Sibel Tiryaki

Single center experience in single-incision laparoscopic surgery in children in Turkey

PURPOSE Minimally invasive surgery has evolved into single-incision laparoscopic surgery (SILS) in the recent years. Few reports have addressed the practicality of SILS in children. Our current experience with regard to feasibility and effectiveness of SILS in children is presented. METHODS A retrospective review of the operative database for patients operated on using SILS in our department from March 2009 to July 2010 was performed. Data regarding the type of the procedure, age, sex, operative performance, hospital stay, and complications were collected. MAIN RESULTS Among 43 patients, cholecystectomy was performed in 11; appendectomy, in 10; unroofing for ovarian cysts, in 5; unroofing for splenic cysts, in 4; oophorectomy, in 6 (ovarian torsion, 2; teratoma, 4); ovary-preserving teratoma excision, in 1; splenectomy, in 1; gonadectomy, in 3; and varicocelectomy, in 2. There were no conversions to standard laparoscopic or open techniques. The only postoperative complication was a wound infection that occurred after an appendectomy. CONCLUSION Although currently more expensive, SILS can be performed in children in almost every pediatric surgical procedure that can be accomplished with conventional laparoscopic techniques. The most significant contribution of SILS procedure is cosmesis. Postoperative pain and length of hospital stay were not improved.

Botulinum injection is useless on fibrotic neuropathic bladders.

OBJECTIVE Studies on the use of intradetrusor botulinum toxin A injection for children with neuropathic bladders are insufficient and the results are controversial. The aim of the present study was to evaluate the effect of intradetrusor botulinum toxin A injection for children with neuropathic bladders that are resistant to anticholinergic treatment, and to reveal any criteria indicating treatment success. PATIENTS/METHODS Hospital records were reviewed of 16 children with neuropathic bladders due to myelomeningocele, and who had botulinum toxin A injections between 2007 and 2010. Botulinum toxin A (10 units/kg) was injected endoscopically into various sites of the detrusor, except the trigone. The success was defined as complete dryness between clean intermittent catheterizations. Urodynamic studies before and after the application were evaluated and parameters, including bladder capacity (measured/expected) and compliance, were also analyzed. Reviewing the results, patients were then classified into two groups: as having fibrotic bladders (noncompliant, acontractile bladders with high pressures) or overactive bladders. Urodynamic findings and therapy success were then compared between the groups. RESULTS A total of 19 injections, including repeat injections in three patients, were performed. Results of the 16 initial injections were evaluated. Nine patients had detrusor overactivity, and five out of nine (56%) applications in this group resulted in complete dryness between clean intermittent catheterizations. In bladders with typical detrusor overactivity, there was a significant increase in both the capacity (from 0.53 to 0.74) and compliance (from 4.7 to 8.6 ml/cm H2O). Looking at the seven patients that displayed fibrotic bladders with very low compliance and no contraction at all, none of them presented with notable clinical improvement from injections. Comparing the urodynamic findings, there was no significant difference in compliance (3.1 ml/cm H2O before and 3.5 ml/cm H2O after) and bladder capacity (0.58 before and 0.52 after the treatment) in the fibrotic bladders. DISCUSSION Despite its worldwide usage and FDA approval, studies on the effectiveness of botulinumtoxin A on neuropathic bladders in children are controversial. There are now numerous studies attesting to the good results of BoNTA in neuropathic detrusor overactivity; however, only scarce reports comment on the specific features of the disease process among patients and reasons for failure in some. In our study, reviewing the urodynamic findings carefully, it was observed that the patients who did not respond to injections were the ones with no contractions, despite high pressures and low compliance. Therefore, describing the indications of BoNTA as neuropathic detrusor overactivity and urinary incontinence despite anticholinergic medications may lead to mistreatment of patients in the decompensated phase of a hyper-reflexive detrusor. Pretreatment urodynamic evaluation might be a good indicator, without biopsies, of estimating the degree of fibrosis and the patients who will benefit from the injection. CONCLUSION Botulinum toxin A injection in the neuropathic bladder of myelomeningocele patients was found to be ineffective if the detrusor was fibrotic, of low compliance and had lost contractility. Urodynamic findings should be carefully analyzed in order to select appropriate patients that may benefit from Botulinum injection.

Involution of multicystic dysplastic kidney: Is it predictable?

OBJECTIVE To evaluate the clinical course of multicystic dysplastic kidney (MCDK) and to reveal any criteria indicating spontaneous involution. MATERIAL METHODS Hospital records of patients with MCDK followed in two different institutions in 1994-2009 were reviewed and data were analyzed regarding involution. RESULTS Records of 96 patients were reviewed, of whom 46 were diagnosed antenatally and followed for more than 1 year. Fourteen patients had undergone nephrectomy. There was one case of hypertension which resided with nephrectomy. There was no malignancy. Involution rate was 53.6% (15/28) for right-sided and only 16.7% (3/18) for left-sided kidneys. The initial size of the kidney was found to be another predictive parameter for involution. Initial sizes of 43 (15 involuted and 28 non-involuted) kidneys were documented. Mean standard deviation score for involuting and non-involuting kidneys was -3.19 and 3.12, respectively. The chance of involution for a large kidney on the left was zero; however, involution risk for a small right-sided kidney was 67%. CONCLUSION Reviewing a 15-year period of our patient records conveyed data supporting current literature mainly encouraging non-operative management of MCDK. Further studies are required; however, our two objective indicators, the initial size and side of dysplastic kidney, may contribute to the management.

Splenogonadal fusion-limb deformity syndrome: a rare but important cause of undescended testis.

BackgroundSplenogonadal fusion is a rare congenital anomaly which is characterized by fusion formation between the spleen and gonad.MethodsWe report a case of a 14-month boy with spleongonadal fusion-limb deformity syndrome focusing on the importance of awareness of this syndrome.ResultsThe patient was admitted to our clinic because of a left undescended testis, and preoperative diagnosis was not made. During the operation, “spleen-like” tissue attached to the gonad induced splenogonadal fusion, which was confirmed by laparoscopy. The patient also had a short right femur, hip dysplasia and a syndromic face.ConclusionSplenogonadal fusion anomaly should be considered in the evaluation of undescended testis, especially in patients with facial and limb deformities.

Unexpected outcome of a modification of Bracka repair for proximal hypospadias: High incidence of diverticula with flaps.

INTRODUCTION Various graft and flap techniques have been proposed for urethral reconstruction in proximal hypospadias repair. The Bracka repair involving the transfer of inner prepuce like a Wolfe graft mostly results in satisfactory results besides a high fistula rate. AIM The aim was to decrease the high fistula rate with Bracka repair; we wanted to use the advantages of vascularized skin in the Bracka method. The aim of this study was to evaluate our results with this modification. STUDY DESIGN Our modification involves using a flap instead of a graft. In the first stage, chordee was corrected by transection of the urethral plate and dorsal midline plication when necessary. Instead of a graft as suggested by Bracka, inner preputial skin with ample blood supply was transferred and stitched to the denuded ventral penile surface. In the second stage after 6 months, this flap was tubularized in the Thiersch-Duplay fashion. Hospital records of patients who had undergone two stage modified Bracka repair between June 2007 and July 2012 were reviewed, including complaints, complications, and need for interventions. RESULTS Thirty-eight patients had undergone this operation. Four patients were lost to follow-up. The main complaint was obstructed urinary flow. Voiding symptoms were first attributed to urethral stenosis, but were, however, found to be due to diverticulum and vortex of the urine in the dilated urethra. Twenty-one patients (61%) had voiding problems and 10 patients (29%) had urinary tract infections. Fistula was observed in 23 and diverticula were observed in 24 patients. Of these, 16 patients had both fistula and diverticula. Only two patients (5%) were free of complications and totally satisfied with the operation, and 23 of the 34 patients had complications requiring intervention (Figure). DISCUSSION Inner preputial flaps used in proximal hypospadias repairs are prone to diverticula formation. They become redundant in time requiring reoperation, thus decreasing the success rate. Careful fixation of the flap to the corpora and allowing time for additional attachment of the urethral plate substitution through fibrotic activity could not overcome this complication. CONCLUSION Our modification of the Bracka technique using a flap for the plate resulted in a high rate of complications (in particular diverticulum formation) and was therefore abandoned. We recommend careful use of flaps in hypospadias surgery and long-term follow-up studies to evaluate actual functional and cosmetic results.

Parental Perception of Terminology of Disorders of Sex Development in Western Turkey

Objective: Disorders of sex development (DSD) is a nomenclature intended to defeat the discomfort of families and patients and has found worldwide usage. The aim of this study was to address the perception and usage of terminology among the parents of DSD patients in a tertiary center in western Turkey. Methods: The records of the DSD council (multidisciplinary team where each patient with DSD is discussed) between years 2008-2015 were reviewed retrospectively. Data including details of the management process, patient characteristics and follow-up details were noted. Then inquiries reflecting parental perception about terminology were implemented during clinical visits. Results: In total, 121 patients were evaluated in monthly meetings of the DSD council and 79 inquiries were completed. Fifty-one percent of the families admitted knowing the terms DSD, ambiguous genitalia, “dubious genitals” and intersex. However, only 2% preferred using DSD, 6% intersex and 14% ambiguous genitalia. Fifty-two percent of the parents used a disease name in Latin (mostly hypospadias) addressing the disorder. The offspring of 69% of the parents who were familiar with the name “dubious genitals” were diagnosed in the neonatal period. The preferred terminology used by parents was strongly associated with the terminology used most commonly in the medical speciality their child most often attended. Conclusion: Each country has its own social norms. We suggest therefore that local committees including medical professionals, patients and families should be employed to develop proper terminology.

Non-neuropathic Neuropathic Bladder: Is it Really Non-neuropathic?

Purpose: Hinman syndrome is a rare disease with urodynamic findings and a clinical course resembling neuropathic bladder, without a neuropathic etiology. Diffusion tensor imaging is a special technique of magnetic resonance imaging that has recently been used to evaluate the peripheral nerves but has been demonstrated to be applicable for evaluation of the lumbosacral plexus. We examined the lumbosacral plexus using diffusion tensor imaging, which has not previously been reported in patients with Hinman syndrome. Materials and Methods: The study included 12 patients who fulfilled criteria for Hinman syndrome, with severe bladder dysfunction on urodynamics, renal scarring on scintigraphy and no pathological findings on magnetic resonance imaging. The 12 subjects serving as controls required pelvic or spinal magnetic resonance imaging for reasons other than spinal abnormalities. Evaluation was performed with a 3.0 Tesla magnetic resonance imaging system and 16-channel body coil. Tractography was done to examine the lumbosacral plexus. Fractional anisotropy and mean diffusivity were computed and compared between groups for the right and left plexuses. Results: Mean fractional anisotropy was 0.24 and 0.35 for the right plexus in patients and controls, respectively, and 0.24 and 0.36 for the left plexus. Mean diffusivity was 1.39 for the right and left plexuses in patients, and 1.28 for the right and left plexuses in controls (p <0.001 for all). Conclusions: Our study focusing on the lumbosacral plexus as a possible origin of neuropathy revealed abnormal findings in patients with Hinman syndrome resembling nerve injury series. This is the first known study to provide data showing that Hinman syndrome may have a neuropathic etiology.

Çocuklarda üriner inkontinens Çağrılı Yazar

Ozet Enurezis nokturna cocukluk caginda idrar kacirmanin en sik sebebidir ve isemenin fizyolojik olmasi kendiliginden duzelmesi ile karakterizedir Idrar kacirma ile basvuran cocuklarin cogunda altta yatan yapisal ya da norolojik herhangi patoloji bulunmamasina ragmen bu cocuklarda dogru tani koymak cok onemlidir Ayrintili ve iyi yapilandirilmis bir oyku inkontinensi buyuk oranda tanimlamaya yeteceginden ek incelemeler yalnizca kisitli sayida hastada gerekmektedir Hekim isemeyle ilgili neyin kusurlu oldugunu anlar ve semptomu degil altta yatan sebebi tedavi ederse hem idrar kacirmanin cocugun sosyal gelisimi uzerine olumsuz etkilerini azaltacak hem de ust uriner sistemin olasi hasarindan koruyacaktir Bu sebeple bu yazi daha cok uriner inkontinens sebeplerinin ayirici tanisina odaklanmistir Turk Arch Ped 2010; 45 Suppl: 90 3 Anahtar kelimeler: Enurezis idrar kacirma uriner inkontinans