The Experts below are selected from a list of 321 Experts worldwide ranked by ideXlab platform
Dix P. Poppas - One of the best experts on this subject based on the ideXlab platform.
-
Nerve Sparing Clitoroplasty is an Option for Adolescent and Adult Female Patients with Congenital Adrenal Hyperplasia and Clitoral Pain following Prior Clitoral Recession or Incomplete Reduction
The Journal of Urology, 2016Co-Authors: Jennifer Reifsnyder, Diane Felsen, John Stites, Kerlly J. Bernabé, Denise Galan, Dix P. PoppasAbstract:Purpose: In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction Clitoroplasty technique.Materials and Methods: We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere. They then presented to our center with clitoral pain or enlargement, where they were treated with nerve sparing Clitoroplasty between 2000 and 2010. We collected patient reported data relating to clitoral sensation and sexual function outcomes.Results: Mean ± SD age at evaluation for repeat clitoral surgery was 21 ± 7 years and mean age at Clitoroplasty was 22 ± 8 years. Median postoperative followup was 9 months (IQR 32–6). All patients showed improvement with resolution of clitoral pain or enlargement.C...
-
Nerve Sparing Clitoroplasty is an Option for Adolescent and Adult Female Patients with Congenital Adrenal Hyperplasia and Clitoral Pain following Prior Clitoral Recession or Incomplete Reduction.
The Journal of urology, 2016Co-Authors: Jennifer Reifsnyder, Diane Felsen, John Stites, Kerlly J. Bernabé, Denise Galan, Dix P. PoppasAbstract:In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction Clitoroplasty technique. We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere. They then presented to our center with clitoral pain or enlargement, where they were treated with nerve sparing Clitoroplasty between 2000 and 2010. We collected patient reported data relating to clitoral sensation and sexual function outcomes. Mean ± SD age at evaluation for repeat clitoral surgery was 21 ± 7 years and mean age at Clitoroplasty was 22 ± 8 years. Median postoperative followup was 9 months (IQR 32-6). All patients showed improvement with resolution of clitoral pain or enlargement. Clitoral pain and enlargement upon arousal can be a major concern for women with clitoromegaly and congenital adrenal hyperplasia after clitoral recession is performed. Our series suggests that clitoral recession or incomplete reduction in childhood may be an inadequate initial solution in the congenital adrenal hyperplasia population due to the potential for future androgen elevation and the possibility of later symptom development. In addition we found that patients may be successfully treated with nerve sparing Clitoroplasty, resulting in resolution of pain and ability to engage in sexual activity. Copyright © 2016 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
-
Clitoroplasty in Congenital Adrenal Hyperplasia: Description of Technique
Advances in Experimental Medicine and Biology, 2011Co-Authors: Dix P. PoppasAbstract:Female patients with congenital adrenal hyperplasia (CAH) may develop masculinized genitalia in utero upon exposure to excess androgen. This masculinization may be characterized by hyperpigmented or fused labioscrotal tissue, enlarged labia majora, a urogenital sinus, and clitoromegaly. Clitoromegaly may vary, ranging from an ordinary clitoris to a significantly enlarged clitoris similar to a near-normal penis, with any degree of intermediate variation. Surgical management of clitoromegaly may ensure adequate sexual function, promote consistent female gender identity development, and provide a typical clitoris aesthetic. Patients and families considering clitoral surgery should make every effort to understand the options, risks, and benefits of this surgery. If surgery is being considered, it should only be performed in centers with significant experience.
-
Nerve Sparing Ventral Clitoroplasty: Analysis of Clitoral Sensitivity and Viability
The Journal of Urology, 2007Co-Authors: Jennifer H. Yang, Diane Felsen, Dix P. PoppasAbstract:Purpose: Enlargement of the clitoris is often a prominent manifestation of virilizing congenital adrenal hyperplasia and other disorders of sexual development. Controversy persists regarding the viability and sensitivity of the clitoris following Clitoroplasty. We present 51 consecutive cases of nerve sparing ventral Clitoroplasty performed by a single surgeon.Materials and Methods: Nerve sparing ventral Clitoroplasty was performed in all patients. Postoperative evaluation for clitoral viability included gross examination and capillary perfusion testing. Patients older than 5 years were evaluated for clitoral sensory testing and vibratory sensory testing.Results: A total of 51 patients 4 months to 24 years old (mean age ± SD 4.6 ± 6.8 years) with clitorimegaly underwent nerve sparing ventral Clitoroplasty. Of the patients 41 had capillary perfusion testing of the clitoris, of whom all had a viable clitoris. Ten of the 41 patients underwent clitoral sensory testing. Patients reported an average degree of s...
-
Nerve Sparing Ventral Clitoroplasty Preserves Dorsal Nerves in Congenital Adrenal Hyperplasia
The Journal of Urology, 2007Co-Authors: Dix P. Poppas, Ariella A Hochsztein, Rebecca N Baergen, Emily Loyd, Jie Chen, Diane FelsenAbstract:Purpose: Masculinization of the female genitalia observed in patients with classic congenital adrenal hyperplasia often results in clitorimegaly. Reduction Clitoroplasty is the most widely practiced corrective surgery for clitorimegaly, yet reservations about surgical intervention exist based on fears of nerve destruction during surgical removal of excess erectile tissue. In this study, we modified the reduction Clitoroplasty and examined excised erectile tissue for the presence of dorsal nerves.Materials and Methods: We describe the development of the nerve sparing ventral Clitoroplasty. Nerves were examined in situ using optical coherence technology. In addition, erectile tissue removed from 27 female patients with congenital adrenal hyperplasia was examined immunohistochemically for the presence of nerves by staining for neurofilament. Nerves outside of the tunica albuginea were counted and measured. Tissue from 2 adult females was also examined by immunohistochemistry.Results: Optical coherence techno...
Shreyasi U. Patil - One of the best experts on this subject based on the ideXlab platform.
-
Reduction Clitoroplasty by Ventral Approach: Technical Refinement
The Journal of Obstetrics and Gynecology of India, 2019Co-Authors: Uddhav A. Patil, Paddmaja U. Patil, Manasi S. Devdikar, Shreyasi U. PatilAbstract:Background Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity. Purpose Clitoral reduction surgery has evolved over the years. Total clitorectomy, clitoral recession and neuro-vascular pedicle sparing Clitoroplasty can be considered as three major milestones in its evolution. In nerve sparing Clitoroplasty, earlier dorso-lateral approach was challenged by ventral approach, which is establishing itself as the approach of choice by its own merits. Clitoral reduction surgery being not a very commonly performed surgery, demands refinements in its technique to improve its results and particularly the aesthetic outcome, which is gaining more importance in recent years. Methods An adult case of late onset congenital adrenal hyperplasia (CAH) with clitoromegaly underwent nerve sparing reduction Clitoroplasty by ventral approach under moderate magnification. We present key points of the preoperative anatomic evaluation. Technical refinements planned and executed step wise during the procedure resulted in satisfactory outcome. Result Patient now has normal appearing genitalia, a small clitoris nicely covered by skin hood and very well preserved sensation to light touch. Conclusions Preservation of sensation in the glans clitoris is of paramount importance during clitoral reduction surgery. Knowledge of the anatomy of the neurovascular supply to the glans clitoris gives us freedom from not isolating it in order to preserve it. In fact a deliberate attempt to dissect it can prove detrimental particularly when operating on a smaller organ during childhood. A ventral approach with refined technique proves safe and gives satisfactory result. Moderate degree of magnification eases dissection. A component-based detailed preoperative planning improves the overall aesthetic appearance of the female genitalia.
-
Reduction Clitoroplasty by Ventral Approach: Technical Refinement.
The Journal of Obstetrics and Gynecology of India, 2019Co-Authors: Uddhav A. Patil, Paddmaja U. Patil, Manasi S. Devdikar, Shreyasi U. PatilAbstract:Background Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity.
-
Reduction Clitoroplasty by Ventral Approach: Technical Refinement.
Journal of obstetrics and gynaecology of India, 2017Co-Authors: Uddhav A. Patil, Paddmaja U. Patil, Manasi S. Devdikar, Shreyasi U. PatilAbstract:Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity. Clitoral reduction surgery has evolved over the years. Total clitorectomy, clitoral recession and neuro-vascular pedicle sparing Clitoroplasty can be considered as three major milestones in its evolution. In nerve sparing Clitoroplasty, earlier dorso-lateral approach was challenged by ventral approach, which is establishing itself as the approach of choice by its own merits. Clitoral reduction surgery being not a very commonly performed surgery, demands refinements in its technique to improve its results and particularly the aesthetic outcome, which is gaining more importance in recent years. An adult case of late onset congenital adrenal hyperplasia (CAH) with clitoromegaly underwent nerve sparing reduction Clitoroplasty by ventral approach under moderate magnification. We present key points of the preoperative anatomic evaluation. Technical refinements planned and executed step wise during the procedure resulted in satisfactory outcome. Patient now has normal appearing genitalia, a small clitoris nicely covered by skin hood and very well preserved sensation to light touch. Preservation of sensation in the glans clitoris is of paramount importance during clitoral reduction surgery. Knowledge of the anatomy of the neurovascular supply to the glans clitoris gives us freedom from not isolating it in order to preserve it. In fact a deliberate attempt to dissect it can prove detrimental particularly when operating on a smaller organ during childhood. A ventral approach with refined technique proves safe and gives satisfactory result. Moderate degree of magnification eases dissection. A component-based detailed preoperative planning improves the overall aesthetic appearance of the female genitalia.
Diane Felsen - One of the best experts on this subject based on the ideXlab platform.
-
Nerve Sparing Clitoroplasty is an Option for Adolescent and Adult Female Patients with Congenital Adrenal Hyperplasia and Clitoral Pain following Prior Clitoral Recession or Incomplete Reduction
The Journal of Urology, 2016Co-Authors: Jennifer Reifsnyder, Diane Felsen, John Stites, Kerlly J. Bernabé, Denise Galan, Dix P. PoppasAbstract:Purpose: In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction Clitoroplasty technique.Materials and Methods: We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere. They then presented to our center with clitoral pain or enlargement, where they were treated with nerve sparing Clitoroplasty between 2000 and 2010. We collected patient reported data relating to clitoral sensation and sexual function outcomes.Results: Mean ± SD age at evaluation for repeat clitoral surgery was 21 ± 7 years and mean age at Clitoroplasty was 22 ± 8 years. Median postoperative followup was 9 months (IQR 32–6). All patients showed improvement with resolution of clitoral pain or enlargement.C...
-
Nerve Sparing Clitoroplasty is an Option for Adolescent and Adult Female Patients with Congenital Adrenal Hyperplasia and Clitoral Pain following Prior Clitoral Recession or Incomplete Reduction.
The Journal of urology, 2016Co-Authors: Jennifer Reifsnyder, Diane Felsen, John Stites, Kerlly J. Bernabé, Denise Galan, Dix P. PoppasAbstract:In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction Clitoroplasty technique. We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere. They then presented to our center with clitoral pain or enlargement, where they were treated with nerve sparing Clitoroplasty between 2000 and 2010. We collected patient reported data relating to clitoral sensation and sexual function outcomes. Mean ± SD age at evaluation for repeat clitoral surgery was 21 ± 7 years and mean age at Clitoroplasty was 22 ± 8 years. Median postoperative followup was 9 months (IQR 32-6). All patients showed improvement with resolution of clitoral pain or enlargement. Clitoral pain and enlargement upon arousal can be a major concern for women with clitoromegaly and congenital adrenal hyperplasia after clitoral recession is performed. Our series suggests that clitoral recession or incomplete reduction in childhood may be an inadequate initial solution in the congenital adrenal hyperplasia population due to the potential for future androgen elevation and the possibility of later symptom development. In addition we found that patients may be successfully treated with nerve sparing Clitoroplasty, resulting in resolution of pain and ability to engage in sexual activity. Copyright © 2016 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
-
Nerve Sparing Ventral Clitoroplasty: Analysis of Clitoral Sensitivity and Viability
The Journal of Urology, 2007Co-Authors: Jennifer H. Yang, Diane Felsen, Dix P. PoppasAbstract:Purpose: Enlargement of the clitoris is often a prominent manifestation of virilizing congenital adrenal hyperplasia and other disorders of sexual development. Controversy persists regarding the viability and sensitivity of the clitoris following Clitoroplasty. We present 51 consecutive cases of nerve sparing ventral Clitoroplasty performed by a single surgeon.Materials and Methods: Nerve sparing ventral Clitoroplasty was performed in all patients. Postoperative evaluation for clitoral viability included gross examination and capillary perfusion testing. Patients older than 5 years were evaluated for clitoral sensory testing and vibratory sensory testing.Results: A total of 51 patients 4 months to 24 years old (mean age ± SD 4.6 ± 6.8 years) with clitorimegaly underwent nerve sparing ventral Clitoroplasty. Of the patients 41 had capillary perfusion testing of the clitoris, of whom all had a viable clitoris. Ten of the 41 patients underwent clitoral sensory testing. Patients reported an average degree of s...
-
Nerve Sparing Ventral Clitoroplasty Preserves Dorsal Nerves in Congenital Adrenal Hyperplasia
The Journal of Urology, 2007Co-Authors: Dix P. Poppas, Ariella A Hochsztein, Rebecca N Baergen, Emily Loyd, Jie Chen, Diane FelsenAbstract:Purpose: Masculinization of the female genitalia observed in patients with classic congenital adrenal hyperplasia often results in clitorimegaly. Reduction Clitoroplasty is the most widely practiced corrective surgery for clitorimegaly, yet reservations about surgical intervention exist based on fears of nerve destruction during surgical removal of excess erectile tissue. In this study, we modified the reduction Clitoroplasty and examined excised erectile tissue for the presence of dorsal nerves.Materials and Methods: We describe the development of the nerve sparing ventral Clitoroplasty. Nerves were examined in situ using optical coherence technology. In addition, erectile tissue removed from 27 female patients with congenital adrenal hyperplasia was examined immunohistochemically for the presence of nerves by staining for neurofilament. Nerves outside of the tunica albuginea were counted and measured. Tissue from 2 adult females was also examined by immunohistochemistry.Results: Optical coherence techno...
-
Nerve sparing ventral Clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia.
The Journal of urology, 2007Co-Authors: Dix P. Poppas, Ariella A Hochsztein, Rebecca N Baergen, Emily Loyd, Jie Chen, Diane FelsenAbstract:Masculinization of the female genitalia observed in patients with classic congenital adrenal hyperplasia often results in clitorimegaly. Reduction Clitoroplasty is the most widely practiced corrective surgery for clitorimegaly, yet reservations about surgical intervention exist based on fears of nerve destruction during surgical removal of excess erectile tissue. In this study, we modified the reduction Clitoroplasty and examined excised erectile tissue for the presence of dorsal nerves. We describe the development of the nerve sparing ventral Clitoroplasty. Nerves were examined in situ using optical coherence technology. In addition, erectile tissue removed from 27 female patients with congenital adrenal hyperplasia was examined immunohistochemically for the presence of nerves by staining for neurofilament. Nerves outside of the tunica albuginea were counted and measured. Tissue from 2 adult females was also examined by immunohistochemistry. Optical coherence technology visualized dorsal nerves in 3 patients with congenital adrenal hyperplasia (size 600 to 800 microm). In 4 of 27 patients undergoing nerve sparing ventral Clitoroplasty, no dorsal nerve branches were visualized in excised erectile tissue. In another 18 patients 10 or fewer nerve branches were found. In patients who underwent nerve sparing ventral Clitoroplasty 92% of dorsal nerves detected were 90 microm or less. In contrast, 88% of dorsal nerves found in the 2 adult specimens were 120 microm or greater. The maximum nerve fiber size observed in patient specimens was significantly smaller than the maximum nerve fiber size observed in control specimens. Scarcity of large dorsal nerves in histological specimens excised using nerve sparing ventral Clitoroplasty likely reflects their preservation within the congenital adrenal hyperplasia patients. This preservation is vital to future somatosensory and motor function of the clitoris.
Uddhav A. Patil - One of the best experts on this subject based on the ideXlab platform.
-
Reduction Clitoroplasty by Ventral Approach: Technical Refinement
The Journal of Obstetrics and Gynecology of India, 2019Co-Authors: Uddhav A. Patil, Paddmaja U. Patil, Manasi S. Devdikar, Shreyasi U. PatilAbstract:Background Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity. Purpose Clitoral reduction surgery has evolved over the years. Total clitorectomy, clitoral recession and neuro-vascular pedicle sparing Clitoroplasty can be considered as three major milestones in its evolution. In nerve sparing Clitoroplasty, earlier dorso-lateral approach was challenged by ventral approach, which is establishing itself as the approach of choice by its own merits. Clitoral reduction surgery being not a very commonly performed surgery, demands refinements in its technique to improve its results and particularly the aesthetic outcome, which is gaining more importance in recent years. Methods An adult case of late onset congenital adrenal hyperplasia (CAH) with clitoromegaly underwent nerve sparing reduction Clitoroplasty by ventral approach under moderate magnification. We present key points of the preoperative anatomic evaluation. Technical refinements planned and executed step wise during the procedure resulted in satisfactory outcome. Result Patient now has normal appearing genitalia, a small clitoris nicely covered by skin hood and very well preserved sensation to light touch. Conclusions Preservation of sensation in the glans clitoris is of paramount importance during clitoral reduction surgery. Knowledge of the anatomy of the neurovascular supply to the glans clitoris gives us freedom from not isolating it in order to preserve it. In fact a deliberate attempt to dissect it can prove detrimental particularly when operating on a smaller organ during childhood. A ventral approach with refined technique proves safe and gives satisfactory result. Moderate degree of magnification eases dissection. A component-based detailed preoperative planning improves the overall aesthetic appearance of the female genitalia.
-
Reduction Clitoroplasty by Ventral Approach: Technical Refinement.
The Journal of Obstetrics and Gynecology of India, 2019Co-Authors: Uddhav A. Patil, Paddmaja U. Patil, Manasi S. Devdikar, Shreyasi U. PatilAbstract:Background Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity.
-
Reduction Clitoroplasty by Ventral Approach: Technical Refinement.
Journal of obstetrics and gynaecology of India, 2017Co-Authors: Uddhav A. Patil, Paddmaja U. Patil, Manasi S. Devdikar, Shreyasi U. PatilAbstract:Aesthetic alteration of the genitalia is increasingly sought by women who are unhappy about the size, shape or overall appearance of their vulva. Clitoral hypertrophy is usually seen in congenital malformations, specifically in intersexual stages of hormonal expression. A large clitoris has an appearance of a small penis, is psychologically disturbing and interferes in sexual activity. Clitoral reduction surgery has evolved over the years. Total clitorectomy, clitoral recession and neuro-vascular pedicle sparing Clitoroplasty can be considered as three major milestones in its evolution. In nerve sparing Clitoroplasty, earlier dorso-lateral approach was challenged by ventral approach, which is establishing itself as the approach of choice by its own merits. Clitoral reduction surgery being not a very commonly performed surgery, demands refinements in its technique to improve its results and particularly the aesthetic outcome, which is gaining more importance in recent years. An adult case of late onset congenital adrenal hyperplasia (CAH) with clitoromegaly underwent nerve sparing reduction Clitoroplasty by ventral approach under moderate magnification. We present key points of the preoperative anatomic evaluation. Technical refinements planned and executed step wise during the procedure resulted in satisfactory outcome. Patient now has normal appearing genitalia, a small clitoris nicely covered by skin hood and very well preserved sensation to light touch. Preservation of sensation in the glans clitoris is of paramount importance during clitoral reduction surgery. Knowledge of the anatomy of the neurovascular supply to the glans clitoris gives us freedom from not isolating it in order to preserve it. In fact a deliberate attempt to dissect it can prove detrimental particularly when operating on a smaller organ during childhood. A ventral approach with refined technique proves safe and gives satisfactory result. Moderate degree of magnification eases dissection. A component-based detailed preoperative planning improves the overall aesthetic appearance of the female genitalia.
Rojas Johana - One of the best experts on this subject based on the ideXlab platform.
-
MANEJO QUIR aRGICO DE PACIENTES CON TRASTORNOS DE LA DIFERENCIACI 3N SEXUAL.SERVICIO DESCONCENTRADO HOSPITAL PEDI\uc1TRICO DR. AGUST\ucdN ZUBILLAGA. VENEZUELA
2017Co-Authors: Rojas JohanaAbstract:Los trastornos de diferenciaci\uf3n sexual (TDS) constituyen una patolog\ueda cong\ue9nita ocasionada por defectos gen\ue9ticos, cromos\uf3micos, gonadales o fenot\uedpicos que ocurren durante las diferentes etapas del desarrollo sexual. Se realiz\uf3 un estudio descriptivo retrospectivo con el objetivo de describir el manejo quir\ufargico de los pacientes con trastornos de la diferenciaci\uf3n sexual que acudieron al Servicio Desconcentrado Hospital Pedi\ue1trico Dr. "Agust\uedn Zubillaga", durante el lapso 2010-2014. Se obtuvo una serie de 23 pacientes cuya edad del diagn\uf3stico fue en el reci\ue9n nacido (56,52%), seguido del lactante menor (21,74%) y adolescencia (13,04%); de los cuales 56,52% eran 46,XX, 34,78% 46,XY, 4,35% fueron 46XY/45X y 45X. De los primeros 34,78% eran hiperplasia suprarrenal cong\ue9nita y 13,04% ovotesticular; los segundos se reportaron 8,70% persistencia del conducto de M\ufcller, deficiencia de 5a reductasa, insensibilidad completa a los andr\uf3genos, hipospadia proximal, respectivamente. La primera intervenci\uf3n se realiz\uf3 entre 1-5 a\uf1os (34,78%) y en menores de 1 a\uf1o (21,74%); mientras, la segunda intervenci\uf3n se registr\uf3 en el rango de 1-5 a\uf1os (17,39%) y 6-10 a\uf1os (17,39%). Las conductas quir\ufargicas m\ue1s frecuentes fueron clitoroplast\ueda (38,88%), gonadectom\ueda para genitoplast\ueda masculina (33,33%), gonadectom\ueda para genitoplast\ueda femenina (22,22%) y cura de hipospadias (22,22%). Solo 22,22% present\uf3 complicaciones postoperatorias, de las cuales 25% fueron mediatas por absceso escrotal derecho y tard\uedas 25% dadas por dehiscencia de sutura de clitoroplast\ueda, hipertrofia de cl\uedtoris y hemat\uf3metra. De esta manera, se logra demostrar la experiencia en el tratamiento quir\ufargico de los pacientes con TDS en este centro. PALABRAS CLAVE: Trastornos de la diferenciaci\uf3n sexual. Manejo quir\ufargico. Complicaciones. ABSTRACT Disorders of Sex development (DSD) are a group of congenital conditions originated from genetic problems or gonadic issues that occurs during the sexual differentiation. There are very few case reports about the diagnosis and management of this conditions which lead to under recognition and poor management of these patients with a physical and psychological burden in their lives. During the years 2010 through 2014, we describe the surgical management of 23 patients at the Pediatric Surgical Service of Agustin Zubillaga Hospital. Of the patients, 57% were neonates, 22% were less than 1 year of age 8% were between 1year through adolescence and 13% were adolescents. Cariotype XY accounted for 35% of the patients, 56% were XX, 4% had 45X and 4% were 46XY/45X. Among the XY patients, there were even diagnosis (Muller Duct Persistence, 5 alpha reductase deficiency, proximal hypospadias and androgen insensitivity syndrome). Among the XX patients, the most common diagnosis was congenital suprarenal hyperplasia. The majority of patients had their first intervention before 5 year of age (56%). Most common surgical procedures were gonadectomy (55%), Clitoroplasty (39%) and hypospadias repair (22%). Postoperative complications were uncommon being scrotal abscess the most common one. These case series shows the most common diagnosis and surgical techniques used in patients with SDD's in our hospital. It also emphasizes that early diagnosis and repair are the most important part in the management of patients. KEY WORDS: Disorders of Sex Development. Surgical management. Complications.
-
MANEJO QUIR aRGICO DE PACIENTES CON TRASTORNOS DE LA DIFERENCIACI 3N SEXUAL. SERVICIO DESCONCENTRADO HOSPITAL PEDI\uc1TRICO DR. "AGUST\ucdN ZUBILLAGA"
2017Co-Authors: Rojas JohanaAbstract:Los trastornos de diferenciaci\uf3n sexual (TDS) constituyen una patolog\ueda cong\ue9nita ocasionada por defectos gen\ue9ticos, cromos\uf3micos, gonadales o fenot\uedpicos que ocurren durante las diferentes etapas del desarrollo sexual. Se realiz\uf3 un estudio descriptivo retrospectivo con el objetivo de describir el manejo quir\ufargico de los pacientes con trastornos de la diferenciaci\uf3n sexual que acudieron al Servicio Desconcentrado Hospital Pedi\ue1trico Dr. "Agust\uedn Zubillaga", durante el lapso 2010-2014. Se obtuvo una serie de 23 pacientes cuya edad del diagn\uf3stico fue en el reci\ue9n nacido (56,52%), seguido del lactante menor (21,74%) y adolescencia (13,04%); de los cuales 56,52% eran 46,XX, 34,78% 46,XY, 4,35% fueron 46XY/45X y 45X. De los primeros 34,78% eran hiperplasia suprarrenal cong\ue9nita y 13,04% ovotesticular; los segundos se reportaron 8,70% persistencia del conducto de M\ufcller, deficiencia de 5a reductasa, insensibilidad completa a los andr\uf3genos, hipospadia proximal, respectivamente. La primera intervenci\uf3n se realiz\uf3 entre 1-5 a\uf1os (34,78%) y en menores de 1 a\uf1o (21,74%); mientras, la segunda intervenci\uf3n se registr\uf3 en el rango de 1-5 a\uf1os (17,39%) y 6-10 a\uf1os (17,39%). Las conductas quir\ufargicas m\ue1s frecuentes fueron clitoroplast\ueda (38,88%), gonadectom\ueda para genitoplast\ueda masculina (33,33%), gonadectom\ueda para genitoplast\ueda femenina (22,22%) y cura de hipospadias (22,22%). Solo 22,22% present\uf3 complicaciones postoperatorias, de las cuales 25% fueron mediatas por absceso escrotal derecho y tard\uedas 25% dadas por dehiscencia de sutura de clitoroplast\ueda, hipertrofia de cl\uedtoris y hemat\uf3metra. De esta manera, se logra demostrar la experiencia en el tratamiento quir\ufargico de los pacientes con TDS en este centro. PALABRAS CLAVE: Trastornos de la diferenciaci\uf3n sexual. Manejo quir\ufargico. Complicaciones. ABSTRACT Disorders of Sex development (DSD) are a group of congenital conditions originated from genetic problems or gonadic issues that occurs during the sexual differentiation. There are very few case reports about the diagnosis and management of this conditions which lead to under recognition and poor management of these patients with a physical and psychological burden in their lives. During the years 2010 through 2014, we describe the surgical management of 23 patients at the Pediatric Surgical Service of Agustin Zubillaga Hospital. Of the patients, 57% were neonates, 22% were less than 1 year of age 8% were between 1 year through adolescence and 13% were adolescents. Cariotype XY accounted for 35% of the patients, 56% were XX, 4% had 45X and 4% were 46XY/45X. Among the XY patients, there were even diagnosis (Muller Duct Persistence, 5 alpha reductase deficiency, proximal hypospadias and androgen insensitivity syndrome). Among the XX patients, the most common diagnosis was congenital suprarenal hyperplasia. The majority of patients had their first intervention before 5 year of age (56%). Most common surgical procedures were gonadectomy (55%), Clitoroplasty (39%) and hypospadias repair (22%). Postoperative complications were uncommon being scrotal abscess the most common one. These case series shows the most common diagnosis and surgical techniques used in patients with SDD\u92s in our hospital. It also emphasizes that early diagnosis and repair are the most important part in the management of patients. KEY WORDS: Disorders of Sexual Differentiation. Surgical management. Complications.