N. Danişmend

Esophageal replacement using the colon : a 15-year review

Abstract Colonic interposition is a treatment option in childhood when esophageal replacement (ER) is necessary. We reviewed 18 children who underwent ER by colon between 1984 and 1999. There were 5 with esophageal atresia and 13 with corrosive esophagitis; 15 had long-term follow-up (mean 38 months). Three procedures were performed by the Waterston technique and 12 by the retrosternal technique. ER was completed in a single stage in all but 1 patient. Pyloroplasty or antireflux surgery were not done routinely during colonic interposition. As early complications, we observed 11 cervical leaks and 2 pulmonary problems. As late complications, there were 4 redundancies, 3 gastrocolic refluxes, 2 cervical anastomotic stenoses, and 1 each intestinal obstruction due to adhesions, cologastric stricture, cosmetic deformity of the thorax, and bulging of the neck. Six patients with complications required secondary surgery. There were 4 deaths, 2 of them unrelated to the surgery. Cervical leakage, which was the most commonly observed problem, healed well. We believe the colon is still one of the best substitutes for the esophagus and that there is no need to perform a routine pyloroplasty or antireflux procedure as an adjunct to the primary surgery.

Quo vadis? Ureteric reimplantation or ignoring reflux during augmentation cystoplasty.

To decide whether antireflux surgery should be used in the presence of vesico‐ureteric reflux (VUR) in children, in whom an augmentation procedure is needed, because secondary VUR in children with a neurogenic bladder, infravesical obstruction and primary VUR in the exstrophy‐epispadias complex is expected to resolve after augmentation, which decreases the intravesical pressure and increases capacity.

Extensive intestinal aganglionosis

Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprungs disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprungs disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.

Effects of male sex hormones on urodynamics in childhood : intersex patients are a natural model

Abstract The effects of sex hormones on bladder function have been evaluated in adult females, especially in regard to postmenopausal incontinence and bladder irritability syndromes. These have not been investigated in children in regard to urodynamic findings. An intersex patient whose bladder is under the influence of androgens is a natural model to investigate the effects of male sex hormones on bladder function in females. To evaluate the urodynamic findings and clinical symptoms in a group of intersex patients and to determine how androgens influence bladder function in female children, clinical and urodynamic records of 12 intersex patients with adrenogenital syndrome were investigated retrospectively. The mean age was 9 ± 5.7 years (1.5–18) and the mean follow-up period was 5.1 ± 4.4 years (1–12). Congenital adrenal hyperplasia (CAH) was present in all cases. Only 3 patients had urinary symptoms and incontinence, but these findings did not correlate with their urodynamic findings. None of the patients required medications for their urinary symptoms. Nine are still being treated medically by the pediatric endocrine team with hydrocortisone for CAH. The upper urinary tract was found to be normal with no hydronephrosis. The mean bladder capacity (269 ± 122 ml) was lower (86.7%) than the estimated capacity for age. The mean compliance was 20 ± 13.7 ml/cmH2O. No unstable detrusor contractions were encountered. The most remarkable finding was this reduced bladder capacity of androgenized female patients for age, which shows the antagonistic effect of androgens on bladder urodynamics in females.

The roles of extracellular matrix proteins, apoptosis and c-kit positive cells in the pathogenesis of ureteropelvic junction obstruction

AIM To investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective. PATIENTS AND METHODS Medical records of patients with UPJO were reviewed and pathological specimens collected. Nephrectomy materials from forensic autopsies were taken as controls. Specimens were assessed with light microscopy. Fibronectin, type 4 collagen, laminin, Bax and Bcl-2 expression for apoptosis, together with interstitial cells of Cajal determination with c-kit were determined immunohistochemically. Staining scores were evaluated semiquantitatively. Results were evaluated using Mann-Whitney U-test. RESULTS Control group comprised 14 children (median age, 3.5 years; 6 months-17 years). Study group comprised 22 children with UPJO (median age, 9 months; 1 month-10 years). Light microscopy revealed non-specific inflammation, epithelial proliferation and atrophy with fibrosis in the smooth muscle of the UPJ in all patients. Fibronectin, type 4 collagen and laminin were found to be significantly increased in UPJO at the intrafascicular space of smooth muscle and the matrix of stroma. Bcl-2 expression was increased in UPJO. c-Kit was unable to stain interstitial cells of Cajal, but staining for mast cells was significant. CONCLUSIONS High expression of fibronectin, laminin and type 4 collagen may indicate a relation to the pathogenesis of UPJO. Defective kidney morphogenesis, during branching and tubulogenesis of ureteric bud, may be responsible for this congenital pathology.

Emotional effects of hypospadias surgery on Turkish boys

PURPOSE The aim of this study is to investigate in Turkish boys the emotional effects of hypospadias repair before and after the optimal age for repair. MATERIALS AND METHODS Forty hypospadias patients treated in our department were included in this study. Group 1 consisted of 13 patients operated on at less than 30 months of age. Group 2 consisted of 27 patients operated on at over 30 months of age. Age at time of study, severity of hypospadias, age at repair and number of operations were evaluated for both groups retrospectively. Socio-economic and socio-demographic characteristics of these patients including place of residence, educational level of their parents and parental occupations were determined. The Turkish versions of the Childrens Depression Inventory and the State-Trait Anxiety Inventory for Children A-State scale were used to evaluate the emotional effects of hypospadias surgery. For statistical analysis, the Mann-Whitney U test, Students t test and Chi-squared test were used. RESULTS There was a significant difference in the age at repair between Groups 1 and 2. No significant differences existed in severity of hypospadias and number of operations between the two groups. All 40 patients had the same socio-economic and socio-demographic characteristics. Group 1 did not significantly differ from Group 2 in regard to the educational level of their parents and parental occupations. Age at repair, which was the only difference between the two groups, did not affect the level of depression and anxiety-state symptoms. CONCLUSIONS There is a close relationship between emotional effect of hypospadias surgery and age at repair. Possibility of depression and anxiety, which were dominating emotional symptoms among boys operated on for hypospadias, was found not to be high after the recommended age. It is concluded that, as hypospadias surgery creates a circumcised penis, the degree of emotional trauma is lower in Turkish boys than in other nationalities.

Experience with Laparoscopy in Nonpalpable Testis

Aim: The aim of this study was to investigate the feasibility and benefits of diagnosis and interventional laparoscopy in those paediatric patients with nonpalpable testes (NPT). Patients and Method: Between 1992 and 1999, 75 patients with NPT (mean age 3 years, range 6 months to 14 years) were treated. 86 testes were evaluated. Results: According to the laparoscopic findings, 4 groups of testes were identified: Vanishing testis (n = 32), low abdominal testis (!2 cm to the internal ring) (n = 26), high abdominal testis (12 cm to the internal ring) (n = 24) group and intersex patients (n = 4). Of the first group, 19 testes (one bilateral) had blind ending spermatic cord and vessels and if an atrophic testicular tissue was identified, it was removed laparoscopically. For those with spermatic cord and vessels beyond the internal ring (13 testes), atrophic testes were removed through a high scrotal incision. 19 testes of the second group had a laparoscopy-assisted orchiopexy. In the same group a laparoscopic orchiopexy was performed on 7 testes. 24 testes in the 3rd group had a Fowler Stephens (FS) stage I and 18 testes had a laparotomy performed for FS stage II procedure (laparotomy and orchiopexy) after 6 months. At laparotomy there was no evidence of testicular atrophy in all but one testis which was removed and the FS stage II procedure was completed in 17 testes. The follow-up period is between 6 months and 4 years, and two more testicular atrophies were noted after FS stage II. The results were satisfactory in 15 testes among 18 (83%). In the intersex group, the patient with testicular feminization had laparoscopic orchiectomy. The other patient with bilateral nonpalpable testis was identified to have a uterus and two intra-abdominally located gonads on laparoscopy and gonadal biopsies were obtained for diagnosis. Histology demonstrated bilateral ovotestes confirming the diagnosis of a true hermaphrodite. Conclusion: We think that laparoscopy decreases the number of laparotomies in NPT, allows a single stage procedure in low abdominal testis and facilitates clip ligation of the testicular artery in high abdominal testis. Also laparoscopy provides diagnostic and therapeutic options in vanishing testis and intersex patients.

Congenital lobar emphysema ― A report of 5 cases

Five infants with congenital lobar emphysema whose main symptoms included dyspnea, cyanosis and recurrent respiratory infections, are presented herein. The most reliable diagnostic tool was plain chest X-ray films with antero-posterior and lateral views, while radio-isotopic investigation of the lung perfusion state took second place in the diagnosis of this disease. The affected lobes were the left upper lobe in four patients and the right middle lobe in one. Lobectomy was performed with good results in 4 patients, however, 1 infant was lost following an emergency thoracotomy.

Total intestinal aganglionosis with involvement of the stomach

Total intestinal aganglionosis is the rarest form of Hirschsprungs disease, with absence of ganglia from the duodenum to the rectum. In addition to those cases reported in the literature, a neonate with total intestinal aganglionosis with involvement of the stomach is presented. Diagnosis of this extensive form of Hirschsprungs disease is a major problem. These patients lie beyond the scope of present surgical expertise, and the outcome is universally fatal.