Mahmoud M. Marei

BACKGROUND: Little has been reported to date on the instillation of antimicrobials directly into the bladder in children. Children with complex urinary tract anomalies struggle frequently with recurrent urinary tract infections (UTI), with frequent emergence of antibiotic resistance. Gentamicin bladder instillation to treat and prevent UTI was described in children since 2006.

OBJECTIVE: We adopted gentamicin bladder instillation in 2016 and evaluate herein our intermediate-term experience with it.

STUDY DESIGN AND METHODS: This study is a retrospective review of a prospectively initiated database and a clinical audit of our practice. The gentamicin bladder instillation was employed in 24 cases. A treatment regime was initiated for symptomatic documented UTI when resistance patterns precluded an oral alternative (14 cases), avoiding hospitalisation for parenteral antibiotics. A prophylaxis regime (19 cases-including 9 of the 14 who received an initial treatment regime) followed at least one breakthrough UTI while receiving oral prophylactic antibiotics. Two instillation volumes (8 mg gentamicin in 20 mL 0.9% NaCl or 20 mg gentamicin in 50 mL 0.9% NaCl) were used to suit different bladder capacities. The irrigation is given twice a day for 7 days in the treatment regime or once a day, every other day, in the prophylactic regime. Gentamicin serum levels (all cases) and audiology/audiometry testing (17/24 cases) were checked to assess the safety of this method.

RESULTS: The median age when either the treatment course or prophylaxis regime was started was 3.8 years. The treatment regime was 86% successful (12/14) to suppress an acute UTI. The mean duration of prophylaxis was 252 days (median: 256 days). The percentage of patients on the prophylactic regime who had no breakthrough UTI was 58%. No serum gentamicin was detectable secondary to the intravesical instillation. No attributable cases of sensorineural hearing loss were detected. Gentamicin resistance emerged in one case (4.16%).

DISCUSSION: Intravesical administration was feasible via various routes for a spectrum of complex lower urinary tract abnormalities (see Summary Figure). Concerns regarding systemic absorption, nephrotoxicity or ototoxicity were investigated and safety ensured. Limitations include being a small series of non-identical pathologies, albeit categorically similar and being a single-arm study, however, statistical significance was proven descriptively and analytically.

CONCLUSION: In selected cases and with the appropriate specialist support and logistics, intravesical gentamicin instillation is well-tolerated and safe to treat and/or prevent urinary tract infections in pateints with complex bladder conditions and lower urinary tract pathologies.

INTRODUCTION: Augmenting the bladder with a seromuscular gastrointestinal flap is a promising alternative approach aiming for a mucus-free bladder augmentation; however, the contraction (shrinkage) of the flaps remains a major concern. Enteric nervous system (ENS) abnormalities cause a failure of relaxation of the intestinal muscle layers in motility disorders such as Hirschsprung's disease and intestinal neuronal dysplasia. In mammals, the submucosal enteric nervous plexus contains nitrergic inhibitory motor neurons responsible for muscle relaxation. The authors hypothesize that mucosectomy disconnects the submucosal nervous plexus from the myenteric plexus resulting in flap shrinkage.

STUDY DESIGN: After ethical approval, mucosectomy was performed on vascularized flaps from the ileum, colon, and stomach in five anesthetized pigs. In Group (I), only the mucosa was scraped off with forceps, creating a sero-musculo-submucosal flap, while in Group (II), the mucosa and submucosa were peeled off as one layer, leaving a seromuscular flap. Isolated and detubularized segments served as control. The width of each flap was measured before and after the mucosectomy. The ENS was assessed by neurofilament immunohistochemistry in conventional sections and by acetylcholinesterase and NADPH-diaphorase enzyme histochemistry in whole-mount preparations.

RESULTS: The stomach contracted to a lesser extent of its original width, 92.82 ± 7.86% in Group (I) and 82.24 ± 6.96% in Group (II). The ileum contracted to 81.68 ± 4.25% in Group (I) and to 72.675 ± 5.36% in Group (II). The shrinkage was most noticeable in the colon: 83.89 ± 15.73% in Group (I) and to 57.13 ± 11.51% in Group (II). One-way equal variance test showed significant difference (P < 0,05) between Group (I) and (II), comparing stomach with ileum and ileum with colon. The histochemistry revealed that the submucosal nervous plexus containing nitrergic inhibitory neurons was disconnected from the myenteric plexus in Group (II) of all specimens.

CONCLUSION: Mucosectomy resulted in significant immediate shrinkage of the flaps. This was more expressed when also the submucosa was peeled off, thus fully disrupting the ENS. The shrinkage affected the stomach the least and the colon the greatest. This phenomenon should be taken into consideration when planning mucus-free bladder augmentation.

Laparoscopic pyeloplasty (LP) is a standard, but demanding operation. According to the European Association of Urology guidelines this procedure scores 13 out of 18 on the difficulty scale [1]. Articulating instruments, 3D video systems and robots may facilitate complex suturing of the ureteropelvic anastomosis at higher costs [2,3]. Magnetic compression anastomosis (magnamosis) has been used safely and effectively in the gastrointestinal tract [4,5], however not been applied in the urinary tract.

Laparoscopic pyeloplasty (LP) is a standard, but demanding operation. According to the European Association of Urology guidelines this procedure scores 13 out of 18 on the difficulty scale [1]. Articulating instruments, 3D video systems and robots may facilitate complex suturing of the ureteropelvic anastomosis at higher costs [2,3]. Magnetic compression anastomosis (magnamosis) has been used safely and effectively in the gastrointestinal tract [4,5], however not been applied in the urinary tract.

BACKGROUND: Several measures were implemented among authors striving to tail off recurrence rates of thoracoscopic congenital diaphragmatic hernia repair. In the presented study, we extended the use of rib-anchoring stitches to reorient the diaphragmatic muscle leaflets in the types B&C diaphragmatic hernias, to achieve tension dispersal at primary thoracoscopic repair.

PATIENTS AND METHODS: Included in this study were early and late-onset lateral congenital diaphragmatic hernia patients, who had been operated upon in the years 2012 through 2018. A preliminary stitch was taken between posterior muscle edge and rib cage to reorient the diaphragmatic defect into a reversed C-shaped line. The lateral portion was closed by additional rib-anchoring stitches, while the medial one necessitated muscle to muscle stitches. Primary outcome being validated was the recurrence rate within a year post repair.

RESULTS: In the 7-year inclusion period, 36 congenital diaphragmatic hernia cases were managed using the described approach. The repair was accomplished thoracoscopically in all but two cases, who were excluded from the study. Mean operative time was 76 min. No pledgets or synthetic patches were applied. Mean length of hospital stay was 7.6 days. Early postoperative course was uneventful in all but four cases; two ventilatory barotrauma and two mortalities. After a mean follow-up period of 29 months, five recurrences were reported (16%). Ipsilateral chest wall deformity was noticed in one case 3 years post repair.

CONCLUSION: In the presented study, authors adopted thoracoscopic reorientation of diaphragmatic muscle leaflets in lateral congenital diaphragmatic hernia cases to achieve tension dispersal at primary repair. Short and mid-term results supported the efficacy and reproducibility of the described approach. However, long-term comparative studies seemed a necessity to validate this outcome.

: To examine the role of laparoscopy in managing unsatisfactory testicular position after an open inguinal orchidopexy. We hypothesised that testes that were originally peeping, where short vessels represented a difficulty and testes that only reached a high scrotal position under tension, especially after an initial surgery performed with the appropriate expertise, are candidates for initial laparoscopic dissection. : Nineteen boys with an initial open inguinal orchidopexy, with a mean age of 31 months, were considered. Twelve were then treated by a laparoscopic-assisted orchidopexy technique. Standard laparoscopy was established and utilised to mobilise the spermatic cord from above, then completed by an open inguinal mobilisation. : The mean age at surgery was 26 months. The laparoscopic redo surgery took place at a mean interval of 11.9 months after the initial operation. The mean operative time was 72 min. A good position and size of the testis were achieved in all cases, evidenced by ultrasonography at 6 months postoperatively and clinically thereafter. : An upfront combined laparoscopic and inguinal approach to redo orchidopexy for recurrent palpable undescended testes is suitable in selected patients. This study identifies the selection criteria and outlines the operative considerations. This laparoscopic-assisted approach is a safe and feasible way to correct unsatisfactory position of the testis, with diminished risk of injury to the vas and vessels, while gaining the maximum possible length by high retroperitoneal dissection. UDT: undescended testis/testes.

INTRODUCTION: Surgical management of the high urogenital sinus (UGS) is challenging. Presence of a short urethra is a contraindication for UGS mobilization as this is likely to affect urinary continence. In addition, vaginal reconstruction of the high suprasphincteric confluence is complex and prone to complications and failure.

METHODS: This study included 7 girls who underwent a laparoscopically assisted vaginal pull-through in the past 2 years. All cases included were diagnosed with congenital adrenal hyperplasia (CAH), with a high UGS, and a short urethra of ≤15 mm above the confluence. Patients were preoperatively assessed by genitography. Mobilization of the vagina was pursued until the confluence became visible and the vaginal caliber tapered off at its junction with the urethra. The connection was sutured or clipped and divided. Uterine round ligaments were divided to allow delivering the vagina to the perineum with minimum tension. The tract for the pull-through was created and the vagina was pulled outside and sutured to the perineal skin. Good vaginal mobilization allowed a direct perineal anastomosis without skin flap augmentation of the vaginal wall. Clitoroplasty and labiaplasty were deferred.

RESULTS: Mobilization of the vagina was possible in all cases attempted, without injuries to the adjacent pelvic structures. Calibration and dilatation started 2 weeks postoperatively. At 12-month follow-up, all the patients had a patent vagina with good caliber, and were able to retain urine without dribbling.

CONCLUSION: The laparoscopically assisted vaginal pull-through approach provides optimal exposure, facilitates vaginal dissection, separation from the urethra, and avoids injuries to the urinary structures. This allows vaginal reconstruction without tension.

BACKGROUND: Virilized females due to congenital adrenal hyperplasia represent the most common form of female disorders of sexual development. The anomaly therein is an external virilization to resemble male genitalia and a persistent urogenital sinus.

OBJECTIVES: To study the anatomical details of the virilized female cases operated upon between 2011 and 2015. This anatomical description is presented to support the current surgical strategy of partial urogenital mobilization to correct this anomaly.

METHODS: Thirty cases (presenting to a single tertiary center) were prospectively studied by genitography, cysto-urethroscopy, and operated upon via a single-stage feminizing genitoplasty. A single surgical team operated upon all cases. External virilization was assessed by the Prader classification. The internal anatomy was studied by measuring the length of the urethra proximal to the confluence, and the vertical depth of the vaginal-urethral confluence from the perineum. The correlation coefficients between the external virilization and the internal anatomical measurements were derived.

RESULTS: The median age at surgery was 19 months (range 6-42 months). External virilization did not obviously correlate with the length of the proximal (prejunctional) urethra (r = -0.03, P = 0.5), or strongly with the depth of the vaginal-urethral confluence (r = 0.2, P = 0.2). The mean length of the proximal urethra was 22 mm (range 10-32 mm), and the mean vertical depth of the vaginal-urethral confluence from the perineum was 16 mm (range 8-31 mm).

DISCUSSION: Due to limitations of the radiological and endoscopic evaluation, the accurate anatomical assessment of this condition may be challenging. In order to assess or compare the anatomy of these cases, there are two important points to address: (1) the length of the urethra proximal to the urogenital sinus, as this will impact the urinary outcome; and (2) the depth (level) of vaginal entry into the urogenital sinus, as this will affect the mobilization required to exteriorize the vagina.

CONCLUSION: The degree of external virilization does not totally correlate with the internal anatomy. The depth of the vaginal-urethral confluence from the perineum is an indicator of the required mobilization for the current perineal approach. In 90% of cases in this age group (1-3 years old), this depth is ≤20 mm. This supports the current understanding that partial urogenital mobilization could be suitable for most cases Figure (Summary).

BACKGROUND: Congenital adrenal hyperplasia in females leads to virilization of external genitalia and persistent urogenital sinus. There are controversies regarding the timing and outcomes of surgery. Deferring surgeries beyond childhood is difficult to implement in conservative societies, and may result in stigmatization and distress to individuals with disorders of sexual differentiation and their families.

METHODS: Thirty girls with virilization due to congenital adrenal hyperplasia were admitted for single-stage feminizing genitoplasty, between 2011 and 2014. We prospectively studied the concerns and input of the families represented by the mothers. After comprehensive counselling, the mothers completed a questionnaire to clarify their priorities and concerns related to surgery.

RESULTS: Surgeries were performed at a mean age of 22 months. Most cases ranged between Prader's degrees III and IV. Egyptian families believe that early surgical reconstruction is in the best interest of their girls. They are marginally more concerned about functional outcomes and future child bearing than external appearance and cosmetic outcomes.

CONCLUSIONS: Social difficulties noticeably add challenges to the management plan within conservative societies. Early genital reconstructive surgery, when reasonably indicated, needs to remain a viable option. Comprehensive psychosocial support within a multidisciplinary approach is needed to defer feminizing genitoplasty in selected cases to adolescence.

BACKGROUND: Virilized females due to congenital adrenal hyperplasia represent the most common form of female disorders of sexual development. The anomaly therein is an external virilization to resemble male genitalia and a persistent urogenital sinus.

OBJECTIVES: To study the anatomical details of the virilized female cases operated upon between 2011 and 2015. This anatomical description is presented to support the current surgical strategy of partial urogenital mobilization to correct this anomaly.

METHODS: Thirty cases (presenting to a single tertiary center) were prospectively studied by genitography, cysto-urethroscopy, and operated upon via a single-stage feminizing genitoplasty. A single surgical team operated upon all cases. External virilization was assessed by the Prader classification. The internal anatomy was studied by measuring the length of the urethra proximal to the confluence, and the vertical depth of the vaginal-urethral confluence from the perineum. The correlation coefficients between the external virilization and the internal anatomical measurements were derived.

RESULTS: The median age at surgery was 19 months (range 6-42 months). External virilization did not obviously correlate with the length of the proximal (prejunctional) urethra (r = -0.03, P = 0.5), or strongly with the depth of the vaginal-urethral confluence (r = 0.2, P = 0.2). The mean length of the proximal urethra was 22 mm (range 10-32 mm), and the mean vertical depth of the vaginal-urethral confluence from the perineum was 16 mm (range 8-31 mm).

DISCUSSION: Due to limitations of the radiological and endoscopic evaluation, the accurate anatomical assessment of this condition may be challenging. In order to assess or compare the anatomy of these cases, there are two important points to address: (1) the length of the urethra proximal to the urogenital sinus, as this will impact the urinary outcome; and (2) the depth (level) of vaginal entry into the urogenital sinus, as this will affect the mobilization required to exteriorize the vagina.

CONCLUSION: The degree of external virilization does not totally correlate with the internal anatomy. The depth of the vaginal-urethral confluence from the perineum is an indicator of the required mobilization for the current perineal approach. In 90% of cases in this age group (1-3 years old), this depth is ≤20 mm. This supports the current understanding that partial urogenital mobilization could be suitable for most cases Figure (Summary).

BACKGROUND: Congenital adrenal hyperplasia in females leads to virilization of external genitalia and persistent urogenital sinus. There are controversies regarding the timing and outcomes of surgery. Deferring surgeries beyond childhood is difficult to implement in conservative societies, and may result in stigmatization and distress to individuals with disorders of sexual differentiation and their families.

METHODS: Thirty girls with virilization due to congenital adrenal hyperplasia were admitted for single-stage feminizing genitoplasty, between 2011 and 2014. We prospectively studied the concerns and input of the families represented by the mothers. After comprehensive counselling, the mothers completed a questionnaire to clarify their priorities and concerns related to surgery.

RESULTS: Surgeries were performed at a mean age of 22 months. Most cases ranged between Prader's degrees III and IV. Egyptian families believe that early surgical reconstruction is in the best interest of their girls. They are marginally more concerned about functional outcomes and future child bearing than external appearance and cosmetic outcomes.

CONCLUSIONS: Social difficulties noticeably add challenges to the management plan within conservative societies. Early genital reconstructive surgery, when reasonably indicated, needs to remain a viable option. Comprehensive psychosocial support within a multidisciplinary approach is needed to defer feminizing genitoplasty in selected cases to adolescence.

Scottish Surgical Paediatric Society
May 2015
M Marei

Review of Remnant Müllerian Structures in Male DSD, The Edinburgh Experience.

Introduction: Müllerian duct regression occurs in males, between the 8th and 11th weeks of intra-uterine life. It is under control of AMH which is produced by the pre-Sertoli cells. Müllerian structures could persist due to lack of AMH, or a defect of its receptors, as well as in the dysgenetic types of male DSD. The cases may present by proximal hypospadias, and/or testicular maldescent of various combinations and severity.

Methods: Five cases of remnant Müllerian structures were managed at our centre in the last four years. The presentations met and operations performed in these complex cases were reviewed, to help devising a protocol and clear indications of surgical removal.

Results: In this series three cases were discovered during groin conditions, one during an inguinal hernia repair, one during a groin orchidopexy for a palpable undescended testis, and one during a laparoscopic orchidopexy for an impalpable testis. Two cases were discovered during laparoscopy, as an investigation for genital ambiguity in the neonatal period, to enable allocating the sex of rearing.

Conclusion: The indications of removal of a Müllerian structures in this series were to enable tension-free orchidopexy, as they anchor the testis to a higher position, and as a preparation for proximal hypospadias repair to avoid their filling with urine due to outflow resistance following urethroplasty, hence stasis and infection. It was possible to remove the Müllerian structures in all cases laparoscopically.

Introduction

Hirschsprung’s disease (HD) should be considered in children with neonatal-onset constipation. Clinical differentiation between HD and idiopathic constipation (IC) is difficult in late presenting infants. Consequently, paediatric surgical centres receive numerous referrals for rectal biopsies, requiring admissions and GA, particularly if suction biopsy is unavailable, and in older children.

Methods

Forty-two cases referred for rectal biopsy, were studied for clinical features, single contrast enema, as compared to rectal biopsy findings, to determine the statistical reliability towards achieving a diagnosis.

Results

The mean age at presentation was 106 days in HD patients, and 172 days in IC. Significant neonatal clinical features were present in 54%. Delayed passage of meconium was present in 86% of HD, compared to 14% of IC (p = 0.001). Rectal examination found a tight segment in 90% of HD, and a distended anorectum in 64% of IC (p = 0.005). The sensitivity of contrast enema was 86%, and the specificity was 90%. The histological analysis of strip rectal biopsy was sensitive in 93%, and inconclusive in 7%.

Conclusion

This audit generated a checklist of 6 clinical and 3 radiological criteria, to differentiate HD from ID, including clinically (1) neonatal onset; (2) male sex; (3) congenital anomalies, dysmorphic features and/or family history of HD; (4) delayed meconium passage; (5) enterocolitis or significant bowel obstruction/impaction; (6) tight segment on rectal examination; and radiologically (7) funnelled transition zone or a reversed rectosigmoid index (<1); (8) delayed evacuation of contrast after 24 h; and (9) absent distension of the anorectum with contrast, absent mucosal irregularities, and absent sigmoid looping.

Background/aim: This type of diaphragmatic hernia describes an anterolateral defect in the diaphragm. The defect boundaries can be clearly defined thoracoscopically as the anterolateral edge: the costal margin and the posteromedial ‘‘C-shaped’’ muscle edge. We describe a simple tension free thoracoscpic technique for its repair.

Methods: Using three 5-mm instruments, several rib anchoring stitches, the C-shaped diaphragmatic defect is reoriented into a sickle shaped repair. This reorientation creates two limbs: one limb is the lateral, between the costal margin and the muscle and a vertical limb where the muscle is sutured to itself. In a five years period 31 patients were treated using this technique.

Results: Their age ranged from 8 months to 3 years. The side of anterolateral diaphragmatic hernia was on left in 26 and right in five cases the cases. The mean operative time was 105min and the mean follow-up time was 29 months. There was no procedure-related major complication or mortality. The repair was completed in all case thoracoscopically using 2/0 Ethibond stitches. Prolonged ileus was noticed in 3 patients. Seven children suffered from mild reflux symptoms that persisted for 6 to 9 months. Reflux symptoms in 7 patients. Buried stitches caused discomfort in two patients. Chest deformity was reported in one patient. No recurrences was reported in any of the patients.

Conclusion: Thoracoscopic revered orientation of the anterolateral diaphragmatic defect offers a tension free repair. An added benefit is the elimination of use of synthetic material.

Annals of Pediatric Surgery 2015, 11:65–112

Virilized females due to congenital adrenal hyperplasia present the most common form of female DSD. The anomaly is an external virilization resembling male genitalia and a persistent urogenital sinus. The length of the proximal urethra, measured from the bladder neck to the vaginal-urethral confluence is an important predictor of surgical complexity. As the reconstruction is through a perineal approach, the vertical depth of the confluence from the perineum is similarly an important measurement. It determines the amount of the dissection required to deliver the vagina. There is no reported description of this level or depth, hence we present the measurements found in our series, and suggest a method to assess the difficulty of cases by applying a calculated ratio.

British Association of Paediatric Surgeons, Edinburgh, BAPS 61st Annual Congress

Congenital adrenal hyperplasia in females leads to virilization of the external genitalia and an anomalous genitourinary tract, with a termination of the urethra and vagina into the persistent urogenital sinus. Surgical repair during infancy is indicated to raise a female child with external genitalia matching her gender and to avoid psychosocial and genitourinary complications. Earlier surgery is believed to be easier and of better results. Twenty five female patients with virilized external genitalia and persistent urogenital sinus due to congenital adrenal hyperplasia were studied. Interpretation of the genitography and cystoscopy was analyzed and compared to the operative findings to assess their accuracy and predictability. Sensitivity of cystoscopy and genitography is comparable (78.5% and 73% respectively), but cystoscopy has a higher specificity than genitography (91% and 83% respectively). Both investigations had a similar predictivity and were able to reveal the internal anatomy and operative difficulty in 56% of cases. In 12% both investigations had a similar predictivity, but failed to expect the operative difficulty. Cystoscopy was superior to genitography in 24%, but surprisingly genitography was more predictive in 8%. Neither genitography nor cystoscopy were independently predictive of the internal anatomy and intraoperative findings or difficulty of the studied cases. We therefore recommend doing both investigations as a routine preoperative workup. The benefit is to assess these cases properly and support decision making and choice of surgery.

Key words: DSD, Feminizing Genitoplasty, Cystoscopy, Genitography.

Kasr El Aini Journal of Surgery. 01/2014; 15(1):21-25.

Background and Rationale: Congenital adrenal hyperplasia in females leads to virilization of the external genitalia and an anomalous lower genitourinary tract. Hormonal therapy controls the endocrinopathy. Surgical repair of the anomaly is indicated to allow raising a female child with external genitalia matching her gender and to avoid psychological complications. Surgery at an earlier stage is believed to be easier and of better results than when deferred to adolescence.

Objectives: To study the anatomical abnormalities of genotypic females with virilized atypical genitalia due to congenital adrenal hyperplasia and their impact on surgery and to review the surgical techniques of feminizing genitoplasty and their early short-term outcomes. We aim to highlight the multidisciplinary management for these complex cases and to discuss the difficulties and challenges met during this.

Patients & Methods: Genotypic prepubertal females with virilized atypical genitalia due to congenital adrenal hyperplasia were included. Radiological studies in the form of sonography to visualize the uterus and gonads and genitography for delineation of the urogenital tract then followed. Endoscopic assessment (cystoscopy) was done at the time of surgery with catheter placement in both the vagina and urethra to guide the surgical procedure. Surgical genitoplasty aimed at a single-stage full correction for all cases preferring partial urogenital mobilization.

Outcome parameters: Achievement of an acceptable female appearance of the external genitalia, achievement of adequately sized and positioned vaginal and urethral orifices, and occurrence of short term complications.

Conclusions: Feminizing genitoplasty can be done safely between the age of 6 months and one year. Flap vaginoplasty and cut-back exteriorization are only suitable for low confluence. Partial urogenital mobilization is a safe and effective technique that is suitable to most cases.

KASR EL AINI MEDICAL JOURNAL. 01/2014; 20(1):17-27.

Congenital adrenal hyperplasia in females leads to virilization of the external genitalia and anomalous genitourinary tract. Surgical repair during infancy is indicated to raise a female child with external genitalia matching her gender and to avoid psychosocial and genitourinary complications. Earlier surgery is believed to be easier and of better results. The outcome parameters studied in this work are; (a) parents' and professionals' satisfaction of the appearance of the feminized external genitalia, (b) adequately sized and positioned vagina and urethra and (c) occurrence of short term complications. We propose a link between the two known theories by stipulating that although the confluence depth and operative difficulty occur in a spectrum rather than either "high" or "low"; this spectrum is concentrated in its middle third where the vagina joins the urethra near the point of pseudo-verumontanum.The degree of virilization does not accurately reflect the depth of the confluence or the operative difficulty.

Key Words: DSD, Intersex, Feminizing Genitoplasty, Urogenital Sinus, Virilization, Female Pseudohermaphrodite, Congenital Adrenal Hyperplasia.

03/2013, Degree: PhD Paediatric Surgery, Supervisors: Ayman H Abdelsattar, Mona Mamdouh Hassan, Mohamed M Elbarbary, Montasser M Elkotby

AIM OF THE WORK

The aim of this work is to study the accuracy of contrast enema in the diagnosis of Hirschsprung's disease comparing it with the results of rectal biopsy which represents the gold standard.

SUMMARY AND CONCLUSION

The diagnosis of Hirschsprung’s disease presents a clinical challenge to the pediatric surgeon. Hirschsprung’s disease should be considered in any child who has a history of constipation dating back to the newborn period. In our study, the results of the contrast enema are well correlated and comparable to the rectal biopsy in the diagnosis of Hirschsprung’s disease. As barium enema is a noninvasive diagnostic tool, the presence of strongly positive enema findings associated with classic clinical presentation is considered in the author’s point of view sufficient evidence to operate. In cases of obistipant constipation and inconclusive contrast enema findings, thorough and careful medical management should be initially attempted, if failed; rectal biopsy by an experienced pediatric surgeon is indicated.