Laparoscopic inguinal hernia repair in children via PIRS (percutaneous internal ring suturing).

INTRODUCTION: The miniinvasive approach is a trend in pediatric surgery nowadays. The new surgical technique called percutaneous internal ring suturing (PIRS) is a promising method bringing all the benefits of miniinvasive surgery. METHODS: Prospective study of patients operated on using the PIRS technique from 01 January 2018 to 01 January 2020 at the Department of Pediatric Surgery, 2nd Faculty of Medicine, Charles University, University Hospital Motol. RESULTS: 73 patients (25 boys and 48 girls) were operated on using PIRS. The median age was 68 months. 90 % of operations were performed by the same team of surgeons. During the procedure there were found 53 right-sided and 38 left-sided inguinal hernias. In 18 cases the hernia was bilateral, but only in 13 cases was this diagnosis made before the operation. A non-absorbable stitch was used in 57 cases to close the internal ring of the inguinal canal, and a non-absorbable monofilament in 16. The median operating time was 34 minutes. There were 3 recurrences (3.3 %) in our study.  Conclusion: In our initial study, the PIRS technique proved to be a safe alternative method to the open inguinal hernia surgery. This method provides the benefit of allowing to revise the contralateral inguinal canal as a prevention of a metachronous inguinal hernia. The cosmetic results were excellent.

Alternations in genes expression of pathway signaling in esophageal tissue with atresia: results of expression microarray profiling.

Esophageal atresia (EA) is a congenital defect of the esophagus involving the interruption of the esophagus with or without connection to the trachea (tracheoesophageal fistula [TEF]). EA/TEF may occur as an isolated anomaly, may be part of a complex of congenital defects (syndromic), or may develop within the context of a known syndrome or association. The molecular mechanisms underlying the development of EA are poorly understood. It is supposed that a combination of multigenic factors and epigenetic modification of genes play a role in its etiology. The aim of our work was to assess the human gene expression microarray study in esophageal tissue samples. Total RNA was extracted from 26 lower pouches of esophageal tissue collected during thoracoscopic EA repair in neonates with the isolated (IEA) and the syndromic form (SEA). We identified 787 downregulated and 841 upregulated transcripts between SEA and controls, and about 817 downregulated and 765 upregulated probes between IEA and controls. Fifty percent of these genes showed differential expression specific for either IEA or SEA. Functional pathway analysis revealed substantial enrichment for Wnt and Sonic hedgehog, as well as cytokine and chemokine signaling pathways. Moreover, we performed reverse transcription polymerase chain reaction study in a group of SHH and Wnt pathways genes with differential expression in microarray profiling to confirm the microarray expression results. We verified the altered expression in SFRP2 gene from the Wnt pathway as well as SHH, GLI1, GLI2, and GLI3 from the Sonic hedgehog pathway. The results suggest an important role of these pathways and genes for EA/TEF etiology.

Normal exon copy number of the GLI2 and GLI3 genes in patients with esophageal atresia.

Esophageal atresia (EA) is a congenital developmental defect of the alimentary tract concerning the interruption of the esophagus with or without connection to the trachea. The incidence of EA is 1 in 3000-3500 of live-born infants, and occurs in both isolated and syndromic (in combination with abnormalities in other organ systems) forms. The molecular mechanisms underlying the development of EA are poorly understood. Knockout studies in mice indicate that genes like Sonic hedgehog, Gli2, and Gli3 play a role in the etiology of EA. These facts led us to hypothesize that Sonic hedgehog-GLI gene rearrangements are associated with EA in humans. To test this hypothesis, we screened patients with isolated and syndromic EA for GLI2 and/or GLI3 microrearrangements using methods to estimate the copy number (Multiplex Ligation-dependent Probe Amplification, real-time polymerase chain reaction). To our best knowledge this is the first study assessing copy number of GLI2 and GLI3 genes in patients with EA.

Severe clinical course of Hirschsprung disease in a Mowat-Wilson syndrome patient.

We present a clinical case of a female infant with multiple anomalies and distinctive facial features, with an exceptionally severe clinical course of Hirschsprung disease. The girl was also diagnosed with Mowat-Wilson syndrome, confirmed by molecular analysis as a heterozygous deletion of the ZEB2 gene. Moreover, molecular karyotyping revealed a deletion involving further genes (KYNU, ARHGAP15, and GTDC1).

Active enlargement of the abdominal cavity--a new method for earlier closure of giant omphalocele and gastroschisis.

The authors present their own modification of surgical staged repair of large abdominal wall defects (AWD). 21 newborns with gastroschisis (GSCH) and 8 with omphalocele (OC) are presented. In all patients the protruding viscera were placed in a prosthetic sac with a semi-permeable lining. The sac was suspended using a system with active external traction with a force amounting to 30 - 40 % of the baby's weight. All babies tolerated the applied method well. A progressive stretching of the abdominal wall and enlargement of the abdominal cavity enabled the defect to be closed between the second and sixth postoperative day. In comparison to the classic method, the presented modification of staged repair of AWD makes an earlier reduction of the viscera into the abdomen possible and does not seem to be associated with a higher risk of mechanical or infectious complications.

Uroflowmetry in children--is it necessary to repeat the study in a single patient?

UNLABELLED: Uroflowmetry, as the only non-invasive urodynamic means of assessing the function of the lower urinary tract is widely used in children. A possible psychological influence on the results is the reason why uroflowmetry is often repeated in the same patient. OBJECTIVES: The investigation was conducted to compare parameters of repeated uroflowmetry in a single child with the results of single uroflowmetry performed in a group of children. The goal of the study was to evaluate the efficacy of a single uroflow procedure in children. MATERIAL AND METHODS: A single uroflowmetric study was performed in 44 children aged from 9 - 11 years (mean 10 yrs). Out of the group one child was chosen who had 32 successive uroflow attempts. The range and average values of the uroflowmetric parameters were compared in both groups. Pearson's correlation coefficient was used for calculation and for a comparison of the uroflowmetric parameters of both groups (significance level p < 0.05). RESULTS: Average values of the voided volume, maximal and average flow were lower in the 32 attempts of a single child than in the control group (44 attempts). The values of the Pearson's correlation coefficient were ranged from 0.2 to 0.4 for flow/voided volume ratio and from 0.4 to 0.8 for flow time/voided volume ratio in both groups. There were no differences between the values in both groups apart from flow time/voided volume correlation (significance level p < 0.05). CONCLUSION: Repetition of uroflowmetric study does not seem to improve the efficacy of the modality in assessing voiding function in children.

[The value of the ultrasonography and computed tomography in diagnosis and monitoring of treatment of renal abscesses in children]. / Wartosc ultrasonografii i tomografii komputerowej w rozpoznawaniu i monitorowaniu leczenia ropni nerek u dzieci.

A retrospective study of 14 patients from 2 to 13 years old with renal abscess was performed. We evaluated the diagnostic value of ultrasonography (US) and computed tomography (CT) in diagnostic investigation and treatment of renal abscess, which have changed during the past ten years from classic surgical treatment to percutaneous drainage. US and CT have revealed the diagnosis by showing a hypoechogenic or hypodense mass in all 14 patients. All had an intensive course of antibiotic treatment. In 10 of them the abscess were drained percutaneously by US guided drainage. Percutaneous drainage was unsuccessful in 2 patients, who subsequently underwent classic surgical exploration, incision and drainage for uncontrolled infection. We conclude that US and CT greatly facilitate the diagnosis and permit the percutaneous drainage of renal abscess in paediatric age group, which benefits from this minimally invasive therapy.

[Hydronephrosis in the course of primary psoas abscess]. / Wodonercze w przebiegu pierwontego ropnia miesnia biodrowo-ledzwiowego.

Primary psoas abscess is rare and presents a difficult diagnostic challenge. We present an unusual case of primary psoas abscess causing hydronephrosis in a 11-year-old boy. The presence of hydronephrosis complicated diagnosis, that was definitely confirmed by computerized tomography. Percutaneous drainage of the abscess under ultrasonography guidance combined with antibiotic therapy provided an effective treatment. Culture of the pus showed Staphylococcus aureus. The subject is discussed noting incidence, etiology and methods of treatment of primary and secondary psoas abscesses.

Division of the genitofemoral nerve in unilateral cryptorchid rats.

The long-term effect of dividing the genitofemoral nerve supply to the undescended testis in naturally cryptorchid rats is reported. 20 male Buffalo rats with an undescended right testis, noted at the age of 30 days, were used for the investigation. 20 healthy animals served as controls. The abdominal division of the right genitofemoral nerve was performed in 10 cryptorchid and 10 healthy animals at the age of 40 days. The animals were mated at 150 days of age. Both testes were removed at 180 days of age. In each excised testis the testicular weight and seminiferous tubular diameters were measured, and the maturity of the germinal epithelium was determined using the Johnsen testiscular biopsy score. The experiment showed that in 70% of the nonoperated cryptorchid rats, both testes were affected at the age of 180 days, and 90% of these animals were infertile. In the operated cryptorchid rats, the contralateral descended were not damaged, and 60% of these animals were fertile. The division of the genitofemoral nerve in healthy animals had no effect. The results suggest that neural transmissions might play a role in the damage to the descended testis in unilateral cryptorchidism. This observation requires further investigations.

The natural course of cryptorchidism in rats and the efficacy of orchidopexy or orchidectomy in its treatment before and after puberty.

Both clinical and experimental evidence suggest that fertility is impaired in unilateral cryptorchidism. To investigate the effect of the undescended testis on the contralateral descended gonad, a new experimental model based on natural cryptorchidism in rats was designed. Seventy male Buffalo rats with an undescended right testis noted at the age of 30 days were used. Fifty healthy animals served as a controls. The natural course of cryptorchidism was investigated at the ages of 30, 90, and 180 days. The effects of orchiopexy and orchiectomy performed in cryptorchid animals before and after puberty were evaluated at the age of 180 days. Both nonoperated and operated animals were mated at the age of 150 days in order to estimate their fertility. The animals were killed at 30, 90, and 180 days of life and the testes were removed. In each excised testis testicular weight and seminiferous tubular diameters were measured and the maturity of the germinal epithelium was determined using the Johnsen testicular biopsy score. The experiment demonstrated reduced testicular weight and seminiferous tubular diameters in undescended testis already at 30 days and arrest of spermatogenesis at the spermatocytes stage at 90 and 180 days. There was no significant difference between contralateral descended testes and controls at the age of 30 and 90 days, but at 180 days the degenerative changes were identical with those in the cryptorchid testes. Cryptorchid rats were completely infertile. Both orchiopexy and orchiectomy prevented the damage to the contralateral testis. A significant improvement in size and spermatogenesis was recorded in most cases of the surgically descended testes.(ABSTRACT TRUNCATED AT 250 WORDS)