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Abstract Background There is no consensus on the initial treatment modality for unilateral Wilms tumor (WT) in Egypt. This multicenter study investigates the factors guiding the decision-making between primary surgery and delayed resection for this tumor in our clinical practice. A retrospective analysis was conducted on all patients with unilateral, non-metastatic WT treated at four major Egyptian institutions from January 2019 to January 2024. Clinical characteristics, management details, and outcomes were reviewed. Timing of nephrectomy was analyzed in relation to tumor characteristics such as size, midline crossing, suspicion of perinephric spread, intravascular extension, and retroperitoneal lymphadenopathy. Results The study included 112 patients with a median age of 2.8 years. The median largest tumor diameter was 12 cm (range, 7–16 cm). Primary nephrectomy was performed in 62 patients (55.4%), while 50 (44.6%) underwent delayed resection after biopsy and neoadjuvant chemotherapy. Tumors larger than 12 cm and tumors crossing the midline were reported in 38.5% and 35% of all patients, respectively. Suspicion of perinephric spread was noted in 31% of patients, and significant lymphadenopathy in 13.5%. Intravascular tumor thrombus was detected in 7 patients (6.25%) at various locations. Statistically significant differences were observed between the primary surgery and delayed resection groups regarding suspicion of perinephric spread, intravascular thrombus, and retroperitoneal lymphadenopathy (p-value

Abstract Purpose To share our experience in the surgical management of patients with Hirschsprung disease (HD) using the trans-anal endorectal pull-through “Soave” technique. Methods The study included cases diagnosed as HD who were operated during the period 2008 through 2018. Cases of total colonic aganglionosis were excluded to be discussed in a separate report. Results The study included 67 consecutive cases of HD. All included cases underwent trans-anal endorectal pull-through which was purely trans-anal in 31 (46%), while abdominal-assisted trans-anal endorectal pull-through was applied in the remaining (54%). Early postoperative complications included 2 cases of partial dehiscence at the colo-anal anastomosis; 1 of them deteriorated after re-exploration and unfortunately died. Twenty-six cases were available to assess functional outcomes after corrective surgery. Their age at follow-up ranged between 44 months and 17 years (mean = 78.8 months; median = 72 months). All cases achieved voluntary defecation. However, fecal soiling was a common finding after surgery for HD (69%). Constipation was another common postoperative finding (73%). Lastly, a history of attacks of postoperative enterocolitis was prevalent in 19 cases (73%), which ranged between 1 and 7 attacks usually in the first 2 years after the operation. Conclusion The trans-anal endorectal pull-through provides an effective surgical treatment for Hirschsprung disease. The high prevalence of fecal soiling after surgery highlights the importance of long-term follow-up to provide the required support for these patients during adolescence and transition into adulthood.

Abstract Purpose To share our experience in the management of a specific type of mild anorectal anomaly presenting with severe constipation. Methods Data of patients with idiopathic constipation who were referred to our pediatric surgical facility during the period from 2013 to 2023 were retrospectively analyzed. The study included patients with significant symptoms of constipation associated with a minor degree of unrecognized anterior anal misplacement. MRI was ordered in most cases to confirm the presence of deeper structural abnormalities. Results Data from 16 cases were available for retrospective analysis. All included cases underwent posterior anoplasty with posterior sphincterotomy. Postoperative recovery was uneventful in all cases with no wound complications. Almost all cases reported obvious improvement of symptoms regarding frequency of defecation and relief of straining which was the most distressing preoperative complaint. After 1 month, 12 cases were off laxatives with regular defecation; 3 cases still required lower doses of laxatives. Only one case reported unsatisfactory response up to 3 months postoperatively, and unfortunately was lost to follow-up. Five cases responded to messages sent to them through registered phone numbers and were available for longer-term follow-up. Conclusion Mild unrecognized forms of anorectal anomalies may present with idiopathic constipation. Severe straining at defecation is an important clue. The subtle abnormality on perineal examination can be supported by pelvic MRI unveiling more structural abnormalities at a deeper level. Posterior anoplasty offered relief of symptoms in most cases.

Abstract Purpose To share our experience in the management of a specific type of vascular malformations (lymphatic) at a specialized multidisciplinary clinic for vascular anomalies. Methods Data of patients attending the vascular anomaly clinic during the period 2015 through 2023 were retrospectively analyzed. The study included cases diagnosed primarily as lymphatic malformations (LMs). We excluded cases associated with complex/syndromic vascular malformations. Available data included regional distribution of the LMs, age at presentation, sex, imaging studies, and different modes of treatment. Results The study included 131 cases of LMs whose data were available for retrospective analysis. Generally, LMs had a benign course with good prognosis apart from two recorded mortalities (1.5%) during the 9-year period of the study. In this series, 93 cases were managed by injection sclerotherapy (Bleomycin). About 57 cases showed satisfactory response to injection sclerotherapy alone without the need to add other treatment modalities. Forty cases underwent surgical excision/debulking. Postoperative wound complications were recorded in 5 cases (12.5%). Sirolimus was offered for patients with LMs after the failure of conventional treatment (injection/surgery) to control associated significant complications. Conclusion Lymphatic malformations represent a common presentation at the vascular anomaly clinic, which usually have a benign course. Complications are mainly cosmetic especially when involving the face, and sometimes superadded infections may occur. Airway compromise is a potential serious complication with submandibular lymphatic malformations in the neck.

Abstract Objectives Nationwide criteria regarding patients with sacrococcygeal teratoma (SCT) are still lacking in Egypt. We aimed to present a multicenter study regarding the management and outcomes of this tumor to evaluate our national treatment strategy. Methods A retrospective analysis including all patients with SCT who were managed at four major Egyptian centers between 2013 and 2023. Clinical data, surgical approaches, and short- and long-term outcomes were discussed. Results The study included 95 patients (74 were females). Antenatal diagnosis was reported in 25% of patients. Seventy-one patients (74.7%) were classified as Altman type I/II. Surgery was performed via a perineal approach in 75 patients, whereas the remaining 20 underwent a combined abdominoperineal approach. Vertical elliptical incision with midline closure was conducted in 51.5% of patients, followed by classic or modified chevron incisions. Benign mature teratoma was detected in 82% of patients. At a median follow-up of 57 months, eight patients (8.5%) had relapsed. The 5-year overall survival (OS) and event-free survival (EFS) of all patients were 94% and 91%, respectively. In the after-care monitoring, 19 patients (20%) had urinary or bowel dysfunctions. Nine of them were managed using medications. Clean intermittent catheterization was practiced in another five patients. The remaining five underwent further surgical interventions. Conclusion Favorable outcomes were achieved in our country during the last decade. Diverse perineal incisions were performed for resection, and vertical elliptical with midline closure was the commonest. During follow-up, 20% of patients developed urological or bowel dysfunctions that required medical and surgical treatment modalities to improve their quality of life.

Abstract Background The application of a second layer between the neourethra and skin was a major contribution, which has improved the outcome of hypospadias repair. Here, we report our experience of revisiting the original Smith technique using a de-epithelialized overlap flap to support the urethroplasty in staged hypospadias repair. Methods The study included primary cases of proximal hypospadias with significant chordee who underwent two-stage repair during the period 2016 through 2021. The ventral curvature was corrected at first stage by excision of the urethral plate, followed by covering the ventral shaft by skin flaps or inner preputial graft. The second stage (Thiersch -Duplay urethroplasty) was performed six months later. The de-epithelialized overlap flap (double breasting) technique was used to cover the neo-urethra in all cases, which was combined with a dartos scrotal flap to cover the proximal neourethra when indicated. Results The study included 17 boys with proximal hypospadias who underwent two-stage repair. Follow up period after the second stage ranged between 6 and 30 months (mean 19.7; median 18.5). Post-operative complications were detected in 7 cases (41%). Most complications were related to distal/glanular disruptions whether partial or complete (5 cases). One case developed a penoscrotal fistula that was closed surgically. Another case (belonging to the group which used preputial graft in the 1st stage) presented 21 months after the second stage with urethral stricture (penoscrotal). Conclusion Applying the de-epithelialized double-breasting skin closure can offer alternative second layer coverage for the neourethra along the penile shaft in staged repair of proximal hypospadias.

We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. The patient is placed in the prone position for a standard posterior sagittal anorectoplasty. The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (∼1 cm) through the posterior wall of the common urogenital sinus toward but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards toward the perineum (posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow-up, as well as improvement of upper urinary tract dilatation. In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.

Abstract Background Y-type urethral duplication is a term frequently used to describe a rare condition in the male associated with double urinary stream: a weak interrupted urinary stream through a hypoplastic penile urethra, while the main urine flow is through the anus/perineum via accessory posterior channel. Fortunately, the advent of MRI has provided a powerful tool to study these anomalies in depth and on multiple planes. The study included boys presenting with abnormal micturition through the anus beside weak interrupted urine stream through the penis. Investigations included pelviabdominal ultrasound to screen for possible associated upper urinary tract anomalies, conventional contrast X-ray studies, and pelvic MRI. Results During the period between 2016 through 2021, the study included two newly diagnosed cases with abnormal communication between the lower urinary tract and the anus (the so-called Y-type urethral duplication), in addition to one previously reported case whose preoperative imaging studies (including pelvic MRI) were available for analysis. Different surgical solutions have been applied for each case. Hypoplasia was not only restricted to the penile urethra but also affected the corporeal bodies of the penis with variable degrees. In two cases, only a single corporeal body could be identified. In another case, the three corporeal bodies were present but appeared distorted (irregularities and interruption of corporeal bodies). Conclusion In our present study, pelvic MRI examination clearly unveiled significant degrees of corporeal dysgenesis among cases diagnosed as Y-type urethral duplication that would strongly suggest these cases to belong to the same disease spectrum of penile dysgenesis/agenesis.

Abstract Background Sacrococcygeal teratoma is a rare tumour, usually presenting in the neonatal period. The benign nature of most tumours and the high survival rates would emphasise on the importance of both cosmetic and functional outcomes. We report on our extended experience with more cases concentrating on the aesthetic outcome of vertical wound closure following excision of large irregular sacrococcygeal tumours. The study included primary cases of sacrococcygeal teratoma who were referred to our surgical team for excision. Cases of presacral tumours associated with anorectal anomalies and sacral bony defects (Currarino triad) were excluded. In all cases, we planned for a vertical midline wound closure after tumour excision. The aesthetic outcomes are evaluated concerning the vertical midline scar, buttock’s contour, and position of the anus. Results In addition to twelve previously reported cases (during the period 2011 through 2016), we included another ten new consecutive cases operated during the period 2017 through 2021. Collectively, the study included 22 cases of sacrococcygeal teratoma that underwent vertical perineal wound closure after excision of the tumour. In 13 cases (those with relatively small or medium-sized tumours), the perineal wound was perfectly closed in the midline (well-hidden vertical scar in the natal cleft). For the rest of the cases (9 cases with large and/or irregular sacrococcygeal tumours), some modification was applied on the vertical linear mid-line skin closure to accommodate for skin redundancy and irregularity at the lower end of the wound, usually ending with an ‘inverted-Y’ skin closure Conclusion Vertical wound closure was always feasible after excision of sacrococcygeal teratomas. Even with large and irregular tumours, the vertical scar was perfectly or partially hidden within the natal cleft. Usually, there was adequate buttock development with minimal disturbance to the normal anal location within the perineum.

Abstract Background The colon is among the best options to substitute the oesophagus; it is well known for its durability and good function that makes it most suitable for paediatric patients. The steps of the procedure, postoperative complications, and outcome assessment were thoroughly discussed in previous reports. However, in this report, we have tried to focus on one basic and essential step of the operation, which is fashioning of the colonic flap used to substitute the oesophagus. Results The study included 50 consecutive paediatric cases who underwent colonic replacement of the oesophagus during the period 2010 through 2020. The indication for oesophageal replacement was either oesophageal atresia (27 cases) or corrosive strictures (23 cases). Our standard technique was using a middle segment of the colon (transverse colon) based on the left colic vessels (vascular pedicle) after ligation of middle colic vessels. Variations of the middle colic vessels were encountered that included single, double, or absent vessels. In a single case (2%), the middle colic vessels were multiple, short, and non-branching with interrupted continuity of marginal vessels at that point. In the latter situation, we had to use a different technique by fashioning a right colonic flap based on the middle colic vessels. Conclusion In colonic replacement of the oesophagus, preparing a pedicled flap from the transverse colon based on the left colic vessels was almost always feasible owing to the stable collateral marginal vessels. On a rare occasion, the marginal vessels were interrupted by disturbed anatomy of the middle colic vessels when we had to shift to another technique using a right colonic flap.

Abstract Background Children are frequently referred to the pediatric radiology department due to complaints related to the urinary tract. Main body We aimed to describe common clinical scenarios in paediatric urology practice and their recommended imaging diagnostic algorithms. Practical imaging approach to following common clinical scenarios and the common radiological findings are discussed: (A) perinatal urinary tract dilatation and other congenital anomalies; (B) recurrent urinary tract infection; (C) enuresis and daytime urinary incontinence; (D) abdominal masses; (E) flank pain; (F) hematuria; (G) trauma; (H) nonpalpable testis, ambiguous genitalia and common urogenital sinus anomalies; and I) renovascular hypertension Conclusions Imaging investigations should be tailored according to the clinical presentation in a stepwise approach aiming for optimum patients’ care.

Abstract Background Redo surgery for anorectal anomalies (ARA) may be considered a special category of reconstructive surgery with less predictable outcomes. In this report, we studied anatomical derangements in a group of boys following a previously complicated PSARP procedure, in addition to the effect of reoperation on rectifying this distorted anatomy. Results The study included 27 boys who were re-operated after a previous complicated PSARP. Included cases were divided into two groups: group A (14 cases) was referred before colostomy closure with an obviously complicated primary operation, and group B (13 cases) was referred with delayed complications after colostomy closure. Pelvic MRI examinations were performed before reoperation in 19 cases. In nine of these cases, a repeat MRI examination was performed at follow-up after reoperation to study the effect of redo surgery on rectifying the distorted anatomy. Abnormal wide anorectal angle and wide pelvic hiatus were common anatomical derangements after a previously complicated PSARP. An important goal of reoperation was reconstruction of the levator ani behind the anorectum trying to create a more acute anorectal angle and a narrower pelvic hiatus. The success of this corrective step was evaluated by MRI comparing pre- and postoperative measurements that showed a favourable decrease in the values of anorectal angle and hiatal/PC ratio. Improvement of faecal continence was documented after reoperation in 8 out of 10 cases in group B. Conclusion A wide pelvic hiatus was a frequently encountered postsurgical complication after failed PSARP that has most probably resulted from poor reconstruction of the pelvic floor at time of the primary repair. Re-approximation of the split halves of levator ani in the midline behind the anorectum at reoperation can help to correct the distorted internal anatomy and improve bowel control in these cases.

Abstract Background Infantile haemangiomas (IH) represent a common benign vascular tumour affecting the paediatric population. Infantile haemangiomas are characterised by a natural history differentiating it from other vascular anomalies. After a transient proliferative phase in early infancy, the tumour passes through a plateau phase before going into spontaneous involution. In this report, we tried to share our experience over the last 5 years in managing cases presenting with IH at a specialised vascular anomaly clinic. Main body of abstract This report included cases of IH who were attending the vascular anomaly clinic during the period 2015 through 2019. Data of all patients attending the clinic were retrospectively examined. Files of 103 cases with IH were available for review. The diagnosis of IH was usually straight forward owing to the typical history and characteristic findings at clinical examination. A significant female predominance was noticed. Generally, IH were more common in the head and neck region (70%). Active intervention was necessary in specific situations (eye occlusion, airway involvement, large lesions with skin ulcerations). Whenever intervention proved to be necessary, propranolol was chosen as the first line of treatment with a favourable response detected in about 90% of cases. Surgery was still a valid option (6%) for lesions amenable to resection; however, we must put in consideration that most lesions will spontaneously regress. Conclusion Infantile haemangiomas are common benign vascular tumours of infancy with relatively few complications. Cosmesis is a major concern especially for lesions affecting the face. Propranolol can induce tumour regression in most cases, and generally, a favourable outcome can be anticipated.

Abstract Background Although the outcome of hypospadias repair has much improved, repair of a small-sized phallus is still challenging. Whether preoperative hormonal therapy is beneficial remains controversial. In this report, we prospectively conducted a clinical trial comparing two groups of patients regarding the effect of preoperative topical testosterone application on the outcome of primary hypospadias repair. Results The study included 40 cases of primary hypospadias with a small glans. Their age ranged from 6 months to 3 years. Patients were divided into two equal groups. Group A were treated by local application of testosterone topical gel 1% for 30–40 days and stopped one month before operation, while cases in group B did not receive preoperative hormonal treatment. Genital skin biopsies were taken for histopathological examination. For group A, penile measurements were documented before and after hormonal therapy. There was a significant increase in all penile parameters after hormonal stimulation in group A. Interestingly, the ventral penile length proximal to the meatus showed more growth in response to hormonal stimulation compared to ventral penile length distal to meatus. We did not find significant difference between both groups regarding the rate of postoperative complications. Also, the cosmesis and parent satisfaction were ‘more or less’ similar. On the histopathological level, we could not detect a difference between both groups regarding micro-vessel density using CD31, which is a vascular marker indicating neovascularization supposed to play a fundamental role in the healing process. Conclusion The use of preoperative topical testosterone significantly increases the size of the hypospadiac phallus. However, this increase in size appears to be disproportionate on the ventral penile surface and has not been reflected on improving the surgical outcome. Level of evidence This is a case control study (level III evidence).

Abstract Background Classifying anorectal anomalies (ARA) has always been a matter of debate among pediatric surgeons. A new classification does not necessarily imply discarding older ones. Several concepts have been introduced in the past and stood the test of time being still valid up till now In this report, we present our experience in managing a group of boys with ARA by PSARP. We have tried to enhance the current classification to include information about the level of the rectum in relation to sacrum, not just the location of the fistula. Results The study included 62 consecutive male patients with anorectal anomalies who underwent PSARP between 2009 and 2019. Included cases were either recto-bulbar fistula (21 cases), recto-prostatic fistula (30 cases), or imperforate anus without fistula (11 cases). Their age at operation ranged from 3 to 36 months (mean 7.8 months). Cases with recto-prostatic fistula were further subclassified according to the level of distal rectum into two subgroups: (type 1) those with the rectum ending opposite the level of S4/S5, and (type 2) those with the rectum ending at a higher level opposite S3. The PSARP procedure proved to be a successful surgical approach to reach, separate, and mobilize the rectum in all cases of recto-bulbar fistula, imperforate anus without fistula, and recto-prostatic fistula type (1). In cases of recto-prostatic fistula type (2), the perineal approach (PSARP) failed to reach and mobilize the rectum in two out of the eleven cases. Conclusion In management of anorectal anomalies, the sacrum can provide two important indicators: a prognostic value for continence, and anatomical landmark to stratify the level of distal rectum in the pelvis which is crucial for planning the best surgical approach.

Abstract Background Early genital reconstruction may be recommended in cases of congenital adrenal hyperplasia to avoid the stigma that can affect these individuals with variable degrees through different cultures. However, the separation and mobilization of a high vagina has remained challenging with less satisfactory surgical outcomes. Therefore, the value of early vaginal reconstruction has been questioned in favour of delaying the whole repair after puberty. In this report, the author has adopted the third option in-between, which comprises early reconstruction of the external genitalia and delaying the more challenging vaginal reconstruction (if needed) to be performed after puberty. Results The study included ten consecutive cases of CAH who underwent feminizing genitoplasty during the period 2016 through 2019. Their age at operation ranged from 8 to 84 months (mean 31; median 15). In five cases (50%), the technique of limited urogenital sinus mobilization adopted in this report succeeded in bringing the vaginal introitus down to the perineum. Those cases had originally a low vagina. In the rest of cases, labial retraction showed a common but wide urogenital introitus perfectly lined by urogenital mucosa. The outcomes have been considered satisfactory to a great extent for both parents and doctors regarding cosmesis and lack of functional complications (voiding problems). Longer follow up is still needed to assess the sexual function when these girls become sexually active. Conclusion In cases of congenital adrenal hyperplasia, reduction clitoroplasty combined with partial urogenital sinus mobilization can achieve predictable and satisfactory outcomes. In about 50% of cases, this approach is sufficient to bring the vagina down to the perineum. In the other half of cases with higher vagina, the possibility of satisfactory sinus intercourse can be studied when these girls grow and become sexually active; otherwise, a delayed vaginal reconstruction may turn to be necessary.

Sirolimus use in vascular tumors is limited. We present two cases of refractory vascular tumors with impressive response to sirolimus, one with refractory kaposiform hemangioendothelioma with rapid reversal of thrombocytopenia and heart failure and the other with advanced beard distribution hemangioma with improvement of respiratory distress after prolonged respiratory support.

“Cloaca” is a term used to describe an anomaly in the female where a single orifice is located in the perineum draining both urogenital and gastrointestinal tracts. Few reports used the same term “cloaca” to describe the counterpart anomaly in the male. We present two “male” cases of anorectal anomalies associated with significant penile deformity (caudally displaced penis) that were managed during the period between January 2010 and September 2021. Characteristically, both cases had a single “central” perineal orifice. The latter was located anterior to the predestined site of the normal anus and just beneath a caudally positioned hypospadiac phallus. The caudal displacement of the penis was strikingly obvious by the presence of severe form of penoscrotal transposition. Both cases were associated with a perineal swelling (hamartoma) just beside the central perineal orifice. The urethra was very short (like that in the female), besides the single perineal orifice, which makes the presentation very similar to cloacal anomalies.

Abstract 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).

Abstract Background Recto-perineal fistula is a common anorectal anomaly, affecting both males and females. The anus is abnormally anteriorly located and is usually stenotic. Management of recto-perineal fistula is a dialectic problem. The most common presenting complaint is constipation; also, long-term follow-up of these patients revealed high incidence of postoperative constipation. Results The study included 30 patients (19 female and 11 male). Their age at time of repair ranged from 3 to 78 months. We compared the pre- and postoperative constipation rate after limited posterior sagittal anorectoplasty (PSARP) using Krickenbeck score. Overall, the rate of constipation has dropped from 77 (23/30) to 30% (9/30) after surgery. Fourteen patients out of 23 patients with preoperative constipation were cured. Another patient with preoperative constipation improved regarding the grade of constipation. The remaining 8 patients with preoperative constipation did not show improvement after operation. These results were statistically significant (McNemar test). Out of the 9 patients who were still constipated after surgery, 7 had associated fecal soiling that showed good response with bowel management of constipation. Conclusion Limited posterior sagittal anorectoplasty can have a role in treating constipation among cases of recto-perineal fistula.

Infantile hemangiomas (IHs) are common vascular tumors. In most cases, a benign course with favorable outcome can be anticipated. IH typically present as cutaneous lesions either with a localized or diffuse segmental distribution. Segmental hemangiomas in the face may be associated with brain and cardiac anomalies (PHACES syndrome), whereas airway involvement has been reported to be associated with hemangiomas in the “beard” area. Multiple cutaneous hemangiomas may be associated with visceral hemangiomas (commonly in the liver). In this report, we present a new association where deep paravertebral hemangiomatous lesions were observed to be associated with cutaneous back hemangiomas in two consecutive cases.

Venous malformations represent a major sector of vascular anomalies. Most cases are asymptomatic or subclinical; however, large extensive lesions can cause severe disability and sometimes mortality. In this report, we present a successful case of sirolimus treatment in managing an extensive venous malformation in the pelvis of a 21-month-old boy who presented with life-threatening complications. With a history dating since the day 2 of life, the patient suffered from chronic bleeding due to scrotal skin ulcerations, in addition to recurrent attacks of severe bleeding per rectum necessitating hospital admission and blood transfusion (three attacks since the age of 7 months). Pelvic magnetic resonance image showed the typical findings of extensive venous malformation involving the pelvis, perineum, scrotum, and extending to the gluteal region. The lesion was seen totally encasing the anorectum with marked thickening of their walls almost occluding their lumen. Oral sirolimus (2 mg/m2) was started with a target blood trough level of 5 to 10 ng/mL. Over a follow-up period of 5 months, there was obvious clinical improvement that included healing of skin lesions (scrotal ulcer) with complete re-epithelialization, absence of bleeding per rectum with improvement of constipation, and rise of hemoglobin level from 7.5 to 11.5 g/dL.

Currarino triad is a rare syndrome that may be occasionally encountered during managing cases of anorectal anomalies. The triad consists of anorectal anomaly, sacral bony defect, and a presacral mass. It may be familial or sporadic, with a reported female predominance. Identification of the characteristic notched sacrum (sacral scimitar) in plain X-ray (anteroposterior view) is considered the key for the diagnosis; however, not infrequently, this radiological sign is overlooked, especially with a small sacral defect. Excision of the presacral cyst is usually performed concomitantly during anorectoplasty. The prone position is the standard approach for posterior sagittal anorectoplasty (PSARP) in males; however, in females, the supine position can be used as an alternative (anterior sagittal anorectoplasty). In this case report, excision of the presacral cyst took place in two steps: the first excision during the PSARP procedure in the prone position, and a second operation in the supine lithotomy position to remove a residual component of the lesion that was missed during the primary operation. It was clear that the supine lithotomy position provided better access to explore the presacral space than the prone position, especially with a deeply located cyst as in our case. The role of magnetic resonance imaging (MRI) in the identification of the exact nature and extent of the lesion before surgery is crucial and should be performed in all cases.

Complete colonic duplication is rare, and usually occurs as a part of the caudal duplication syndrome. In such cases, the diagnosis is clinically evident by the presence of two ani arranged side by side in the perineum, which is commonly associated with duplication of the external genitalia as well (double phallus or double vestibule). In this report, we present a special case of anorectal anomaly that was associated with complete tubular colonic duplication. The diagnosis was initially missed due to the uncommon sagittal arrangement of duplicated rectum: one rectum was ending externally into the perineum by rectoperineal fistula, while the other was hidden by its internal termination into the vagina. Our final diagnosis for this case was a variant of anorectal anomaly in the female, which was associated with complete colonic duplication. One colon (which was in the free mesenteric border) terminated anteriorly into the vagina as a part of a “short common channel” cloaca, while the other colon terminated by rectoperineal fistula. Although the anomaly seems to be rather complex and confusing, yet our case was associated with an excellent outcome due to the benign type of anorectal anomalies (rectoperineal fistula and “short common channel” cloaca) and absence of significant sacral dysplasia; in addition to adequate identification of the abnormal anatomy by appropriate investigations and the staged approach for surgical reconstruction.

Aim: The aim of this article was to describe our experience with 14 patients with double urethra. Patients and methods: We retrospectively examined the patients’ records including their clinical presentations, investigations, operative findings, and outcome. In addition to Effmann’s classification, we used a newly proposed classification that depends on the orientation of the double urethral channels. Results: During the last 15 years, 18 patients were diagnosed to have double urethra at our pediatric surgical unit. We excluded four patients with ‘Y-type’ urethral duplication. The remaining 14 patients were divided into either sagittal or collateral duplication. Their age at presentation ranged from the neonatal period to 9 years. The sagittal urethral duplication included 12 male patients. All patients had two urethral channels, one above the other. The dorsal urethral channel was always characterized by poor function and ectopic course. The ventral channel was always the more functioning urethra, with a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). The collateral urethral duplication included two patients. Both patients were associated with duplication of the urinary bladder and the external genitalia as a part of caudal duplication syndrome. In this group, both urethrae had comparable function, lying side by side, and each draining a separate urinary bladder. Conclusion: The double urethra is a diverse spectrum comprising different pathologies. Our proposed classification system of duplicated urethras is clinically relevant as it guides surgical management and allows prognostication of outcome.