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Chordee

Chordee is a congenital anomaly characterized by abnormal curvature of the penis, typically in a ventral (downward) direction, resulting from disproportionate growth or fibrous tethering during embryological development, with the deformity becoming most evident during erection.^[1]^[2] This condition arises from structural imbalances such as short urethral plate, dartos fascia contracture, or corporeal disproportion, though isolated cases without hypospadias are uncommon, affecting fewer than 1% of males.^[3]^[4] While chordee often coexists with hypospadias—a urethral malformation occurring in approximately 1 in 300 male births, where up to 25% of cases involve associated curvature—isolated chordee lacks the misplaced meatus but shares similar etiologies rooted in failed penile straightening during fetal development.^[5]^[4] Symptoms range from mild bending with minimal functional impact to severe angulation exceeding 30 degrees, potentially impairing urination, erection, or future sexual intercourse if untreated; curvature greater than 20 degrees is generally deemed clinically significant, prompting intervention.^[6] Surgical correction, typically performed in infancy or early childhood via techniques like dorsal plication or ventral corporal rotation, achieves high success rates with complication frequencies around 8%, though recurrence or fibrosis can necessitate reoperation.^[7]^[6] The condition's management emphasizes precise intraoperative assessment under artificial erection to ensure straightness, as incomplete correction risks persistent deformity or iatrogenic issues like erectile dysfunction.^[4] Long-term outcomes depend on severity and surgical expertise, with most cases resolving without affecting adult fertility or voiding, though epidemiological trends show rising congenital penile anomalies, including chordee, potentially linked to environmental factors beyond genetic predisposition.^[8]00082-9/fulltext)

Definition and Classification

Definition

Chordee is a congenital anomaly characterized by abnormal curvature of the penis, typically ventral (downward) and most evident during erection due to tethering by fibrous bands or disparate tissue growth on the penile shaft. This condition results from disrupted embryological development of the genital tubercle, where unequal expansion of the corpora cavernosa or presence of dysgenetic dartos fascia prevents straight penile alignment.^[9]^[1] The curvature often manifests at the penoscrotal junction or mid-shaft, stemming from ventral shortening relative to the dorsal aspect, and may involve skin, Buck's fascia, or corporal disproportion without underlying urethral defects in isolated cases.^[10] Chordee frequently coexists with hypospadias, occurring in up to 90% of such repairs, but isolated chordee without hypospadias—where the urethral opening remains at the glans tip—affects approximately 4-10% of penile curvature cases requiring intervention.^[11]^[12] Clinically, chordee is deemed significant when penile deviation exceeds 20-30 degrees on artificial erection testing, as lesser angles rarely impair function, though severe forms can redirect urinary flow or hinder intromission.^[6] Unlike acquired curvatures such as Peyronie's disease, congenital chordee lacks plaque formation and arises purely from developmental asymmetry rather than fibrosis or trauma.^[13]

Types and Severity

Chordee is primarily classified into two categories: that associated with hypospadias, where ventral penile curvature accompanies urethral meatus malposition, and isolated chordee without hypospadias (CWH), characterized by a normally positioned glans meatus but distorted prepuce and curvature due to other tissue abnormalities.^[10] In CWH, which accounts for 4-10% of hypospadias-like presentations, operative classifications further delineate subtypes based on underlying tissue defects identified intraoperatively. One such system divides cases into six types: Type A (cutaneous chordee from skin and dartos tethering, treated by degloving); Type B (fibrous chordee, managed by chordectomy); Type C (corporocavernosal disproportion, corrected via corporoplasty with or without urethral mobilization); Type D (urethral tethering with hypoplastic urethra, addressed by mobilization or reconstruction); Type E (congenital short urethra, requiring excision and urethroplasty); and Type F (complex multifactorial chordee, involving combined techniques).^[10] An alternative etiology-based approach for CWH emphasizes proportions such as skin tethering (32%), fibrotic fascia (33%), corporal body disproportion (28%), and congenitally short urethra (7%), guiding targeted surgical release.^[1] The Devine and Horton classification for CWH grades defects by fascial and spongiosal involvement: Class I (most severe, with corpus spongiosum deficiency from chordee site to glans and thin mucosal strip); Class II (normal spongiosum but abnormal Buck's and dartos fascia); and Class III (isolated dartos fascia abnormality).^[1] Severity of chordee, whether isolated or hypospadias-associated, is assessed by the angle of ventral deviation from the straight axis during artificial erection, as subjective visual estimation shows poor inter-observer reliability beyond extremes (≤15° or >30°).^[14] ^[15] Mild curvature is typically defined as <30°, often not requiring intervention if asymptomatic; moderate ranges from 30° to approximately 60°, and severe exceeds 60° or involves functional impairment like urinary stream deflection or future coital difficulties, with >30° generally prompting surgical correction in proximal hypospadias cases (occurring in 5-10% of instances).^[16] ^[6] ^[17] Curvature grading informs repair complexity, with high-grade (>30°) defects in hypospadias often necessitating dorsal plication or staged urethroplasty, while milder forms may resolve with simple degloving or fascial excision.^[16] ^[18] In hypospadias contexts, chordee severity correlates with meatus location post-release, escalating from mild in anterior (50% of cases) to severe in posterior types (20%), though isolated chordee remains a minor anomaly independent of hypospadias grading.^[18] ^[19]

Epidemiology

Prevalence and Incidence

Chordee without hypospadias, also known as isolated congenital penile curvature, has a reported incidence ranging from 4% to 10% among newborn males, though this estimate derives from clinical series where the penile shaft is degloved or examined during erection, potentially capturing mild cases that may not require intervention or be symptomatic.^[20]^[16]00188-0/pdf) More conservative population-based estimates suggest a lower prevalence of around 0.04% to 1%, reflecting underdiagnosis of subtle curvatures in routine newborn exams.^[3]^[21] When associated with hypospadias, chordee accompanies approximately 20-30% of cases, with higher rates in proximal forms of the defect; hypospadias itself occurs in about 1 in 250 live male births (40 per 10,000), yielding an attributable incidence for chordee-with-hypospadias of roughly 8-12 per 10,000 males.^[22]^[23] Isolated chordee represents a minority of total congenital penile curvatures, comprising 4-10% of all chordee cases in surgical cohorts.^[24] Overall incidence trends mirror those of hypospadias, with no clear evidence of significant increase over time independent of diagnostic awareness.^[8]

Demographic Patterns and Risk Factors

Chordee, a congenital condition characterized by abnormal penile curvature, predominantly affects males and is typically identified at birth or during early infancy.^[25] Ethnic variations in prevalence mirror patterns observed in associated congenital penile anomalies, with higher incidence rates among Caucasian newborns (approximately 9.2 per 1,000 live births) compared to Black (7.5 per 1,000), Asian (5.0 per 1,000), and Hispanic (4.7 per 1,000) populations.^[8] ^[26] Geographically, rates exhibit regional differences within the United States, with elevated incidences reported in states such as Colorado, Tennessee, and Alabama, potentially influenced by diagnostic practices, environmental factors, or genetic predispositions in these areas.^[27] Key risk factors include familial aggregation, where the condition recurs in approximately 8% of sons of affected fathers and 14% of brothers of affected individuals, indicating a heritable component independent of hypospadias association.^[23] Advanced parental age, particularly maternal age exceeding 35 years, correlates with increased risk, as does preterm birth, low birth weight, and multiple gestations.^[19] ^[27] Maternal conditions such as hypertension during pregnancy and environmental exposures, including pesticides and smoking, have been linked to higher odds, though causality remains under investigation due to confounding variables like socioeconomic factors.^[28] ^[29] Consanguinity, rural residence, and preterm labor further elevate epidemiological risk in certain cohorts.^[30] Isolated chordee without hypospadias shows similar patterns but may underreport due to milder cases evading early detection.^[31]

Etiology and Pathophysiology

Developmental Causes

Chordee arises during fetal penile development, which normally features an initial ventral curvature between weeks 9 and 12 of gestation due to rapid growth of the dorsal structures relative to the ventral urethral plate and folds. This curvature typically resolves by week 14 as mesenchymal proliferation completes urethral canalization and balances corporal body elongation, facilitated by androgen-driven differentiation of the corpora cavernosa and spongiosum.^[32]^[33] Persistent chordee without hypospadias reflects an arrest in this embryogenic progression, where failure to elongate ventral tissues leaves disproportionate dorsal expansion or fibrotic remnants, maintaining ventral bending.^[34]^[35] In hypospadias-associated cases, defective mesenchymal development surrounding the primitive urethra leads to inadequate proliferation, forming a fibrous plaque or band that tethers the ventral penis and exacerbates curvature.^[33]^[36] Androgen receptor signaling disruptions during a critical prenatal window (approximately 8-15 weeks) underlie many instances, as selective inactivation yields chordee by impairing urethral fold fusion and ventral mesenchymal maturation without broader genital ambiguity.^[37]^[38] Environmental anti-androgens, maternal estrogen exposure, or genetic variants affecting steroidogenesis (e.g., malformations in Müllerian inhibiting substance pathways) further contribute by hindering proximal urethral development and corporal symmetry.^[39]^[40]

Pathophysiological Mechanisms

Chordee arises primarily from asymmetrical development of penile tissues during embryogenesis, leading to ventral shortening or tethering that manifests as curvature, particularly during erection. The core mechanism involves disproportionate growth between dorsal and ventral penile structures, where hypoplasia or dysplasia of the ventral corpora spongiosum, urethral plate, or surrounding fascial layers (such as Buck's fascia and dartos) restricts elongation relative to the more robust dorsal corpora cavernosa.^[39] This imbalance is exacerbated by fibrosis in the affected tissues, often resulting from localized ischemia or necrosis during critical virilization phases between gestational weeks 8 and 14.^[39]^[41] Histopathological analyses reveal dense fibrous plaques composed of fibroblasts, smooth muscle cells, and vascular lacunae beneath the urethral plate, contributing to inelasticity and tethering. In cases associated with hypospadias, failure of mesenchymal tissue invasion into the urethral folds disrupts normal canalization and fusion, promoting epithelial metaplasia (e.g., squamous transformation) and subsequent scarring that enforces ventral curvature.^[39] Isolated chordee, without hypospadias, frequently implicates corporeal disproportion or fibrotic dartos/Buck's fascia, where excessive collagen deposition or disorganized extracellular matrix impairs ventral tissue compliance without urethral involvement.^[42] Hormonal dysregulation plays a contributory role, with impaired androgen signaling or imbalances in Müllerian inhibiting substance, estrogens, and prostaglandins hindering androgen-dependent mesenchymal proliferation and urethral groove closure.^[39] Vascular endothelial growth factor (VEGF) underexpression in dartos fascia correlates with chordee severity, suggesting deficient angiogenesis leads to hypovascularity, necrosis, and fibrotic replacement in ventral tissues.^[43] Environmental endocrine disruptors may amplify these defects by interfering with androgen receptor function during penile morphogenesis.^[42] Despite these insights, the precise interplay remains incompletely elucidated, with no single unifying mechanism identified across all presentations.^[42]

Clinical Presentation and Diagnosis

Signs and Symptoms

Chordee manifests as a congenital curvature of the penis, typically ventral (downward), though it may occur dorsally, laterally, or with rotation, and is most apparent during erection.^[44]^[45] The degree of curvature varies from mild to severe, often becoming noticeable in infancy during diaper changes or urination, or later during puberty when erections occur more frequently.^[44] In cases associated with hypospadias, which is common, additional signs include a urethral meatus located on the ventral surface proximal to the glans tip and a splayed or downward-directed urinary stream due to the abnormal opening.^[45]^[46] Isolated chordee without hypospadias may present with minimal symptoms until erection, potentially causing self-consciousness about penile appearance or difficulties with urinary aiming in young children.^[44] The condition is generally painless, distinguishing it from acquired penile curvatures like Peyronie's disease, though uncorrected chordee can lead to painful intercourse or functional issues in adulthood.^[44]^[46]

Diagnostic Approaches

Diagnosis of chordee primarily relies on clinical examination, as the condition manifests as ventral penile curvature, often evident during erection but potentially subtle in the flaccid state.^[47] Initial identification occurs during neonatal physical assessment, where inspection of the penis reveals asymmetry, foreskin deficiency, or deviation, particularly in cases associated with hypospadias.^[48] A thorough history, including family predisposition to genital anomalies, complements the exam to rule out syndromic associations.^[47] To accurately assess curvature severity, which is critical for treatment planning, an induced erection test is employed. This involves intracavernosal injection of saline (artificial erection) or pharmacological agents like prostaglandin E1 to simulate tumescence, allowing visualization and quantification of the bend—typically defined as significant if exceeding 20-30 degrees.^[6] ^[35] Saline injection uses a 25-27 gauge needle into the corpora cavernosa with controlled pressure to avoid artifactual distortion, while pharmacological methods provide a more physiological response but carry minor risks of priapism.^[49] Intraoperative repetition of this test confirms residual chordee post-degloving or mobilization.^[10] Quantitative measurement during induced erection may use a goniometer or visual estimation, though inter-observer variability limits reliability, with studies showing inconsistent agreement among clinicians.^[50] Routine imaging, such as ultrasound, is not standard, as clinical findings suffice for diagnosis; however, it may evaluate associated anomalies like cryptorchidism in complex cases.^[47] For isolated chordee without hypospadias, the approach mirrors that of associated forms, emphasizing erection induction to differentiate fibrous bands from skin discrepancies.^[10]

Treatment Approaches

Indications and Timing

Surgical correction of chordee is indicated when ventral penile curvature, typically exceeding 20–30 degrees during erection, impairs normal urinary stream, causes pain, or risks future sexual dysfunction such as painful intercourse.^[35]^[51] In cases associated with hypospadias, chordee repair is often performed concurrently to achieve penile straightening as a prerequisite for urethroplasty.^[52] Isolated chordee without hypospadias warrants intervention if curvature persists after conservative assessment and affects function, though mild cases under 20 degrees may not require surgery if asymptomatic.^[44] The optimal timing for chordee repair is between 6 and 18 months of age, balancing reduced risks of general anesthesia after infancy with opportunities for single-stage correction before psychological awareness or toilet training complications arise.^[53]^[54] Delaying beyond age 2 increases scarring risks and may necessitate staged procedures, while earlier intervention in severe cases (e.g., profound chordee) can be performed as young as 5 months with favorable neurovascular preservation outcomes.^[55] Preoperative evaluation, including artificial erection testing, confirms persistent curvature necessitating prompt repair to optimize long-term cosmesis and function.^[56]

Surgical Interventions

Surgical correction of chordee involves techniques to release fibrotic ventral tissue or balance corporal lengths, achieving penile straightening while minimizing complications like shortening or recurrence. Procedures typically commence with penile degloving—a circumferential incision to mobilize skin and fascia—allowing assessment of curvature magnitude under induced erection via saline injection or pharmacological agents.^[57] This step identifies residual deformity after superficial dissection, guiding subsequent interventions.^[58] In isolated chordee without hypospadias, dorsal plication predominates for mild-to-moderate ventral curvature (typically 20°–40°), shortening the convex dorsal tunica albuginea via sutures or excision. The Nesbit procedure, involving elliptical excision of dorsal tunica followed by corporotomy closure, corrects congenital or acquired chordee by deliberate dorsal shortening, with success rates of 84.2%–100% reported across studies.^[59]^[57] Alternatives like corporal rotation preserve length by rotating ventral corpora laterally, achieving 100% straightening in select series without hypospadias, though palpable knots occur in 4.8%–28.6% of cases.^[57] When chordee accompanies hypospadias, correction integrates with urethroplasty, prioritizing urethral plate preservation for mild cases via dorsal approaches like extensive mobilization or limited dorsal incision to release tethering.^[58] For severe proximal hypospadias (e.g., penoscrotal), ventral techniques prevail: urethral plate transection combined with dorsal plication yields 77.8% initial success, surpassing transection alone (50%), but risks urethrocutaneous fistula (4.65%).^[57] Ventral corporotomy—incising the corpora cavernosa ventrally—outperforms plication in severe curvature (90.63% vs. 72.06% success), often augmented by tunica vaginalis flaps or grafts (85%–100% success).^[57] Two-stage repairs, transecting the plate and grafting in stage one followed by tubularization, suit complex cases, reducing one-stage failure rates.^[58] No universal algorithm exists due to variability in curvature etiology and surgeon preference; a 2024 scoping review of 42 studies (3,180 patients) found overall success of 85%–100%, with ventral methods favored for severe hypospadias-associated chordee to avoid dorsal over-shortening.^[57] Postoperative care includes catheterization (1–2 weeks) and monitoring for recurrence, which may necessitate reoperation in 4%–15% of proximal cases.^[60]

Non-Surgical Management

Non-surgical management of chordee is generally reserved for mild cases, defined as curvatures of less than 15-20 degrees that do not significantly impair urination, erection, or future sexual function.^[61] ^[62] In these instances, watchful waiting with periodic clinical evaluations is the standard approach, allowing for natural resolution or minimal progression observed in some patients, particularly if the curvature diminishes or remains asymptomatic into puberty.^[62] ^[44] Follow-up typically includes physical examinations during routine well-child visits and reassessment during adolescence, when erections can better delineate the degree of deformity and any functional impact.^[63] No pharmacological agents, such as testosterone or hormonal therapies, have demonstrated reliable efficacy in correcting congenital chordee without hypospadias, and their use is not routinely recommended due to lack of supporting evidence from controlled studies.^[64] Similarly, mechanical interventions like penile stretching exercises or traction devices, while explored in acquired curvatures such as Peyronie's disease, lack proven benefits for congenital cases and are not endorsed by major pediatric urology guidelines.^[64] ^[25] Emerging data from a 2023 prospective study on adult men with untreated congenital penile curvature suggested that intralesional injections of collagenase clostridium histolyticum could reduce mean curvature by approximately 15-20 degrees with minimal adverse events, providing preliminary evidence for a non-surgical option in select older patients.^[65] However, this approach remains investigational, untested in pediatric cohorts, and not applicable to infants or children where surgical correction, if needed, is preferred for definitive straightening during early development.^[65] Overall, non-surgical strategies prioritize reassurance and monitoring over intervention, with progression to surgery advised if curvature worsens or causes cosmetic or functional concerns.^[44]^[63]

Outcomes, Complications, and Prognosis

Surgical Success Rates

Surgical correction of isolated chordee without hypospadias yields high success rates, typically ranging from 84% to 100% across multiple studies involving over 500 patients, with Nesbit's dorsal plication as the predominant technique.^[57] Complications in these cases remain low, with recurrence rates up to 33% but often manageable, and long-term follow-up data indicating sustained penile straightness in most instances.^[57] One series of 55 patients treated with a modified Nesbit technique reported 92.8% achieving complete penile straightness after a mean follow-up of 36.7 months, with minimal impact on erectile function (3.6% worsening).^[66] In cases associated with hypospadias, success rates for chordee correction vary more widely (50%–100%), influenced by hypospadias severity and surgical approach, based on reviews of over 2,200 patients.^[57] Ventral corporotomy techniques demonstrate superior outcomes compared to dorsal plication, particularly in proximal hypospadias, where one institutional series of 191 patients achieved 96% correction through penile degloving combined with plication or ventral grafting (tunica vaginalis or dermal).^[60]^[57] Recurrence rates post-plication can reach 83%, with higher risks emerging after puberty, underscoring the need for techniques preserving urethral integrity in two-stage repairs.^[57] Long-term data reveal potential for persistent curvature, with one cohort of hypospadias patients repaired in childhood showing 29% residual chordee at 13.7-year follow-up, though most cases were minor (<45°).^[67] Overall satisfaction remains high (86%), but reoperation risks elevate with proximal defects and inadequate initial straightening, emphasizing surgeon experience and comprehensive preoperative assessment.^[67] Success metrics prioritize erect penile straightness, with complications like shortening or sensory changes more pronounced in plication-heavy approaches.^[57]

Common Complications

Surgical correction of isolated chordee without hypospadias achieves high success rates, with an overall complication rate of 8% in a series of 87 patients treated via targeted release of tethering tissues such as skin, dartos/Buck's fasciae, or corporal disproportion.^[7] Recurrent penile curvature represents a primary complication, occurring in up to 8% of cases involving fibrotic fasciae or corporal imbalance, while urethrocutaneous fistula arises rarely (approximately 4% overall), typically in instances of minor urethral tethering.^[7] Plication techniques, such as Nesbit's procedure, commonly employed for ventral curvature correction in isolated cases, yield success rates of 84.2% to 100%, but are associated with penile shortening in 50% to 73.6% of patients and hematoma formation in 4% to 18.2%.^[57] Decreased penile sensitivity affects 5.56% to 75% postoperatively, though long-term functional impairment remains uncommon; recurrence of curvature varies widely from 0% to 33.3%, influenced by underlying etiology and surgical precision.^[57] When chordee accompanies hypospadias, complication profiles shift toward urological issues, with urethrocutaneous fistula emerging as the most frequent at 4.65% to 31.5%, followed by glans dehiscence (5.21% to 24.3%) and urethral stricture or meatal stenosis (1.5% to 25.6%).^[57] Wound infection occurs in up to 23% of isolated cases in smaller cohorts, often resolving conservatively without impacting final cosmesis or function.^[10] Reoperation rates for persistent issues, including residual curvature or fistula closure, range from 0% to 50% depending on initial severity and technique, underscoring the need for etiology-specific approaches to minimize revisions.^[7]^[57]

Long-Term Functional and Psychological Effects

Long-term functional outcomes following chordee correction, typically performed alongside hypospadias repair in infancy or early childhood, generally yield satisfactory urinary and sexual function for most patients into adulthood. Studies indicate that approximately 86% of patients report overall satisfaction with surgical results, though persistent voiding issues such as urinary spraying and hesitancy affect a notable subset, with rates of spraying reported in up to 40% of cases and hesitancy in around 20%.^[68] Sexual function is preserved in the majority, with normal erectile capability and ejaculation achieved in over 80% of adults post-repair, though 12-16% may experience difficulties including reduced penile length perception or mild curvature recurrence impacting intercourse.^[69] Persistent chordee occurs in about 29% of long-term follow-ups, potentially necessitating reoperation, while urethrocutaneous fistula rates stabilize at 11% beyond the initial postoperative period.^[70] Untreated chordee, though less common due to standard surgical intervention, correlates with higher rates of functional impairment in adulthood, including ventral curvature hindering penetration in up to 50% of cases and increased urinary dribbling.^[71] Reoperation risks decline over time post-initial repair, from 7.3 per 100 patient-years in the first decade to 0.6 in the third, reflecting stabilization of functional gains but underscoring the need for vigilant monitoring.^[72] Psychological effects of chordee and its correction can persist into adulthood, with affected individuals showing elevated risks of psychiatric symptoms such as anxiety, hostility, and diminished self-esteem compared to the general population.^[73] Men with repaired chordee often report lower satisfaction with penile appearance and self-perception, contributing to reduced confidence in social and intimate relationships, though overall sexual life satisfaction remains moderate to high in cohort studies.^[74] Untreated cases exacerbate these issues, with greater dissatisfaction in penile axis and curvature leading to avoidance of sexual activity or emotional withdrawal.^[71] Despite these challenges, psychosexual adjustment is generally positive when cosmetic and functional outcomes align with expectations, with fewer than 20% requiring psychological intervention for body image concerns in long-term assessments.^[68]

Controversies and Debates

Necessity of Intervention in Mild Cases

In mild cases of chordee, defined as ventral penile curvature typically less than 20-25 degrees without associated hypospadias or significant functional impairment, surgical intervention is often not necessary, with observation preferred to avoid operative risks such as scarring or shortening.^[75]^[44] Mild curvatures may remain asymptomatic during childhood, allowing normal urination and development without progression to severe deformity in many instances, though regular monitoring is recommended to assess for worsening during puberty when erectile tissue expands.^[62]^[75] The decision hinges on functional and cosmetic impact: intervention is indicated only if curvature exceeds thresholds that impair straight erection, sexual intercourse, or cause pain/psychological distress, as mild cases frequently do not compromise these outcomes long-term.^[44]^[54] Intraoperative assessment via degloving alone resolves up to a high percentage of mild curvatures by releasing fibrotic bands, supporting conservative approaches preoperatively for isolated mild chordee without hypospadias.^[35] Debate persists on preemptively correcting mild chordee to mitigate potential adult sexual dysfunction or body image issues, but empirical evidence favors selectivity, as overtreatment in asymptomatic boys risks unnecessary anesthesia exposure and complications without proven benefits over watchful waiting.^[62]^[44] Guidelines from pediatric urology centers emphasize patient-specific evaluation, including parental counseling on natural history, rather than universal early surgery for curvatures under 30 degrees that permit functional erections.^[75]^[54]

Debates on Surgical Techniques and Long-Term Risks

Debates center on whether dorsal plication techniques, which shorten the convex dorsal side to straighten the penis, or ventral lengthening procedures, such as corporal rotation or urethral mobilization with spongiosum, offer superior outcomes in correcting chordee without compromising penile length or function. Dorsal plication is favored for its simplicity and lower initial complication rates in mild to moderate cases, but critics argue it risks progressive penile shortening with repeated applications and higher recurrence in severe or redo surgeries due to persistent ventral disproportion.^[57]^[76] Ventral approaches, including corporal rotation pioneered by Ransley, aim to elongate the shorter ventral corpora directly, potentially preserving length better, though they carry risks of urethral injury or fistula formation, particularly when combined with hypospadias repair.00104-2/pdf)^[77] A scoping review of available studies found no randomized head-to-head trials, with mixed evidence on recurrence: one meta-analysis suggested ventral lengthening may reduce recurrent curvature risk compared to plication, while others reported equivalent straightening rates but highlighted plication's inferiority in revisions.^[57]^[78] Long-term risks of chordee correction include recurrence of curvature, often manifesting in adolescence or adulthood due to growth-related corporeal disproportion or fibrotic scarring, with historical rates of 20-30% prior to refined techniques like midline dorsal plication.^[79]^[80] In a cohort followed for up to 87 months, partial recurrence occurred in only 2% of cases after various techniques, but broader hypospadias-associated repairs show cumulative reoperation risks escalating to 51.8% at 15 years, driven partly by chordee persistence.^[81]^[22] Erectile dysfunction remains rare, affecting 0.5% in one 5-year follow-up of incisional corporoplasty, though proximal repairs (often involving severe chordee) confer 2-4 times higher odds of mild-to-moderate impairment compared to distal cases; plication does not demonstrably alter overall function despite length reduction.^[21]^[82]^[83] Grafting for ventral defects raises additional concerns for contraction and fibrosis, potentially exacerbating dysfunction, prompting preference for rotation or mobilization where feasible to minimize foreign material.^[84] These risks underscore ongoing evaluation of technique selection based on curvature severity and surgeon expertise, as no consensus exists on optimizing long-term cosmesis and sexuality.^[57]

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Comparing the incidence of hypospadias across the United States
The incidence of hypospadias in the United States was 71.6 per 10,000 male births in 2014–2018, with the highest rates reported in Colorado, Tennessee, Alabama, ...
[28]
Differences in risk factors for 2nd and 3rd degree hypospadias ... - NIH
Associations with both second and third degree hypospadias were observed for maternal age, family history, parity, plurality, and hypertension during pregnancy.
[29]
Parental risk factors associated with hypospadias
Our findings indicate that parental pesticide exposure and smoking were associated with hypospadias, maternal occupational exposure, diet and life style were ...Missing: epidemiology demographics
[30]
Genetic and other epidemiological risk factors of infants and children ...
Oct 16, 2023 · Higher maternal and paternal age, consanguinity, rural residence and preterm labor carry significant epidemiological risk factors for hypospadias.
[31]
Familial appearance of congenital penile curvature – case history of ...
Severe penile curvature is associated with difficulty in vaginal intromission, discomfort to the patient or his partner during sexual intercourse, and ...
[32]
Hypospadias: A Comprehensive Review Including Its Embryology ...
Jul 31, 2022 · North America has the highest prevalence, with 34.2 cases per 10,000 ... No chordee Mild < 30 degree chordee Moderate ( 30-60 degree) chordee ...
[33]
Embryology of the Male External Genitalia - ScienceDirect.com
If the mesenchyme fails to form any of the structures around the urethra, a fibrous band will form, causing chordee. The developmental defect causing exstrophy ...
[34]
Embryogenesis of chordee - PubMed
We propose that ventral curvature of the penis is a normal stage of embryogenesis and chordee without hypospadias may represent an arrest of development at this ...Missing: causes | Show results with:causes
[35]
Management of High-Grade Penile Curvature Associated With ... - NIH
Sep 4, 2017 · An arrest of the embryonic development at some point during this process maintains this curvature. ... chordee (45). Several other authors ...
[36]
Skin chordee without hypospadias--an under-recognised entity
Skin chordee is a congenital abnormality due to failure of development of the mesenchyme surrounding the primitive urethra. It is frequently misdiagnosed as ...Missing: fetal | Show results with:fetal
[37]
Timing of androgen receptor disruption and estrogen ... - PubMed
Dec 29, 2015 · Inactivation of AR during a narrow window of prenatal development results in hypospadias and chordee, whereas earlier disruptions cause ...Missing: etiology fetal
[38]
Aetiology of hypospadias: a systematic review of genes and ...
... chordee. More than 50% of cases have anterior hypospadias, with a small ... embryonic development. Whether these mutations have functional consequences ...
[39]
Etiopathogenesis of Chordee in Hypospadias - PMC - NIH
Malformation of Mullerian Inhibiting substance, estrogen, progesterone and corticosteroids are also indicted for the abnormal development of proximal urethra.
[40]
Pathoembryology of hypospadias and chordee - MedCrave online
Jan 9, 2018 · Genetic as well as environmental antiandrogenic and estrogenic factors are blamed for the etiology of congenital penile malformations.
[41]
[PDF] Pathoembryology of hypospadias and chordee - MedCrave online
Jan 9, 2018 · It refers to displacement of the external urethral meatus toward the undersurface of penis, the scrotum or the perineum. Congenital abnormal ...
[42]
exploring the controversies of chordee pathophysiology and its ...
Feb 2, 2024 · Treatment modalities for chordee range from surgical techniques to conservative management, each with its controversies and challenges. This ...
[43]
Expression of mRNA vascular endothelial growth factor in ...
Nov 27, 2021 · This study aimed to evaluate VEGF expressions in (1) fascia dartos between hypospadias and controls and (2) chordee severity.<|separator|>
[44]
Congenital Penile Curvature or Chordee - Cleveland Clinic
Congenital penile curvature (chordee) is a condition present at birth. It causes your penis to bend or curve. It becomes more apparent during an erection.Missing: etiology pathophysiology
[45]
Hypospadias - Symptoms and causes - Mayo Clinic
Sep 12, 2024 · Symptoms · Opening of the urethra at a place other than the tip of the penis. · Downward curve of the penis. This condition is called chordee.Overview · Symptoms · Risk Factors<|separator|>
[46]
Health Library Hypospadias / Chordee - Cincinnati Children's Hospital
Chordee is a downward curve of the penis. This can occur with or without a hypospadias. The types of hypospadias include: Distal or glandular: The most common ...Missing: classification | Show results with:classification
[47]
Hypospadias - StatPearls - NCBI Bookshelf - NIH
Distal hypospadias is the most common (60% to 70%). It is also called anterior or minor and includes glandular and sub-coronal hypospadias.Etiology · Pathophysiology · Review Questions
[48]
Current hypospadias management: Diagnosis, surgical ... - NIH
The initial diagnosis of hypospadias is typically made after birth during physical exam, where boys with hypospadias are found to have a ventral skin deficiency ...
[49]
Intraoperative pharmacological erection as an aid to pediatric ...
Artificial erection with saline injection should be performed with only moderate force since over filling is unphysiological and may falsely induce chordee.
[50]
Evaluation of penile curvature in patients with hypospadias
The degree of chordee associated with hypospadias impacts operative management. Unfortunately, poor inter-observer reliability in assessing chordee by ...Review Article · Introduction · Acknowledgement
[51]
Pediatric Urology Chordee: varied opinions and treatments as ...
VC was classified as mild, moderate or severe according to the American Academy of Pediatrics survey, which defines mild curvature as less than 30 degrees ...
[52]
Hypospadias Urogenital Reconstruction - StatPearls - NCBI Bookshelf
Lower urinary tract symptoms, including obstructive urinary flow, hesitancy, etc. are common in men after surgical repair of hypospadias, particularly the ...
[53]
General considerations in hypospadias surgery - PMC - NIH
The best time for hypospadias repair is between 6 and 18 months. Preoperative evaluation in proximal hypospadias includes hormonal and radiological examination.
[54]
[PDF] Hypospadias & Chordee - Michigan Medicine
How are hypospadias and chordee treated? When the urethra opening is just slightly out of position and when there is no significant chordee, surgical correction ...
[55]
Chordee repair utilizing a novel technique ensuring neurovascular ...
Since 1994, 37 boys with chordee have been repaired using this approach. Their ages at the time of operation ranged from 5 months to 28 years (mean 9 months).
[56]
Hypospadias repair - series—Indications - MedlinePlus
Jan 1, 2025 · Surgery is necessary to allow normal urination, to correct the penis deformity, and to ensure normal sexual functioning. The repair is performed ...
[57]
A scoping review on chordee correction in boys with ventral ... - NIH
However, the most severe forms of chordee require urethral division and a staged urethroplasty.[25,48] Unlike hypospadias, where ventral corporotomy is very ...
[58]
Hypospadias Repair: An Overview of the Actual Techniques - PMC
Chordee correction is, as any surgeon would agree, the key to the successful repair of hypospadias. Again, there are as many techniques freeing the chordee ...
[59]
Correction of chordee. The Nesbit procedure - PubMed
In select patients the Nesbit operation is a useful procedure for the correction of congenital or acquired chordee. This operation results in a deliberate ...
[60]
Correction of Chordee in Proximal Hypospadias
Oct 5, 2023 · We have found that by using the aforementioned techniques, we were able to successfully correct chordee in 96% of the proximal hypospadias patients.
[61]
Chordee Correction in hypospadias
Chordee without hypospadias is often missed on diagnosis but occurs at a rate of almost 10% as the incidence of hypospadias itself.
[62]
Chordee | Children's Hospital Colorado
Chordee (congenital penile curvature) causes the penis to bend or twist, especially while it is erect. Chordee does not always have negative impacts during ...
[63]
A guide to congenital penile curvature (CPC) - Top Doctors
Jun 25, 2024 · Congenital penile curvature (CPC) is a condition that can ... Observation and reassurance: For minor curvatures that do not impact ...
[64]
Congenital Penile Curvature (Chordee) - San Diego Sexual Medicine
Congenital penile curvature, or chordee, is defined as a congenital process which can be associated with hypospadias and is manifest by a curvature of the ...
[65]
Efficacy of collagenase Clostridium histolyticum in men ... - PubMed
Feb 14, 2023 · ... congenital penile curvature (CPC). Aim: To evaluate the safety and ... These data provide the first evidence for the efficacy of a non-surgical ...
[66]
Long-Term Results after Surgical Treatment of Congenital Penile ...
Sep 4, 2019 · Totally straightness of the penis was achieved in 51/55 patients (92.8%). After a mean long-term follow-up of 36.68 months (4–87 months), ...
[67]
Long-term functional outcome and satisfaction of patients with ... - NIH
Our series has demonstrated positive long-term outcomes in adult hypospadias patients repaired as children. Patients were satisfied with their overall surgical ...
[68]
Long-term functional outcome and satisfaction of patients ... - PubMed
Abstract. Introduction: Potential long-term sequelae of hypospadias and its surgical correction include difficulties in voiding, sexual function, psychosexual ...
[69]
Adult Sexual Function Following Hypospadias Repair in Childhood
Hypospadias repair during childhood generally leads to positive long-term sexual function outcomes. However, approximately 12%-16% of individuals may ...
[70]
[PDF] Long-term functional outcome and satisfaction of patients with ...
Overall, 86% of patients were satisfied with their surgical result, and 52% wished they had been provided longer follow-up. Conclusion: Long-term outcomes data ...
[71]
Do adult men with untreated ventral penile curvature have adverse ...
Jun 20, 2016 · Men with possible untreated ventral penile curvature reported more dissatisfaction with penile appearance, increased difficulty with intercourse, and more ...
[72]
Long-Term Risk of Reoperation After Hypospadias Surgery in ...
Aug 12, 2025 · The rates of penile reoperations was 7.3 per 100 patient-years in the first, 1.7 in the second, and 0.6 in the third decade of life (P < .001).Missing: complications | Show results with:complications
[73]
Hypospadias and Increased Risk for Psychiatric Symptoms in Both ...
Feb 23, 2022 · Patients with hypospadias have significant higher risk of hostility, reduction of social relations and emotional relations, anxiety, less self- ...
[74]
The psychosexual aspects of hypospadias repair: A review
Conclusions. Psychosexual effects of hypospadias repair they endure in adulthood, although affected men maintain satisfaction with their sexual life.
[75]
Pediatric Chordee (Penile Curvation) Treatment in Atlanta, GA
Mild cases are monitored for an increase in symptoms or curvature. Surgical Intervention. Dorsal plication is often performed on mild to moderate curvatures.Missing: observation | Show results with:observation
[76]
Long-term impact of commonly performed operations in pediatric ...
May 9, 2024 · While plication for chordee has been shown to decrease penile length, it has not been shown to alter erectile function in adulthood. A study by ...
[77]
CHORDEE CORRECTION BY CORPORAL ROTATION: THE SPLIT ...
Dorsal plication tends to shorten the phallus and ventrally positioned grafts often mandate a staged procedure. An alternative approach is presented using ...
[78]
A scoping review on chordee correction in boys with ventral ... - LWW
This study aims to address the paucity of high-level evidence by comprehensively reviewing the outcomes of PC correction procedures in patients with and ...<|separator|>
[79]
LATE ONSET RECURRENT PENILE CHORDEE AFTER ...
Recurrent chordee may be secondary to the redevelopment of corporeal disproportion and/or extensive urethral fibrosis.
[80]
Midline Dorsal Plication to Repair Recurrent Chordee at ...
For chordee that cannot be repaired by degloving alone penile straightening techniques such as Nesbit's procedure and tunica albuginea plication have been ...
[81]
Long-Term Results after Surgical Treatment of Congenital Penile ...
Sep 4, 2019 · Surgical correction of CPC is the gold standard treatment in affected patients. Various surgical techniques have been reported for the treatment ...
[82]
Adult Sexual Function and Fertility Concerns After Pediatric Repair
Apr 20, 2023 · Those with a history of proximal hypospadias repair may have a 2-4 times increased risk of mild to moderate ED compared to distal repairs, and ...
[83]
Long-term impact of commonly performed operations in pediatric ...
May 9, 2024 · For chordee, surgical plication or corporal grafting may be employed. Erectile function remains unaltered post-surgery, while penile length may ...
[84]
Correction of Penile Curvature by Rotation of the Corpora Cavernosa
Long‐term follow‐up data have showed that grafting can be complicated with graft contraction and erectile dysfunction [11]. Corporal rotation has been suggested ...