Posterior Sinuplasty: A New Strategy for Managing Hydrocolpos in Cloaca-Case Series.

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.

Management strategy and outcomes of sacrococcygeal teratoma - an Egyptian multicenter experience.

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.

De-epithelialized overlap flap to secure urethroplasty in second stage hypospadias repair: revisiting the Smith technique.

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.

Postnatal imaging of conjoined twins: a customized multimodality approach.

Medical imaging plays a crucial role in the management of conjoined twins. The role of imaging is to explore the anatomy, outline the shared organs and determine whether surgery is feasible. It also serves as a roadmap for successful separation. Additionally, imaging helps with counseling parents about prognosis. This review aims to illustrate recent advances in different imaging modalities and their role in the management of the various types of conjoined twins with an emphasis on relevant tips for optimal imaging.

Urethral Plate Substitution in Two-Stage Hypospadias Repair: Grafts Versus Flaps.

BACKGROUND: Choosing between flaps or grafts to substitute the urethral plate in 2-stage hypospadias repair has been a matter of debate with no consensus in the literature. Flaps have reliable blood supply that maybe theoretically less liable for strictures or contractures. Grafts are more versatile, which can be used in primary and recurrent cases when healthy local skin is deficient.In this report, our aim was to compare outcomes of grafts and flaps when used to substitute the urethral plate in two-stage repair of primary proximal hypospadias with ventral curvature. METHODS: This retrospective study included primary cases of hypospadias with significant curvature who underwent two-stage repair using either grafts or flaps to substitute the urethral plate in the first stage. Cases included in the study were divided into two groups according to the technique of substituting the urethral plate at the first stage of repair. During first part of the study period (2015 through 2018), we mostly used grafts to substitute the urethral plate (group A); later, we shifted to skin flaps (Group B) during the period 2019 through 2021. RESULTS: The study included 37 boys with primary proximal hypospadias who underwent two-stage hypospadias repair. The meatus position was penoscrotal in 18, scrotal in 16, and perineal in three. Inner preputial graft was used to substitute the urethral plate in 18 cases (group A), while dorsal skin flaps were used in 19 (group B). Out of the 37 cases, 27 were available at follow-up after second stage (group A = 14; group B = 13). Follow up period ranged between 6 and 42 months (mean 19.7; median 18.5). Overall, 14 cases required reoperations for different indications: partial disruptions of distal part of the repair in six, closure of urethro-cutaneous fistula in six, and urethral strictures in two. The rate of complications was higher in group A (10 cases: 71%) compared to group B (4 cases: 31%) (Fisher exact test, p-value = 0.057). CONCLUSION: Grafts were associated with higher complication rate than flaps when used to substitute the urethral plate in two-stage repair of proximal hypospadias with chordee. TYPE OF STUDY AND LEVEL OF EVIDENCE: This is non-randomized comparative study (level III evidence).

External phallopexy: A revisited technique and algorithm for simple management of buried penis.

INTRODUCTION: Buried penis (BP) is a distressing condition for both the child and his caregivers. A lot of techniques are available for the management of such condition. External phallopexy represents a simple technique designed for selected cases. This study aimed at reexploring the technique of "3 stitches phallopexy" in the context of correction of selected cases with BP, validating a prospectively designed algorithm for BP management, and detecting the parental satisfaction and possible complications of this technique. METHODS: This was a prospective study performed over a 2 years' period on cases diagnosed with BP. Patients were excluded if they have previous urethral repair e.g., hypospadias or epispadias, BP with normal penopubic angle, micropenis, and insufficient skin coverage after phallopexy simulation test. A questionnaire with 0-12 points score was used for assessment of satisfaction by caregivers. RESULTS: 28 cases were included, with a mean age at the procedure of 5.03±2.6 years. The mean BMI was 15.25±1.1. The intraoperative flaccid penile length measurement was 4.74±1.62 cm, and the mean gained extra-length with phallopexy simulation test was 1.8±0.63 cm. The mean operative time was 20±7 minutes. No wound infection was noted. 2 cases were concerned about the stitch marks at the penopubic junction skin. No recurrence of symptoms was reported after at least 6 months (mean11±4 months) follow up. The mean preoperative satisfaction score was 4.7±1.2, while at the 6 months follow up visit it rose to 10.8±0.67. CONCLUSIONS: External phallopexy represents a simple technique for managing buried penis, with minimal complications during follow up period and satisfactory cosmetic outcome.

Cloaca-Like Anomalies in the Male: A Report on Two Cases.

"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.

Autologous platelet gel improves outcomes in tubularized incised plate repair of hypospadias.

BACKGROUND: hypospadias is one of the most widespread male congenital anomalies, occurring in 1:250 to 1:300 live births. Several repair techniques have been developing to improve the outcomes. PURPOSE: a randomized prospective controlled study was adopted to evaluate effectiveness of autologous platelet gel in healing promotion and improving the outcomes of hypospadias repair. METHODS: thirty children who aged between 6 months and 12 years were recruited and subdivided into two groups; group A had tubularized incised plate (TIP) repair with autologous platelet gel application and group B had TIP repair without autologous platelet gel. RESULTS: there was no significant difference in duration of operation between both groups. All patients in groups A and B had slit-like meatus shape in the distal glans. While all those of group A had one urine stream, yet only 11 of group B had one. There were complications that happened exclusively in group B such as spray stream (27%) and fistula (20%). Whereas other complications occurred insignificantly more in group B than in A including meatal stenosis (53 versus 27%), glans dehiscence, (20 versus 7%), bleeding (33 versus 13%), infection (33 versus 27%), edema (27% versus13), respectively. The incidence of skin necrosis was equal in both groups. CONCLUSION: autologous platelet gel usage in TIP hypospadias repair can be a reliable technique to promote wound healing, and to limit of postoperative surgical complications.

Posterior Mediastinal and Cutaneous Back Hemangiomas in Infants: A New Association.

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.

Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.

Congenital haemangioma (CH) is a rare benign vascular tumour presenting at birth with excellent prognosis. Usually, CH regresses without treatment within the first few months of life. Kaposiform Haemangioendothelioma (KHE) is another type of vascular tumours that has been described as benign with locally aggressive potential. Although the diagnosis of vascular tumours is usually straightforward based on typical clinical presentation, yet some confusing similarities may exist with congenital sarcomas.Conclusion: Data of cases managed at the vascular anomaly clinic during the period 2015 through 2019 were retrospectively analysed. The study included three groups of patients: cases diagnosed as congenital haemangioma (9 cases), cases of Kaposiform Haemangioendothelioma who presented in the neonatal period (7 cases), as well as cases of congenital fibrosarcoma (4 cases) that were referred to the vascular anomaly clinic because of apparent similarity with vascular tumours. The hallmark of the study was to compare clinical and imaging features in the three groups to facilitate differentiation and remove diagnostic confusion when managing these rare cases in the future. What is Known: • Congenital haemangioma is a rare benign vascular tumour presenting at birth. • Kaposiform Haemangioendothelioma is another type of vascular tumours that has been described as benign with locally aggressive potential. What is New: • Confusing similarities may exist between vascular tumours and congenital sarcomas.

Anorectal anomalies in the female: Highlights on surgical management.

BACKGROUND: Anorectal anomalies (ARA) are characterized by different clinical presentations in both sexes with consequently different management protocols. There exist several controversies and different strategies to manage ARA in the female. PURPOSE: To present our experience in managing girls with ARA highlighting the rationale behind the chosen protocol. PATIENTS AND METHODS: The study included 121 girls with ARA who underwent sagittal anorectoplasty during the period 2009 through 2019. Their age ranged from 3 to 57 months (median = 7 months). There were 68 cases with rectoperineal fistula, 51 with rectovestibular fistula, 1 case with rectovaginal fistula and another case without fistula. Preoperative colostomy was performed in only 5 cases (3 rectovestibular; 1 rectovaginal; 1 without fistula). The rest of cases underwent delayed primary sagittal anorectoplasty (beyond the neonatal period). RESULTS: Postoperative wound complications were detected in 14 cases (11.7%). In general, wound complications were more common among the rectoperineal group. Delayed healing problems in the form of mucosal prolapse/ectropion were detected in 10 cases. Thirty-four cases were available for delayed functional assessment. Voluntary bowel control was present in 91% of cases. Constipation was detected in 7 cases (20%) at follow up. Fecal soiling was present in 6 cases (17.6%); 3 of them were associated with constipation. CONCLUSION: Delayed primary repair of rectoperineal and rectovestibular fistula is feasible with low complications. By adopting this protocol of management, we could achieve comparable results while avoiding unnecessary operations during the neonatal period. LEVEL OF EVIDENCE: This is a case series (Level IV Evidence).

Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation.

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/m 2 ) 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.

Histopathological perspective of the pulled-through colon in Hirschsprung disease: Impact on clinical outcome.

BACKGROUND: The outcome in HD has not been always satisfactory even after a technically sound operation. PURPOSE: To define the characteristic histopathological features of the pulled-through colon in patients with HD, and it is impact on clinical outcome. PATIENTS AND METHODS: The study included patients with HD who underwent surgical repair between 2010 through 2016. The proximal margin of resected bowel segments (which corresponds to the pulled through colon) was subjected to detailed histopathological examination by two experienced pathologists. Based on the frequency of postoperative attacks of HAEC (fever, vomiting, abdominal distention, fluid offensive stools), cases included in the study were divided into two groups: Group A, those with less frequent attacks of HAEC; and Group B, those with recurrent attacks of HAEC (more than 3). RESULTS: The study included 35 patients (25 in group A; and 10 in group B). Their age ranged from 0.2 to 144 months (median 6 months). Comparing the histopathological findings in the two clinical groups, we have found that Group B (recurrent attacks of HAEC) had significantly more frequent focal disarray of nerve bundles and thicker nerve bundle diameter. Also, histopathological features of acute inflammation were more prevalent in examined specimens from group B. CONCLUSION: Several histopathological features of the examined bowel specimens in HD, other than presence or absence of ganglion cells, are indicative of postoperative functional outcome. These include the thickness and maturity of nerve bundles, in addition to the presence of histopathological features of acute inflammation. LEVEL OF EVIDENCE: This is a case control study (level III evidence).

Transformation of the female genitalia in congenital adrenal hyperplasia: MRI study.

PURPOSE: In this report, we aim to define the different degrees of structural abnormality affecting the female genitalia in cases of CAH by using the multiplanar capabilities and high soft tissue resolution of MRI. PATIENTS AND METHODS: The study included cases of CAH who were referred to our pediatric surgical facility for genital reconstruction during the period 2016 through 2019. We studied the pelvic MRI anatomy in cases of CAH while referring to clinical and operative findings. To set up a grading scale for the degree of virilization in cases of CAH, we included another two control groups of normal boys and girls representing the two ends of the spectrum. RESULTS: The study included 23 cases of CAH who underwent preoperative pelvic MRI examination. All cases had normal chromosomal analysis (46 XX). Their age ranged from 1 to 156 months at time of MRI examination (mean 42.4; median 25). The level of the lower end of the vagina was identified in midsagittal T2WI and confirmed in sequential axial cuts. Based on the level of the lower end of the vagina in relation to the pubic symphysis, we classified cases of CAH into either low or high types. Moreover, we could observe a correlation between the degree of vaginal descent and structural transformation of erectile tissue between both genders. CONCLUSION: MRI can have an important role in the evaluation of cases of CAH by displaying the severity of internal anomaly which is crucial for proper preoperative counseling. TYPE OF STUDY: Case control study. LEVEL OF EVIDENCE: Level III.

Colonic replacement of the esophagus: towards standardization of the technique.

Since we have started colonic replacement of the esophagus in children, several successive modifications have led to evolution of the surgical technique aiming to decrease complications and achieve the best functional outcome. Currently, our surgical group has reached a single standardized technique that has been applied to all cases included in this report. A colonic flap (including the transverse colon) is prepared based on the left colic vessels. The proximal end of the colonic flap is transferred upwards passing at first behind the stomach and then anteriorly through the lesser omentum to traverse the thoracic cavity via a previously prepared retrosternal tunnel. The proximal end of the colonic flap is anastomosed to the proximal esophagus in the neck (end to end anastomosis). Most specifically, the distal end of the colonic flap is anastomosed to the posterior wall of the body of the stomach in order to prevent regurgitation of gastric contents into the colonic flap. The retro-sternal colon bypass is among the successful options to replace the esophagus in children. Adding a posterior cologastric anastomosis to the procedure greatly prevents gastric regurgitation that can be responsible for short- and long-term complications. LEVEL OF EVIDENCE: This is a case series with no comparison group (level IV evidence).