Urogenital Anomalies Research Articles

A Practical Approach to Congenital Urogenital Anomalies in Female Pediatric Patients.

Congenital anomalies of the female reproductive tract are relatively common and can be both confusing to understand as well as challenging to diagnose and manage in a busy pediatric clinical practice. Here, we lay out some of the most common genitourinary tract anomalies in female pediatric patients. We highlight the key embryologic development, present case examples, and discuss appropriate testing, treatment, and counseling for patients and their families regarding congenital disorders of the vulva, vagina, uterus, ovaries, and associated pathology. The goal of this review is to demystify these conditions and provide a practical guide for the general pediatrician who is often at the frontline making the initial diagnosis and caring for these patients. [Pediatr Ann. 2020;49(4):e188-e195.].

Mowat-Wilson syndrome in a Chinese population: A case series.

Mowat-Wilson syndrome (MWS) is characterized clinically by a distinctive facial gestalt, intellectual disability, microcephaly, epilepsy, and nonobligatory congenital malformations such as Hirschsprung disease, urogenital anomalies, congenital heart disease, eye malformations. This article summarized the clinical features and molecular findings of 15 Chinese MWS patients. The results revealed a higher incidence of congenital heart disease in Chinese MWS patients compared to that previously reported in Caucasian cohorts, while the incidence of Hirschsprung disease and genitourinary malformation appeared to be lower. This suggests possible ethnicity-related modifying effects in the MWS phenotype.

Long-term follow-up in surgical newborns: A single-institution experience

BackgroundTo assess the actual experiences of long-term follow-up and discuss ways to improve care during the period from childhood to adulthood in newborns who have undergone surgery. MethodsA total of 306 patients with congenital anomalies requiring newborn surgery who survived to discharge from 1994 to 2013 were eligible for inclusion. Survivors with severe chromosomal and cardiac anomalies were excluded. Patients with myelomenigocele, urogenital anomalies and miscellaneous diagnoses were also excluded. Patients with Hirschsprung’s disease were excluded since many of them underwent surgery after the neonatal period. Patients with hypertrophic pyloric stenosis were also excluded since their duration of follow-up was too short for this study. ResultsAccording to the follow-up status, survivors were categorized into 4 groups: under follow-up as an outpatient (UF, n = 67), moved (MV, n = 60), follow-up suspended by doctor (Sus, n = 87), and lost to follow-up (LF, n = 92). The incidence of active medical problems was high, and the duration of follow-up was significantly longer in the survivors with esophageal atresia, congenital diaphragmatic hernia and high-type anorectal malformations than in those with other anomalies. Survivors followed by pediatric surgeons alone, free from active medical problems or free from adverse events during the initial hospitalization were at risk of being LF. ConclusionsMore than 30% of the surgical newborn cases were LF. Disease-specific and standardized multidisciplinary follow-up programs that increase both children’s and parents’ satisfaction and compliance are needed. (230/250 words).

Secuencia de Prune Belly: a propósito de un caso

Antecedentes: La secuencia Prune-Belly es un trastorno genético que se caracteriza por una tríada que incluye deficiente musculatura de la pared abdominal, anomalías urogenitales y criptorquidia bilateral. Suele acompañarse de malformaciones asociadas. La aproximación quirúrgica debe reservarse para aquellos pacientes con complicaciones severas.Resumen del caso: Se presenta el caso de un neonato de sexo masculino con agenesia de la musculatura de la pared abdominal, displasia renal multiquística y criptorquidia bilateral, que es transferido de un hospital básico del oriente ecuatoriano y que durante su hospitalización se le sostuvo con un manejo conservador.Conclusiones: El abordaje de la secuencia de Prune-Belly, debe ser multidisciplinario por su frecuente asociación con otras malformaciones; el diagnóstico prenatal cumple un rol fundamental en la detección precoz de anomalías severas.

Comparison Between Processus Vaginalis Sac Tightening Technique and the Conventional Technique in Orchiopexy Surgery Over 10 Years.

BackgroundUndescended testis (UDT) is a common congenital urogenital anomaly that is treated by orchiopexy. We aimed to introduce patent processus vaginalis (PPV) sac tightening (PVST) technique and compare it to the conventional technique.MethodsWe retrospectively studied all the operated UDT patients during 10 years. In the conventional technique, it was necessary to ligate PPV sac after being peeled off from the spermatic cord. PVST was dissected longitudinally from the two sides of where the PPV sac wall was attached to the spermatic cord till the proximal part, and only a narrow thin layer sticking to the spermatic cord was left and the proximal PVV sac opening was tightened as much as possible with vicryl suture at the internal inguinal ring level. The significance level was <0.05.ResultsOf 821 orchiopexy (mean age 24.5±24.2 months), 36.3% were done by conventional and 63.7% by PVST technique. Hematoma, edema, hydrocele, and wound infection were lower in the PVST technique, but it was not significant (p>0.05). Testicular atrophy and operation time were significantly lower in the PVST than the conventional technique (p<0.001).ConclusionThe orchiopexy PVST technique has lower complications and seems to be easier, faster and safer than the conventional technique.

A Rare Case of Complete Penile Agenesis

Aphallia, or penile agenesis, is rare (1:30 million)1 and associated with other urogenital anomalies.2 Under 100 cases of aphallia have been reported worldwide.1 Pathogenesis is uncertain but thought to be failure of formation of the genital tubercle.2 We present a case of complete penile agenesis with anorectal and urological malformations.

A single centre experience in the management of undescended testes

Introduction: Undescended testes (UDT) is one of the most common congenital anomalies of the urogenital system. In spite of its common occurrence, lots of variations are still reported regarding the time of surgery, mode of investigation and surgery. The present study tried to report the single centre experience of management of UDT. Materials and Methods: The data of patients with UDT including time of presentation, time of surgery, position of testes, investigations, associated urogenital anomalies, complications were collected over a period of 9 years and 6 months and analysed. Results: A total of 118 children were recorded of which the testes of 6 infants became descended before the age of 6 months. Out of the remaining, 112 children, 24 children had bilateral UDT, 24 children had nonpalpable testes (NPT). Ultrasound could locate 19 NPT pre-operatively. Maximum children were operated between 6 months to 1 year in 43 children (38.39 %). Laparoscopy was done for intra-abdominal testes and NPT whose locations could not be located by US and the rest by open inguinal exploration. Four children had pre-operative complications with obstructed inguinal hernias (2 cases) and testicular torsion (2 cases). Conclusion: Majority of the children were being operated before the age of 1 year and US is a good non-invasive to effectively locate the NPT.

Differences between Risk Factors for Sepsis and Septic Shock in Obstructive Urolithiasis.

BackgroundComplicated acute pyelonephritis (APN) is a life-threatening condition that requires immediate intervention. This study examined the characteristics of APN occurring as a complication of ureteral stone.MethodsWe retrospectively reviewed 85 patients diagnosed with APN complicated by ureteral stone between December 2006 and July 2017 at our institution. Patients with concomitant renal stone, multiple ureteral stones, ureteral strictures, ureteral cancer, and urogenital anomalies, including vesicoureteral reflux were excluded. Clinical characteristics including age, sex, underlying disease, medical history, stone characteristics, initial laboratory data, and the procedure used to correct urinary obstruction were summarized, and the risk factors associated with sepsis and septic shock were analyzed.ResultsSepsis was diagnosed at initial presentation in 62 patients, 17 of whom suffered from septic shock. Disease-related death did not occur in any patient. Previous history of stone (P = 0.015), leukocytosis (P < 0.001), elevated C-reactive protein levels (P = 0.006), and low albumin (P = 0.038) were significant risk factors for progression to sepsis. The absence of hypertension (P = 0.047), thrombocytopenia (P = 0.006), decreased erythrocyte sedimentation rate (ESR) (P = 0.003), elevated blood urea nitrogen (P = 0.016), and positive blood culture (P = 0.018) were significant predictors for progression to septic shock. Multivariate analysis revealed that previous history of stone (P = 0.015) was an independent risk factor for sepsis, while the absence of hypertension (P = 0.047), thrombocytopenia (P = 0.013), and decreased ESR (P = 0.009) were risk factors for shock.ConclusionThe risk factors associated with the progression from APN to sepsis differed from those associated with the progression from sepsis to septic shock. Various factors should be considered while selecting treatment options based on the severity of APN associated with ureteral stone. It should be managed with aggressive treatment and close observation, especially in the presence of risk factors.

Severe thoracolumbar congenital kyphosis treated with single posterior approach and gradual "in situ" correction.

Congenital kyphosis results from the failed formation of the vertebrae during the embryonic period and may be associated with cardiac, urogenital, or spinal cord anomalies. Surgical treatment is the best choice through anterior, posterior, or both approaches. This study aims to evaluate the effect of posterior gradual correction using the "in situ" bender to correct severe thoracolumbar congenital kyphosis with or without osteotomy or excision of the vertebra. Twenty-five patients with an age range of 2-23 (mean ± SD = 12.58 ± 6.03) years with severe thoracolumbar kyphosis were treated surgically at our institution between 2004 and 2013. Pedicle screwing, osteotomy, and gradual "in situ" bending through the single posterior approach were the choices of treatment. Cobb's angle, patient's height, and SRS-22r were used to evaluate the patients preoperatively and postoperatively. Follow-up periods were 35-136 months. Cobb's angle pre-surgery range was 35-180 (81.48 ± 39.1) degrees improved post-surgery to 0-45 (21.72 ± 13.47) degrees (p-value <0.0001). The range of patients' standing height pre-surgery was 79-170 (142.42 ± 24.85) centimeters increased after surgery to 81-175 (147.76 ± 26.33) centimeters (p-value <0.0001). SRS-22r pre-surgery range was between 2.12 and 3.904 (3.2 ± 0.77) and improved post-surgery to 4.16 and 4.96 (4.59 ± 0.29) (p-value = 0.046). Gradual correction with the "in situ" bender with or without osteotomy through the single posterior approach can give satisfying clinical (patients' standing height and SRS-22r scores) and radiological (Cobb's angle) results to treat severe congenital thoracolumbar kyphosis.

First experience: management of Prune Belly syndrome in Harapan Kita Women and Children’s Hospital

Prune belly is a rare congenital syndrome, consisting of congenital abdominal wall musculature absence, urogenital tract anomalies, and cryptorchidism in male patient. The incidence of this syndrome is 1 in 35,000 to 1 in 50,000 live births and rarely occurs in females (3-5%). One step operation of ureteral re-implantation, orchidopexy, and abdominoplasty is one option of treatment in managing this condition. This was our first experience, involving a multidisciplinary approach. A 30 year old Asian Woman G4P3A0 at 33-34 weeks of gestation was referred to the Urology Department with a fetal ultrasound showing bilateral hydronephrosis and undescended testis. Prune Belly was first suspected when abnormal abdominal circumference was noted by the obstetrician while doing a fetomaternal ultrasound at the 19 weeks gestation. No intervention was undertaken during gestation. The baby delivered spontaneously at 38 weeks gestation. Testes were impalpable and the abdominal contour was suggestive of Prune Belly. CT urography showed bilateral hydronephrosis and hydroureter. Micturating cysto-urethrography showed bilateral vesicoureteral reflux while renogram showing a cortical defect. Serial follow-ip ultrasounds were done showing bilateral hydroureter with 4th grade bilateral hydronephrosis. By the age of 2 the patient underwent one step operation of ureteral reimplantation, orchidopexy, and abdominoplasty. After the operation, the patient recovered with good function and cosmetic result. Prune belly is a rare and complex congenital syndrome. From our experience at Harapan Kita Women and Children’s Hospital, one step operation involving multidisciplinary approach of ureteral re-implantation, orchidopexy, and abdominoplasty has shown a good result. Further follow up is needed to evaluate either this choice of treatment could improve patient outcome.

Loss-of-Function Variants in PPP1R12A: From Isolated Sex Reversal to Holoprosencephaly Spectrum and Urogenital Malformations

In two independent ongoing next-generation sequencing projects for individuals with holoprosencephaly and individuals with disorders of sex development, and through international research collaboration, we identified twelve individuals with de novo loss-of-function (LoF) variants in protein phosphatase 1, regulatory subunit 12a (PPP1R12A), an important developmental gene involved in cell migration, adhesion, and morphogenesis. This gene has not been previously reported in association with human disease, and it has intolerance to LoF as illustrated by a very low observed-to-expected ratio of LoF variants in gnomAD. Of the twelve individuals, midline brain malformations were found in five, urogenital anomalies in nine, and a combination of both phenotypes in two. Other congenital anomalies identified included omphalocele, jejunal, and ileal atresia with aberrant mesenteric blood supply, and syndactyly. Six individuals had stop gain variants, five had a deletion or duplication resulting in a frameshift, and one had a canonical splice acceptor site loss. Murine and human in situ hybridization and immunostaining revealed PPP1R12A expression in the prosencephalic neural folds and protein localization in the lower urinary tract at critical periods for forebrain division and urogenital development. Based on these clinical and molecular findings, we propose the association of PPP1R12A pathogenic variants with a congenital malformations syndrome affecting the embryogenesis of the brain and genitourinary systems and including disorders of sex development.

Colpolithiasis: A mini review

Objective: Vaginal calculus is an unusual finding. In this study we describe a case of primary vaginal calculus in a rather rare urogenital anomaly- Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome. This syndrome is shrouded in controversies. Here we present a mini review about vaginal calculus and theory of embryological development of female genital tract as related to OHVIRA syndrome. Case: A 50 years-old unmarried lady with primary amenorrhoea presented with pain abdomen. She was found to have calculus in the pelvis by x-ray which was thought to be bladder calculus initially. On local examination the vaginal opening was absent. Imaging studies such as NCCT and MRI confirmed that it is a vaginal calculus, located below the uterine cervix, with absent right kidney. She was diagnosed with OHVIRA syndrome with colpolithiasis. On laparotomy she was detected to have unicornuate uterus with absence of right Mullerian structures. The calculus occupied the whole of upper part of vagina, which was a blind pouch. The patient underwent total abdominal hysterectomy, left salpingo-ophorectomy with removal of calculus after opening the vaginal vault. Postoperative recovery was uneventful. Chemical analysis of the calculus revealed calcium magnesium oxalate which is of hematic origin. Keywords: Primary vaginal calculus, Mullerian duct anomaly, Obstructed hemivagina, OHVIRA syndrome, Herlyn-Werner-Wunderlich syndrome.

The use of fetal MRI for renal and urogenital tract anomalies.

Fetal anomalies are detected in approximately 2% of all fetuses and, among these, genitourinary tract abnormalities account for 30% to 50% of all structural anomalies present at birth. Although ultrasound remains the first line diagnostic modality, fetal MRI provides important additional structural and functional information, especially with the development of faster sequences and the use of functional sequences. The added value of MRI-based imaging is three-fold: (a) improvement of diagnostic accuracy by adequate morphological examination, (b) detection of additional anomalies, and (c) in addition, MRI has the potential to provide information regarding renal function. In this review, we describe the role of fetal MRI in the anatomical evaluation of renal and urogenital tract anomalies, and we also touch upon the contribution of functional MRI to the diagnostic workup of these conditions.

Herlyn-Werner-Wunderlich syndrome presented with amenorrhea: A rare urogenital anomaly in females

A 13-year-old prepubertal girl had with primary amenorrhea and severe lower abdominal pain. Pelvic ultrasound showed double uterine cavity and a fluid collection in left uterine cavity with diffuse low level internal echoes, which appeared contiguous with the endocervix. Hymen could not be evaluated because the patient could not be taken to the gynecological table the patient was initially diagnosed as imperforate hymen, gynecologic examination was performed with sedation, and hymen has a normal patency. Magnetic resonance imaging (MRI) revealed two uterus and two cervix, normal right endometrial cavity and 17 mm fluid in the left endometrial cavity and compatible with hematometra. Hematocolpos was observed in the left half of the vagina. There was no left kidney and the right kidney was normal. Based on these findings, the patient was diagnosed as Herlyn-Werner-Wunderlich syndrome and vaginal septum resection was performed as treatment. Evaluation of the genital tract by means of MRI scanning or ultrasonography should be recommended in all girls with known renal abnormalities before the onset of menstruation.

Long-term Outcome of Children with Ureteropelvic Junction Obstruction: Thirty-one years’ Experience at a Tertiary Teaching Hospital in South Africa.

B ackground and Aim: Being the most common pathologic cause of hydronephrosis in children, we characterized and evaluated the long-term outcome of Ureteropelvic junction obstruction (UPJO) at a tertiary hospital in South Africa Methods: Children confirmed to have UPJO between 1985 and 2016 were characterized based on demographic and baseline clinical data. Long term outcomes were need for surgical intervention, loss to follow up rates, anthropometric measures, renal outcomes (Glomerular Filtration Rate and Blood pressure). Results: Of 107 children, 74.8% were male, 47% had hydronephrosis identified antenatally, 47.7% had the left kidney unilaterally affected, 31.8% had an additional urogenital anomaly, 19.6% presented with an abdominal mass, and 37.4% had a urinary tract infection. On enrolment, 54.2% and 30.8% had normal systolic and diastolic BP, 59.8% had normal BMI for age and 72% had normal length/height for age. The median follow-up time was 35(9.0 - 191.0) months, 65% had surgery with a median time to surgery of 2 (0 - 6.8) months. Children lost to follow-up had a higher proportion of extrinsic causes of UPJO (12.0% vs. 0%, P =0.041) and other urogenital anomalies (38.7% vs. 15.6%, P=0.019). There was no significant effect of time or surgical intervention on mean BMI and height for age, blood pressure percentile and eGFR for age. Conclusion: In our setting, UPJO has an early presentation, with an early time to surgery. Long-term outcome is favorable, but loss to follow-up presents a significant drawback that needs to be addressed. Keywords: Ureter; Hypertension; Congenital anomalies of kidney and urinary tract; CAKUT; Urological Diseases; Hydronephrosis.

Birth Anthropometry Characteristics of Hypospadias Patients in Palembang, Indonesia

ABSTRACT.&#x0D; Background. Hypospadias is one of the most common urogenital anomaly in males. The etiology is poorly understood, but is believed to be multifactorial where Intra Uterine Growth Restriction (IUGR) is one of the suspected risk factors, leading to low birth weight and low birth length in affected patients.&#x0D; Objective. This study aims to identify the birth anthropometry characteristics of hypospadias patients who visited RSUP. Dr. Mohammad Hoesin Palembang.&#x0D; Materials and Methods. This was an observational descriptive study with cross sectional design. This study included hypospadias patients with detailed birth characteristics in RSUP Dr. Mohammad Hoesin Palembang during the period of 2012-2015.&#x0D; Results. Out of 106 hypospadias patients, only 19 (17.92%) of them presented with detailed birth characteristics. There were 12 (63,2%) patients with low birth weight and 7 (36,8%) patients with normal birth weight. There were 10 (52,6%) patients with low birth length and 9 (47,4%) patients with normal birth length. The mean birth weight is 2442 grams and the mean birth length is 46,9 centimeters.&#x0D; Conclusion. The majority of hypospadias patients in this study showed a low birth weight and low birth length, signalling an accordance to the existing theory.&#x0D; &#x0D; Keywords: Hypospadias, birth anthropometry, characteristics

The embryology of persistent cloaca and urogenital sinus malformations.

Cloacal malformations are characterized by the confluence of the lower urinary tract, the female reproductive tract, and the rectum to create a common channel with a single opening on the perineum. The presence of a cloaca is a normal phase of early human embryological development. Between the 4th and 7th weeks of gestation, the cloaca undergoes subdivision to form the hindgut and urogenital sinus. Failure of this process results in the congenital anomaly termed persistent cloaca (PC). The term urorectal septum malformation sequence (URSMS) is also used to describe this anomaly. The classic description of this process which is still cited in many standard textbooks dates from the 19th century. However, this has been increasingly called into question by the findings of studies using modern scientific methodology. Urogenital sinus anomalies are defined by the confluence of the urethra and vagina to form a common channel of varying length with a single perineal opening. In this condition, the anorectal canal opens separately on the perineum. The presence of a urogenital sinus represents a transient phase of the normal development of the lower genital tract in the female fetus. However, the form of urogenital sinus most commonly encountered in the developed world is a feature of disordered sexual differentiation and does not arise simply from the persistence of the anatomical structure which is a feature of normal fetal development.

Molecular cytogenetic characterization of partial monosomy 2p and trisomy 16q in a newborn: A case report.

Trisomy 16q is a rare disorder with severe abnormalities, which always leads to early postnatal mortality. It usually results from a parental translocation, exhibiting 16q duplication associated with another chromosomal deletion. The present study reports on the clinical presentation and molecular cytogenetic results of a small-for-gestational-age infant, consisting of partial trisomy 16q21→qter and monosomy 2p25.3→pter. The proband presented with moderately low birthweight, small anterior fontanelles, prominent forehead, low hairline, telecanthus, flat nasal bridge, choanal atresia, clinodactyly of the fifth fingers, urogenital anomalies, congenital muscular torticollis and congenital laryngomalacia. The last two traits have not previously been reported in any trisomy 16q and monosomy 2p cases. The proband was trisomic for the 16q21→qter chromosomal region with the karyotype 46,XY,der(2)t(2;16)(p25;q21)pat. The chromosomal anomaly was the result of unbalanced segregation of a paternal balanced translocation, 46,XY,t(2;16)(p25;q21). In this case, molecular cytogenetic analysis had a critical role in delineating the proband's clinical phenotype. Although this patient had a 16q21→qter duplication and a 2p25.3→pter deletion, the latter may have had mild phenotypic effects when associated with trisomy 16q. The literature was also reviewed, focusing on cases with the same breakpoints, localizations and clinical features reported in recent years.

1716-P: MODY5 and Serous Ovarian Carcinoma in 17q12 Recurrent Deletion Syndrome

Background: Maturity onset diabetes of the young type 5 (MODY5) is due to a mutation in the hepatocyte nuclear factor 1b (HNF1b) gene on chromosome 17q12. HNF1b mutations have been found in clear cell ovarian carcinomas (CCOC) while non-CCOC express this mutation rarely. 17q12 recurrent deletion syndrome presents with urogenital anomalies, MODY5, neurodevelopmental and psychiatric disorders. We report a patient with 17q12 recurrent deletion syndrome with MODY5, uterine abnormalities and low grade serous ovarian cancer. Clinical Case: The female proband was diagnosed with diabetes at age 12. GAD65, islet cell and insulin auto-antibodies were negative, C-peptide was 2.7 ng/mL. Childhood history included strabismus, depression and chronic transaminitis with unremarkable liver biopsy. Mother’s medical history was notable for single kidney, partially-septated uterus, and diabetes. At age 25, the patient presented with abdominal pain. CT showed pancreatic atrophy, ascites, omental and peritoneal nodularity and calcifications. Tumor markers were normal except an elevated CA-125 of 240 U/mL. Short stature was noted, height 147 cm, BMI 27 kg/m2. Laparoscopy revealed bicornuate uterus, 2 cervices and vaginal septum. She underwent TAH-BSO, lymph node dissection and omentectomy for a stage IIIC low grade serous ovarian carcinoma. Chromosomal microarray analysis of the tumor revealed a pathogenic ∼1.8 Mb loss of 17q12, denoted arr[hg19] 17q12(34477479_36283807)x1. Conclusion: While 17q12 has been described as a susceptibility locus in some ovarian cancers, ovarian cancer predisposition is not a reported feature of MODY5 or 17q12 recurrent deletion syndrome. The disease association reported here suggests that women with MODY5 should have periodic evaluation for ovarian cancer in addition to screening for gut and urogenital tract abnormalities. Patients with diabetes plus urogenital tract abnormalities or 17q12 deletion in an ovarian tumor should have genetic evaluation for MODY5. Disclosure L. Hollar: None. M. Salam: None. P.H. Thaker: Advisory Panel; Self; Clovis. Consultant; Self; AbbVie Inc., Celsion, Tesaro, UpToDate. Research Support; Self; Celsion, Merck &amp; Co., Inc. Speaker’s Bureau; Self; Merck &amp; Co., Inc., Tesaro. J.B. McGill: Advisory Panel; Self; Boehringer Ingelheim Pharmaceuticals, Inc., Gilead Sciences, Inc., Novo Nordisk Inc., Sanofi US. Research Support; Self; Dexcom, Inc., Medtronic, Novartis AG, Sanofi US. Speaker's Bureau; Self; Aegerion Pharmaceuticals, Dexcom, Inc., Janssen Pharmaceuticals, Inc., MannKind Corporation.

Use of small intestinal submucosa for corporal body grafting in cases of epispadias and epispadias/exstrophy complex

Male epispadias is a rare congenital urogenital anomaly in which the meatus is ectopically located along the dorsal midline of the penile shaft. In cases associated with severe curvature, functional and cosmetic outcomes could be accomplished by lengthening the shorter dorsal surface with the use of corporal grafting. Various graft materials have been used in the past for hypospadias repair including tunica vaginalis, dermis, and small intestinal submucosa (SIS). The use of SIS grafting for corporoplasty during epispadias repair has rarely been described in the literature. To report the experience in the management of dorsal corporal body grafting using SIS in children with severe penile curvature due to epispadias. The authors retrospectively reviewed the charts of all patients with epispadias or bladder exstrophy/epispadias complex and severe dorsal chordee (>40°) who underwent epispadias repair with single-layer SIS for corporal body grafting. Clinical variables, surgical technique, and outcomes were analyzed. A total of nine consecutive patients underwent staged epispadias repair with dorsal corporal single-layer SIS grafting (summary figure). Of these, four (44.4%) had primary penopubic epispadias, one (11.1%) had mid-shaft epispadias, and four (44.4%) had bladder exstrophy/epispadias complex. The mean age at surgery was 13.4±6 months. After phalloplasty with SIS grafting, there were no reported complications related to the graft during the post-operative period or follow-up visits. Although traditional techniques for epispadias repair allow some degree of corporal lengthening, they also result in abrupt medial rotation of the corporal bodies leading to torqueing and potential unsatisfactory cosmetic results. In contrast, the authors use single-layer SIS for corporal body grafting, and this study technique results in a more gradual inward rotation thus allowing more anatomical accuracy. Furthermore, an advantage of the use of SIS over other grafting materials is that there is no need to harvest an autologous graft such as tunica vaginalis or dermis. Epispadias repair using single-layer SIS corporal body grafting is an effective, safe, and feasible method, which provides satisfactory cosmesis and correction of dorsal curvature in congenital epispadias in children. Furthermore, a more normal penis appearance, without a decrease in the corporal length or diameter, is achieved with this technique.