength, and changes in collagen metabolism.

[Method of approximative intestinal anastomosis in experimental model]. / Metoda aproximacní strevní anastomózy v experimentálním modelu.

AIM: A technique of approximative anastomosis in witch integrity and continuity of bowel is achieved with limited number of interrupted seromuscular stitches was evaluated in experimental study. MATERIAL AND METHODS: Small bowel anastomosis were performed in twelve rats (Wistar, male) with weight range 197-242 g. An approximative anastomosis in the ileum of six rats was performed with five seromuscular-interrupted sutures only; in the second study group anastomosis was performed with conventional technique of interrupted sutures. The approximative anastomosis was evaluated concerning operating time, anastomotic healing, bursting pressure and adhesions in comparison to the conventional anastomosis. Statistics was calculated with Anova test. RESULTS: All anastomosis in both group healed well without obstruction. The median operating time needed for approximative anastomosis was shorter (31.7 +/- 1.6 minutes versus 35.2 +/- 1.5 minutes, p = 0.002). The strength of approximative anastomosis after 7 days was 249 +/- 39 torr; strength of standard anastomosis was 218 +/- 23 torr (p = 0.118). There were no significant differences in the others evaluated parameters between two study groups. CONCLUSION: In the animal model presented, the approximative anastomosis shows time saving alternative to standard anastomosis, with the same parameters of anastomotic strength, healing, and adhesions.

[Approximative intestinal anastomosis in newborns with multifocal necrotizing enterocolitis]. / Aproximacní strevní anastomóza pri multifokální nekrotizující enterokolitide u novorozence.

Multifocal necrotizing enterocolitis (NEC) may result in extensive bowel necrosis and short bowel syndrome. Authors present case report of premature newborn (BW 1700 g, gestational age 30 w.) where an extensive multisegmental NEC of small and large intestine was found during first explorative laparotomy. Proximal jejunostomy 28 cm beyond ligament of Treitz was performed and the rest of involved intestine was left in situ. After 48 hours multiple small bowel resections were performed leaving 12 cm of small intestine (5 short segments) distal to the jejunostomy. Five approximative anastomoses were performed to restore continuity among these segments and ileocaecal valve. Each of approximative anastomosis was constructed with limited number of 4-6 interrupted stitches and all anastomoses healed without complication. Intestinal continuity between proximal jejunostomy and the reconstructed segment of ileum was reestablished nine weeks later. Total length of small bowel was 50 cm. The patient was discharged at the age of 5 months weighing 4145 g with supplemental pareneteral nutrition. The technique of rapid approximative anastomosis may contribute to save maximal intestinal length in cases with the risk of short bowel syndrome.

[Artificial sphincter in patients with congenital anorectal malformations]. / Umelý sverac u nemocných po kongenitálních malformacích anorekta.

Fecal incontinence represents one of consequences of congenital anorectal malformations (CAM) which continues in some patients even after surgeries performed due to malformation and reconstructions. Implantation of artificial bowel sphincter (ABS) is a new solution not proved in daily practice. The aim of the study is to prove the ABS implantation as a feasible solution even in young adults after CAM, which have never been continent, and to verify ABS implantation as a suitable solution. GROUP OF PATIENTS: 11 patients in the age between 18-25 y. were enrolled. 6 patients refused, 5 patients were operated on. 2 ABS implantations were performed. Due to perforation of rectal wall in 1 patient the implantation was postponed. In 2 patients preparative surgeries were performed. RESULTS: 2 patients with ABS implantation and 2 ones after prep surgeries healed primarily. Both ABS are fully functional. Special care is needed to adopt a new defecation habits. Patients with ABS registered increase of QOL score; all pts operated on are both of high motivation and satisfaction.

T-tube ileostomy for intestinal perforation in extremely low birth weight neonates.

To evaluate the results of use of T-tube ileostomy in selected cases of intestinal perforation in extremely low birth weight (ELBW) neonates. The records of 288 ELBW neonates treated at author's institution, from 1998 to 2003 were retrospectively reviewed to identify neonates operated for intestinal perforation with T-tube placement. T-tube was inserted into the bowel through the site of perforation or proximally to the perforated gut via separate stab incision. T-tubes were used in five ELBW neonates (BW 600-900 g, gestational age 25-27 weeks) with intestinal perforation, in four of them at the time of primary surgery and in one neonate 8 days after primary anastomosis. All patients survived and there were no serious complications related to the T-tube insertion. Median duration of T-tube placement was 4 weeks (range 3-8 weeks), full enteral feeding after T-tube insertion was achieved in 4 weeks (range 1-6 weeks). All sites of T-tube insertion closed spontaneously. T-tube ileostomy is an effective and safe technique for treatment of selected cases of intestinal perforation in ELBW neonates. With respect to the hypoperistalsis of immature bowel, we recommend the use of T-tube in all cases of isolated intestinal perforation in ELWB neonates.

[Thyroid carcinomas and Hirschsprung's disease--10-year experience with molecular genetic testing of the RET proto-oncogene]. / Nádory stítné zlázy a Hirschsprungova choroba: desetileté zkusenosti s molekulárne genetickou diagnostikou RET proto-onkogenu.

In the last ten years, research has confirmed the role of the RET proto-oncogene in the pathogenesis of thyroid cancer such as medullary thyroid carcinoma (MTC) and papillary thyroid carcinoma (PTC), multiple endocrine neoplasia type 2 (MEN 2) and Hirschsprung's disease that can be associated with MTC or MEN 2. Through the use of molecular genetic testing, we are able to detect gene mutations and the course the disease might take can be predicted, thus enabling us to cure mutation carriers among the high-risk patients can at a very early, clinically asymptomatic stage of the disease; prophylactic total thyreoidectomy in said patients is recommended. At this juncture, there is extensive on-going research on the physiological role played by the RET proto-oncogene on the normal proliferation, differentiation and survival of the cell. Thanks to the new findings there are now possibilities of the theurapeutic use of gene therapy on an RET signaling cascade level in near future.

Acute gastro-intestinal obstruction as a late presentation of congenital diaphragmatic hernia. A report of three cases.

Three cases of acute gastro-intestinal obstruction due to incarceration of congenital diaphragmatic hernia (Bochdalek hernia) in infants are reported. The level of incarceration was stomach, small and large intestine. All of the presented posterolateral diaphragmatic defects were small and without a sac. History of trauma was absent in all patients. Two girls recovered well while a boy died of intracerebral bleeding one month after surgery. Acute gastro-intestinal obstruction as a late, post neonatal, presentation of congenital diaphragmatic hernia is a rare, life-threatening emergency. The combination of gastro-intestinal obstruction, circulatory and respiratory distress requires urgent gastro-intestinal decompression, fluid resuscitation and ventilatory support. Surgery can be performed safely only after pre-operative stabilization.

[Morgagni diaphragmatic hernia in childhood]. / Morgagniho bránicní kýly v detském veku.

The aim of this work was to retrospectively assess clinical and anatomical features of children with Morgagni congenital diaphragmatic hernias. The study group included 5 boys and 3 girls, aged between 5 months and 13 years, at the time of the diagnosis. Morgagni diaphragmatic hernia was diagnosed with non-acute symptoms in 8 children. A retrosternal defect of the diaphragm was left-sided in 7 subjects and bilateral in 1 boy. A hernial sac was found in 6 children. In 3 subjects, the hernial sac contained the liver, in 2 subjects the omentum, in 2 subjects the transverse colon and small intestinal loops and in one child it contained the transverse colon with the omentum. In all cases, the diaphragmatic defect was closed using primary plasty. All subjects healed without complications. A differential diagnosis of congenital Morgagni hernia must be considered in children with unusual respiratory and gastrointestinal symptoms with abnormal x-ray findings on chest examination. A stricture of the congenital Morgagni diaphragmatic hernia is rare, and was not recorded in our group.

[Rates and histological characteristics of rectal polyps in childhood]. / Výskyt a histologická charakteristika rektálních polypu v detském veku.

AIM OF STUDY: To determine a single centre incidence, rate of early and late complications and sequelae and histological characteristics of solitary rectosigmoideal polyps in children. METHODS: Retrospective analysis of hospital charts and histological characteristics of patients operated for rectosigmoideal polyps between January 1995 and December 2005. There were 23 children operated on, the study group consists of 20 patients (3 patients were excluded because of insufficient documentation). Demographics, symptomatology, season of first manifestation, localization, histology, postoperative complications and recurrence rate were evaluated. RESULTS: There were 7 boys and 13 girls in the study group (1:1.9), age at manifestation was 2-17 years (average 5.6 y.). The presenting symptom was bleeding in 14 pts, anal prolaps once and combination of both symptoms 5 times. The polyps were localized 3-15 cm from anocutaneous border, on the posterior intestinal wall. Histological examination showed a juvenile polyp in 15 (75%) patients, atypical juvenile polyp and inflammatory polyp in 2 patients each and lymphoid polyp in one case. One patient suffered from a mild bleeding postoperatively. No recurrence was noted. CONCLUSION: Polyps in children are rare, surgical removal of polyps is a relatively easy operation with minimum complications. Long term follow-up is not necessary. The seasonal occurrence of first symptoms predominantly in autumn and winter months (19 pts) has not been to our knowledge previously published.

[Treatment of congenital diaphragmatic hernia in neonates]. / Lécba vrozené bránicní kýly u novorozencu.

BACKGROUND: Treatment of the Congenital Diaphragmatic Hernia in neonates with early manifestations has been accompanied with significant mortality. Authors present the treatment protocol employed in such children and results from the period January 1994 to December 2003. METHODS AND RESULTS: Since January 1994 to December 2003 96 children were cured for the Congenital Diaphragmatic Hernia. Ratio F:M was 36:60. 29 children (30%) were diagnosed prenatally, the remaining newborns were diagnosed on the basis of thoracic X-ray, which was required for the early signs of respiratory insufficiency. The postnatal treatment protocol included circulation-ventilation stabilisation, operation, early extubation and initiation of the peroral nourishment. Altogether 78 (81%) of children were operated after the stabilization using the standard laparotomy method, 5 children (6%) were operated with extra corporal membrane oxygenation, in 20 children (26%) a Goretex patch was used to cover an extensi e defect in the diaphragm. Overall survival was 74%, survival of operated children was 91%, 75% of children with a patch survived, survival of children operated with ECMO was 40%. Relapse occurred in one child (1%) with a patch. CONCLUSIONS: Specification of the prenatal diagnosis and collection of patients with CDH into tertiary centres with ECMO possibility can improve prognosis of neonates with the malformation.

Alimentary tract duplications in children: high incidence of associated anomalies.

BACKGROUND: Alimentary tract duplications (ATDs) are rare congenital anomalies affecting about 1 in 4500 newborns with a male/female ratio of 1.2 : 1. The presence of associated, sometimes complex congenital anomalies can pose a challenge to surgeons treating patients with ATDs. The aim of this work was to study the characteristics of ATDs and associated anomalies in our patients. METHODS: Retrospective study. Medical charts of 24 patients (7 males, 17 females) diagnosed with ATD during the years 1990 - 2001 at the Department of Paediatric Surgery, Motol Children's Hospital in Prague, Czech Republic were reviewed. RESULTS: Sixteen patients (67 %) were diagnosed at ages younger than 3 years. Eight cases were foregut duplications, 9 were midgut, and 7 hindgut anomalies. In 13 patients (54 %) associated malformations were documented. Eight patients (33 %) developed postoperative complications, 1 patient died. Complications were more frequent in the group of patients with associated anomalies (53 % vs. 9 %). CONCLUSIONS: Associated congenital malformations are frequently present in patients with ATDs. The complexity of the surgical repair of these anomalies may contribute to higher morbidity and mortality rates. A thorough examination to identify and characterise the presence of ATDs and associated congenital defects would provide appropriate diagnostic and surgical approaches for these patients.

Evidence of secondary neuronal intestinal dysplasia in a rat model of chronic intestinal obstruction.

The etiology of neuronal intestinal dysplasia remains largely unknown. There is, however, supporting evidence of the existence of Hirschprung's disease or chronic intestinal obstruction associated with neuronal intestinal dysplasia. With the aim of investigating the possible development of neuronal intestinal dysplasia linked to chronic intestinal obstruction, we have examined the enteric nervous system response to long-term obstruction in a rat model. Three different surgical techniques were tested in Wistar male rats. In animals that survived longer than the cutoff chronic intestinal obstruction point (6 weeks), full-thickness biopsies and acetylcholinesterase (AChE), NADH, hematoxylin-eosin, and anti-S100 protein stainings were performed. The results of our model indicate that chronic intestinal obstruction induced different degrees of enteric nervous system dysplasia, including histological features of neuronal intestinal dysplasia. The relationship between chronic intestinal obstruction and anomalies of the enteric nervous system, including neuronal intestinal dysplasia, needs to be further studied.

[Treatment of the Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine, from 1979 to 2004]. / Lécba Hirschsprungovy choroby na Klinice detské chirurgie 2. LF UK a FN Motol v letech 1979-2004.

BACKGROUND: Survey of surgical techniques, strategy and results of the treatment of patients with Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine in Prague from 1979 to 2004 is presented. METHODS AND RESULTS: Paper summarises a twenty-five years long retrospective study of medical records of 274 patients who were operated on 1979-2004. 173 (67,3 %) pts had classical rectosigmoid (CRA) and/or long colonic aganglionosis (LCA). 19 (6,4 %) pts had total colonic aganlionosis with small bowel involvement (TCA). 72 (26,3 %) pts suffered from ultra-short rectal aganglionosis (URA). 74 pts with CRA and LCA operated on 1979-1991 underwent Kasai's colorectoplasty. 8 (10,8 %) of them had anastomotic leak, 7 (9,5 %) pts had anastomotic stricture, 2 (2,7 %) pts had postoperative enterocolitis (PEC) and 10 (13,5 %) had chronic constipation. In 6 pts an additional partial sphincteromyectomy of the internal anal sphincter SFME) had to be done. In period 1991-2004 93 pts underwent Swenson's procedure with (SFME). Anastomotic leak occurred in 3 (3,2 %) pts. Anastomotic stricture, PEC and/or chronic constipation were not registered. In 6 pts with anastomotic leak from both groups Soave re-do pull-through was done. In 2003-2004, 10 pts with CRA underwent a transanal resection of aganglionic segment with Swenson's colorectoplasty. One pt developed anal stricture. 3 of 19 pts with TCA operated on in 1979-1990 underwent classic Martin's long side to side ileo-recto-colic anastomosis. In 16 pts of 1991-2004 only short ileo-recto anastomosis according to Kasai and/or Swenson were done. In 93 pts suffering from URA a Lynn's SFME was performed. Three-stage procedure for the last time was used in 3 pts in 1984. Two- and one-stage procedures are still used. CONCLUSIONS: A number of postoperative complications decreased, a three-stage procedure was abandonned and the operation of HD is currently performed in newborn. In CRA a TAR replaced the laparatomy and in TCA the short ileo-rectal anastomosis is performed only.