Dariusz Patkowski

Laparoscopic splenectomy in children–a multicenter experience

PURPOSE Splenectomy is frequently required in children for various hematologic pathologic findings. Because of progress in minimally invasive techniques, laparoscopic splenectomy (LS) has become feasible. The objective of this report is to present a multicenter experience of 159 laparoscopic splenectomies. METHODS Records of 159 children, who underwent LS in 3 pediatric surgery centers between 1996 and 2006, were reviewed retrospectively with a special questionnaire. The indication for splenectomy were hematologic disorders (147), esophageal varices (6), splenic cyst (5), and tumor of the tail of the pancreas (1). The LS was performed using standard laparoscopic technique and instrumentation. RESULTS There were 75 boys and 84 girls. Median age was 12.5 years (range, 2-19.4 years). Laparoscopic splenectomy alone was performed in 118 cases and LS with cholecystectomy or cholecystotomy in 36. In 5 cases, LS was performed together with fundoplication. Eight LS required conversion to an open procedure for following reasons: severe bleeding (3), massive splenomegaly (1), anatomical (2), and technical aspects (2). Accessory spleens were identified in 20 cases (12.6%). There was no mortality. Complications consisted of 8 conversions and postoperatively of mild generalized infection in 3 children and abdominal bleeding that resulted in re-laparoscopy in 1 case. CONCLUSIONS Laparoscopic splenectomy can be safely and effectively performed in children.

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)

Experiences with recombinant activated factor VII in the treatment of severe refractory thrombocytopenia.

Approximately 5% of chronic immune thrombocytopenic purpura (ITP) manifests itself as symptomatic, severe thrombocytopenia requiring splenectomy. The surgical procedure increases the risk of serious hemorrhage, especially in patients refractory to platelet transfusions. Recombinant factor VIIa (rFVIIa) has been found to enhance thrombin generation on activated platelets and may be a promising agent in preventing life‐threatening bleedings. The administration of rFVIIa in two patients with severe refractory ITP, who underwent splenectomy, is presented. Combined therapy with agents of different mechanisms of action could be useful in cases with the highest probability of bleeding. Pediatr Blood Cancer 2006;47:729–730.

Bladder agenesis in a male neonate

Bladder agenesis, especially in male, is a rare congenital anomaly. This is a case report of a male patient with several congenital anomalies including penoscrotal transposition, severe kidneys dysplasia, and agenesis of the bladder. The patient lived for 4 months and died because of severe pneumonia.

Assessment of Clinical Symptoms and Multichannel Intraluminal Impedance and pH Monitoring in Children After Thoracoscopic Repair of Esophageal Atresia and Distal Tracheoesophageal Fistula

BACKGROUND Motility disturbances of the esophagus and gastroesophageal reflux disease (GERD) are observed frequently in children after repair of congenital esophageal atresia with distal tracheoesophageal fistula (EA/TEF). Recently, in many pediatric surgical centers, thoracoscopic repair was introduced, which theoretically would change the postoperative course. OBJECTIVES The goal of the study was to assess physical development, disease symptoms, and GERD symptom frequency in children who underwent thoracoscopic surgery of congenital EA/TEF. MATERIAL AND METHODS The study comprised 22 children (14 boys and 8 girls), aged 16 to 79 months (average age 47.3 months) after surgery of EA/TEF. Clinical symptoms and physical development were analyzed. In 19 children, multichannel intraluminal esophageal impedance connected with pH-metry (MII/pH) was performed. In 11 patients, esophagogastroduodenoscopy with a histological study of mucosa samples was done. RESULTS The most frequent symptoms were dysphagia, belching, cough and recurrent bronchitis. In 31.5% of the children, body mass deficiency was observed and in 28.6%, low body mass and short stature. Prematurity was present in half of the patients. Depending on the result of the MII/pH study, the children were divided into two groups: 10 children with GERD and 9 children without diagnosis of gastroesophageal reflux. In the 10 with GERD, acid reflux was diagnosed in 9 and non-acid reflux was diagnosed in one. MII/pH demonstrated statistically significant differences in the number of reflux episodes, reflux index, bolus exposure index, mean time of esophageal exposure and acid exposure and mean acid clearance time in children with GERD in comparison to children without this disease. In 36.4% of children who underwent endoscopy, esophagitis was diagnosed, esophageal stricture in 18% and gastric metaplasia in 9.1%. CONCLUSIONS In children who underwent corrective thoracoscopic surgery of EA/TEF, GERD caused by motility disorders of the esophagus was frequently observed. These children require constant monitoring and early treatment of the complication.

Single nucleotide polymorphisms in the RET gene and their correlations with Hirschsprung disease phenotype

Hirschsprung disease (HSCR) is a congenital, heterogeneous disorder, characterized by the absence of intestinal ganglion cells. Recent advances show that theRET gene is a major locus involved in the pathogenesis of HSCR. The aim of this study was to analyse if the HSCR phenotype in the Polish population is associated with the presence of polymorphisms in exons 2, 3, 7, 11, 13, 14 and 15 of theRET gene. Molecular results were compared with clinical and long-term follow-up data in 70 Polish patients with HSCR (84.3% with a short segment and 15.7% with a long segment of aganglionic gut). Single-nucleotide polymorphisms were analysed by using the minisequencing SNaPshot multiplex method. The 135G>A polymorphism inRET exon 2 was overrepresented in HSCR patients, compared with a healthy control group. Moreover, the 135G>A variant was shown to be associated with the severe HSCR phenotype. Two other polymorphisms, 2071G>A in exon 11 and 2712C>G in exon 15, were underrepresented in the patients. The results confirm that theseRET polymorphisms play a role in the aetiology of HSCR.

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.

Electrocoagulation versus clips in laparoscopic varicocelectomy in boys

INTRODUCTION Varicocele occurs in about 15% of adolescents, but it is hardly ever noticed in children under 10 years old. Surgical treatment in adolescents is still controversial. OBJECTIVES The aim of this work was to assess the outcome of laparoscopic varicoelectomy by using electrocoagulation versus clips. MATERIALS AND METHODS In the Pediatric Surgery and Urology Departments of the Medical University of Wroclaw, 139 boys with varicocele were operated on by using laparoscopy between 1995 and 2007. In total, 68 (49%) patients appeared for the control ultrasound after the surgery, and only those patients were included in this study. The spermatic vessels (both the artery and the vein) were trans-sected by monopolar diatermy (38 patients; 55.8%) or clips (29 patients; 42%). The spermatic Artery was saved in 5 cases, which have been operated on by using diatermy and in 3 by using clips. The follow-up was from 0.5 to 7 years (mean, 2.7 years). RESULTS Among 68 patients from the control group of persistent varicocele with grade II and grade III (Dubin-Amelaar classification) were recognized in 5 (7.3%) boys. In the group of boys, who have been operated on by using diatermy, persistent varicocele was noticed in 10.5% (4), while in those who have been operated on by using clips, the persistent varicocele was recognized only in 3.4% (1). Varicocele with grade I, not demanding a second operation, was recognized in 9 boys (14.7%). The most often found complication was hydrocele. It was recognized in 23.7% (9) boys operated on by using diatermy and in 13.8% (4) boys operated on by using clips. CONCLUSIONS Varicocelectomy using clips gives much less complications then the electrocoagulation and should be firstly chosen.

Pyelonephritis xanthogranulomatosa in a 7-year-old girl.

Xanthogranulomatous pyelonephritis (XGPN) is rare form of chronic, usually unilateral, renal infection that involves damage to the renal glomeruli and periglomerular tissue and destruction of the renal parenchyma. Nephrectomy is usually necessary and the prognosis is good if XGPN is unilateral and treated early. XGPN typically affects middle-aged females and is extremely rare in children. We report one case of XGPN in a seven-year-old girl who was operated on at our institution be-cause of lower urinary tract dysfunction and renal insufficiency – the left kidney was removed and the bladder was augmented by means of the remaining ureter. The histopathological evaluation of the removed kidney showed chronic XGPN.

Pielęgnacja noworodka z zarośnięciem przełyku

Nursing care of a newborn with Congenital Esophageal Atresia is extremely demanding, and its quality is important in the recovery process of the baby. Appropriate pre and postoperative treatment can help to improve the outcome of the surgery. This is the stage when the child is most susceptible to all sorts of complications and problems. Nursing requires assiduous attention, vigilance and careful analysis of clinical parameters of the newborn. Understanding the nature and significance of the defect and proper care can remarkably improve the outcome of the treatment. The aim of the study is to describe the standards of nursing care of a newborn with Congenital Esophageal Atresia before and after surgery. Nursing Topics 2015; 23 (2): 251–258