The state of continence with the laparoscopic technique in high anorectal malformations in this study showed acceptable results but needs bigger series with longer follow-up for a proper evaluation of this technique.
Two doses factor concentrate and gelatin sponge application were generally enough to prevent bleeding after circumcision of severe hemophilia A. Circumcision and low-dose FVIII protocol were not an additional risk for development of high-titer inhibitor.
Background: Persistent cloaca or cloacal anomalies represent a special category of anorectal anomalies affecting the female sex with a reported incidence of about one in 25,000 live birth. The study included 34 cases of cloaca that were managed at our unit between 2003 through 2017. We retrospectively reviewed patients' records that included clinical presentation, investigations, operative data, and follow-up notes. Anatomically, we stratified cloaca into three types according to the level of urogenital confluence. A low confluence (type 1) was defined by being at or below the level of the lower border of pubic symphysis with a short common channel (11 cases). A high confluence (type 3) was defined by being at or above the level of the upper border of pubic symphysis (9 cases). Between the low and high types, we defined an intermediate type (type 2) where the urogenital confluence was behind the mid-portion of pubic symphysis (14 cases). Results: Renal anomalies were common association: solitary kidney in seven, pelvic kidney in two, and urinary tract dilatation (hydroureteronephrosis) in 12 cases. At follow-up, chronic renal insufficiency was detected in seven cases The prognosis for urinary continence was excellent in low confluence (type 1) cloaca. On the other hand, urinary incontinence was common among type 3 (high confluence) cloaca (62.5%). Conclusion: Renal anomalies represent a common association with cloaca and a major cause of morbidity. Efforts should be directed to preserve renal function during the initial management, and to preserve the continence potential following the definitive repair. Level of evidence: This is a case series with no comparison group (level IV).
Background
Undescended testis or cryptorchidism is one of the most common congenital abnormalities of the genitourinary system in young boys, approximately 1-2% of boys at the age of 1 year have undescended testis, the disorder being unilateral in about 90% of cases and bilateral in about 10%.
Aim of the Work
We conducted this prospective study to assess the efficacy and safety of single-incision, transscrotal orchidopexy in children with palpable UDT.
Patients and Methods
A prospective study was adopted to fulfill the purpose of the study. The study was conducted at Pediatric Surgery Department, Ain Shams University Hospitals in Cairo. The included study population was pediatric patients with palpable, inguinal mal-descended testes who attend to Pediatric Surgery Outpatient Clinic, Ain Shams University Hospital till the fulfillment of the sample size.
Results
The most common sites of undescended testis were intracanalicular and scrotal neck regions. The most common postoperative complication was scrotal edema. Mean operating time was 21 minutes. There was no significant difference in the testicular size pre and post operative.
Conclusion
Based on these results, we concluded that the undescended testis represents a common pediatric problem requiring surgical intervention. Trans-scrotal orchidopexy is an effective, less invasive and highly acceptable cosmetically approach for the treatment of palpable undescended testis.
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Background
Circumcision is a cultural practice for males in the Middle-East during first weeks of life. All parents of hemophilics are eager to do circumcision to their sibs, however, it may carry a risk for development of factor VIII inhibitors as well as risk of excessive bleeding.
Objective
To evaluate post-circumcision bleeding and assess incidence and time of inhibitor development over 12 months follow-up period of minimally treated severe hemophilia A patients.
Patients and methods
This prospective analysis has been conducted on eighteen minimally treated patients with severe hemophilia A (age range 8–36 months) with a median age of 18 months, who underwent circumcision during 2009 and twenty four age matched non circumcised patients minimally treated severe hemophilia A. Both groups were followed up for12 months from study entry and all were treated on demand therapy with a single plasma-derived factor VIII product. Hemophilic patients who underwent circumcision were inhibitor negative except two with low- titer inhibitor(3.3 and 4.4 BU/ml) respectively. One hour before the operation, intravenous tranexamic acid (25 mg/ kg) and first dose of factor concentrate (25 unit / Kg) were given to the patients. After reaching a trough plasma factor level more than 90%, patients underwent circumcision using general anesthesia and same surgical technique for all. Bolus injections of factor VIII concentrate were repeated in a dose of (25 units / Kg ) twenty four hours after operation. However, the two patients with inhibitors were given factor VIII concentrate in a dose of (50 units /Kg) with an extra dose at forty-eight hours. Another dose of factor concentrate (25 units/ Kg) was given just before removal of gauze dressing at 5th −7th day post operative. Follow up for inhibitor development was assessed every 8 exposure days (EDs) for 12 months or 100 EDs whichever comes first.
Results:
Of the eighteen patients enrolled, only one of the 2 patients with low- titer inhibitor had postoperative bleeding at day 5 and 7 respectively. First attack responded to a single dose of factor administration (50 units/Kg), whereas haemostasis was achieved in the second episode after a single dose of Recombinant Factor VIIa (90 microgram/kg) and applying absorbable haemostatic agent (gelatin sponge) and binding. None of the other patients had any bleeding or infection at site of surgery. High -titer inhibitors developed in three patients (16.6 % ) during the follow-up; after 8, 16 and 40 EDs respectively in contrast to four patients (16.6 %) developed high titer inhibitor in the non circumcised group; after a median of 16 exposure days (range 8– 60 EDs).
Conclusion:
Our study has shown that bleeding following circumcision was absent except in low- titer inhibitor patient necessitating administration of Recombinant Factor VIIa. Moreover, circumcision was not a risk for development of inhibitor where the incidence of high- titer inhibitors during12 months follow up was low in this cohort of minimally treated patients and comparable to non circumcised group.
Disclosures:
No relevant conflicts of interest to declare.
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