Malignant transformation and tumour recurrence in sacrococcygeal teratoma: a global, retrospective cohort study.

INTRODUCTION: Sacrococcygeal teratoma (SCT) is a rare congenital tumour. The risk of malignancy and recurrence are not well defined. Previous studies are small and report differing conclusions about the timing of surgery and the duration of follow-up. We studied the risk of malignant transformation and SCT recurrence after surgery to address these gaps. METHODS: This was a global retrospective cohort study. Data of consecutive SCT patients was obtained from 145 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence or death due to malignancy, and its risk factors were analysed. RESULTS: Of the 3612 included patients, 3407 entered analysis. Risk of malignant transformation of the initial tumour, was 3.3%, 5.1%, 10.1%, and 32.9% at age three months, six months, one year, and two years, respectively. After six years, the censored risk of malignancy (64%) did not further increase. Recurrent SCT was diagnosed in 349 (10·2%) children with 126 (36·1%) malignant recurrences. Risk factors for recurrence were Altman type II (odds ratio (OR): 1·6, 95% confidence interval (CI): 1·2-2·3), Altman type III (OR: 1·6, 95% CI: 1·2-2·3), initial immature histology (OR: 1·9, 95% CI: 1·4-2·6), and initial malignant histology (OR: 4·0, 95% CI: 2·9-5·4). CONCLUSION: The risk of malignancy at initial resection in SCT increases with age reaching a plateau at six years of age. Recurrence after resection occurred in 10% of patients and 36% of these were malignant at that time. Altman type II or type III, and immature or malignant histology were associated with recurrence. LEVEL OF EVIDENCE: level III.

Resultados del uso del irrigador transanal en la disfunción intestinal asociada a malformaciones anorrectales / Results of the use of the transanal irrigator in intestinal dysfunction associated with anorectal malformations

Introducción: Existen múltiples herramientas para optimizar la función defecatoria en pacientes con malformación anorrectal (MAR): hábitos, laxantes, irrigaciones retrógradas o anterógradas. Estas se adecuan de forma progresiva y combinada. El objetivo de este estudio fue evaluar la incorporación del dispositivo de irrigación transanal (ITA) al tratamiento del estreñimiento y la incontinencia fecal en pacientes con MAR. Materiales y métodos: Estudio retrospectivo en pacientes con MAR con indicación del ITA según el protocolo de manejo intestinal del consultorio de patología colorrectal desde el 2015 al 2022. Tras usarlo más de 3 meses, los pacientes o sus cuidadores completaron un cuestionario telefónico propio aprobado por el comité de ética. Resultados: 39 pacientes con MAR: 11 fístulas rectouretrales, 6 rectovesicales, 16 cloacas, 2 rectovaginales, 2 perineales y 2 vestibulares. El 44% presentó un Índice Sacro < 0,4. El 62% presentaban estreñimiento y el 38% incontinencia. Debido al uso del ITA, mejoró la sensación de confianza y seguridad en muy alto y alto grado en el 89% de los pacientes. Disminuyó mucho el tiempo dedicado a su manejo intestinal en el 68%. El 79% refiere mejoría en su calidad de vida con 9 y 10 puntos. El 92% calificaron su satisfacción general con ITA con un valor de 8, 9 y 10 siendo 10 completamente satisfecho. El 100% lo recomiendan. Conclusión. El ITA es una buena alternativa para el manejo intestinal de la incontinencia fecal y el estreñimiento.(AU)

Introduction: There are multiple tools available to optimize defecation in patients with anorectal malformation (ARM), such as habits, laxatives, and retrograde or anterograde irrigations, which are usually adapted in a progressive and combined fashion. The objective of this study was to assess the incorporation of transanal irrigation (TAI) to constipation and fecal incontinence treatment in patients with ARM. Materials and methods: A retrospective study of ARM patients with indication of TAI according to the colorectal pathology unit’s intestinal management protocol from 2015 to 2022 was carried out. Following use for over 3 months, patients or their guardians completed a phone survey of our own approved by the ethics committee. Results. 39 ARM patients participated in the study. Pathologies included 11 rectourethral fistulas, 6 rectovesical fistulas, 16 cloacae, 2 rectovaginal fistulas, 2 perineal fistulas, and 2 vestibular fistulas. 44% of them had a sacral index < 0.4. 62% had constipation, and 38% had incontinence. Thanks to TAI, confidence and safety improved in a very high and a high degree in 89% of the patients, whereas time devoted to intestinal management decreased a lot in 68% of them. 79% reported a 9- and 10-point quality-of-life improvement. 92% rated overall satisfaction with TAI with a score of 8, 9, and 10 –10 meaning “completely satisfied.” 100% recommend TAI. Conclusion. TAI is a good alternative for the intestinal management of fecal incontinence and constipation.(AU)

Results of transanal irrigation in intestinal dysfunction associated with anorectal malformations. / Resultados del uso del irrigador transanal en la disfunción intestinal asociada a malformaciones anorrectales.

INTRODUCTION: There are multiple tools available to optimize defecation in patients with anorectal malformation (ARM), such as habits, laxatives, and retrograde or anterograde irrigations, which are usually adapted in a progressive and combined fashion. The objective of this study was to assess the incorporation of transanal irrigation (TAI) to constipation and fecal incontinence treatment in patients with ARM. MATERIALS AND METHODS: A retrospective study of ARM patients with indication of TAI according to the colorectal pathology unit's intestinal management protocol from 2015 to 2022 was carried out. Following use for over 3 months, patients or their guardians completed a phone survey of our own approved by the ethics committee. RESULTS: 39 ARM patients participated in the study. Pathologies included 11 rectourethral fistulas, 6 rectovesical fistulas, 16 cloacae, 2 rectovaginal fistulas, 2 perineal fistulas, and 2 vestibular fistulas. 44% of them had a sacral index < 0.4. 62% had constipation, and 38% had incontinence. Thanks to TAI, confidence and safety improved in a very high and a high degree in 89% of the patients, whereas time devoted to intestinal management decreased a lot in 68% of them. 79% reported a 9- and 10-point quality-of-life improvement. 92% rated overall satisfaction with TAI with a score of 8, 9, and 10 - 10 meaning "completely satisfied." 100% recommend TAI. CONCLUSION: TAI is a good alternative for the intestinal management of fecal incontinence and constipation.

INTRODUCCION: Existen múltiples herramientas para optimizar la función defecatoria en pacientes con malformación anorrectal (MAR): hábitos, laxantes, irrigaciones retrógradas o anterógradas. Estas se adecuan de forma progresiva y combinada. El objetivo de este estudio fue evaluar la incorporación del dispositivo de irrigación transanal (ITA) al tratamiento del estreñimiento y la incontinencia fecal en pacientes con MAR. MATERIAL Y METODOS: Estudio retrospectivo en pacientes con MAR con indicación del ITA según el protocolo de manejo intestinal del consultorio de patología colorrectal desde el 2015 al 2022. Tras usarlo más de 3 meses, los pacientes o sus cuidadores completaron un cuestionario telefónico propio aprobado por el comité de ética. RESULTADOS: 39 pacientes con MAR: 11 fístulas rectouretrales, 6 rectovesicales, 16 cloacas, 2 rectovaginales, 2 perineales y 2 vestibulares. El 44% presentó un Índice Sacro < 0,4. El 62% presentaban estreñimiento y el 38% incontinencia. Debido al uso del ITA, mejoró la sensación de confianza y seguridad en muy alto y alto grado en el 89% de los pacientes. Disminuyó mucho el tiempo dedicado a su manejo intestinal en el 68%. El 79% refiere mejoría en su calidad de vida con 9 y 10 puntos. El 92% calificaron su satisfacción general con ITA con un valor de 8, 9 y 10 siendo 10 completamente satisfecho. El 100% lo recomiendan. CONCLUSION: El ITA es una buena alternativa para el manejo intestinal de la incontinencia fecal y el estreñimiento.

Comparative analysis between laparoscopic (UCL) and open (UCO) technique for the treatment of ulcerative colitis in pediatric patients.

PURPOSE: The aim of this study is to compare laparoscopic with open approach in the surgical treatment of ulcerative colitis (UC) MATERIAL AND METHODS: Between July 1991 and August 2009, 32 consecutive unselective patients with UC received surgical treatment. The population analyzed was divided into three groups: subtotal colectomy + ileocolostomy (Group 1), proctectomy + ileoanal pouch (Group 2), one-step proctocolectomy + ileoanal pouch (Group 3). We analyzed the mean operating time, postoperative oral intake, use of opiates, the length of hospital stay and postoperative morbidity in each group. Open and laparoscopic approaches were compared in each group retrospectively. RESULTS: In Group 1 the mean operating time was longer for the laparoscopic group (301 vs. 197 min; p < 0.01). The length of postoperative stay was longer for the open group (8 vs. 19 days; p < 0.05) and the oral intake started earlier in the laparoscopic group (3, 5 vs. 6, 2 days; p = 0.05). No significant difference was found in the use of opiates (p = ns). A total of four major complications occurred in the laparoscopic group and another four in the open approach. In Group 2, there was no significant difference in operating time between laparoscopic and open approach. The laparoscopic group started earlier to tolerate (p < 0.05) and there were significantly differences in the use of narcotics and hospital stay (p < 0.05). General complications were related to the pouch. In Group 3 the mean operating time was longer for the laparoscopic group (470 vs. 330 min p < 0.05). Patients with a laparoscopic approach had a shorter hospital stay (5, 6 vs. 10 days; p < 0.05) and postoperative narcotic use and they started earlier to tolerate (p < 0.05). One major complication was presented in the laparoscopic procedure and two in the open approach. CONCLUSIONS: Of the 165 patients with UC in our hospital, 32 underwent surgery. The laparoscopic approach seems feasible and safe. A single staged approach (Group 1 + Group 2) remains the most reasonable choice for most patients. One-step approach was done only in selected cases (Group 3). The advantages of laparoscopy, such as improved cosmetic aspects, shorter postoperative ileus and hospital stay, were observed in the laparoscopic colectomy, proctectomy and ileoanal pouch in our study.

Nódulos mamarios de tratamiento quirúrgico en la adolescencia / Mmamary nodules. the surgical treatment in adolescents

La mayoría de los tumores mamarios en adolescentes son benignos. De ellos el fibroadenoma es el más frecuente. A pesar de que existen numerosas publicaciones acerca de patología mamaria quirúrgica en adolescentes, los casos presentados en cada una de ellas no son numerosos. Quince pacientes portadores de tumores mamarios tratados quirúrgicamente fueron asistidas en el Hospital J.P. Garrahan entre agosto 87 y julio 96. Tres pacientes presentaron tumores múltiples. Trece eran de sexo femenino y dos de sexo masculino. El rango de edad fue de 12 a 19 años (media:15a, 10m). El exámen físico mostró la presencia de tumores bien delimitados, duros, redondeados y móviles en 14 de ellos. Cinco pacientes presentaron dolor. La localización más frecuente de los tumores únicos fue en los cuadrantes exteriores. La ecografía mostró en 13 pacientes imagen hipoecoica, nodular, homogenea y en un cado de tejido mamario homogeneo. El abordaje fue periareolar, de extensión variable de acuerdo al tamaño del tumor, en 13 pacientes y transareolar transversa en 2. Los resultados cosméticos fueron muy buenos. La histología de los tumores fue: Fibroadenoma (n:10); Adenoma tubular (n:1) Fibroadenoma mas Adenoma tubular (n:1) Fibroadenoma mas mastopatía fibroquística (n:1) y Neurofibroma (n:1)

Nódulos mamarios de tratamiento quirúrgico en la adolescencia / Mmamary nodules. the surgical treatment in adolescents

La mayoría de los tumores mamarios en adolescentes son benignos. De ellos el fibroadenoma es el más frecuente. A pesar de que existen numerosas publicaciones acerca de patología mamaria quirúrgica en adolescentes, los casos presentados en cada una de ellas no son numerosos. Quince pacientes portadores de tumores mamarios tratados quirúrgicamente fueron asistidas en el Hospital J.P. Garrahan entre agosto 87 y julio 96. Tres pacientes presentaron tumores múltiples. Trece eran de sexo femenino y dos de sexo masculino. El rango de edad fue de 12 a 19 años (media:15a, 10m). El exámen físico mostró la presencia de tumores bien delimitados, duros, redondeados y móviles en 14 de ellos. Cinco pacientes presentaron dolor. La localización más frecuente de los tumores únicos fue en los cuadrantes exteriores. La ecografía mostró en 13 pacientes imagen hipoecoica, nodular, homogenea y en un cado de tejido mamario homogeneo. El abordaje fue periareolar, de extensión variable de acuerdo al tamaño del tumor, en 13 pacientes y transareolar transversa en 2. Los resultados cosméticos fueron muy buenos. La histología de los tumores fue: Fibroadenoma (n:10); Adenoma tubular (n:1) Fibroadenoma mas Adenoma tubular (n:1) Fibroadenoma mas mastopatía fibroquística (n:1) y Neurofibroma (n:1)

Vaginal reconstruction after initial construction of the external genitalia in girls with salt-wasting adrenal hyperplasia.

A total of 28 patients with salt-wasting adrenal hyperplasia who underwent revision of the external genitalia at an early age (mean age 21.4 months) has been followed for 12 to 20 years (mean 16.3 years). The age at initial surgery ranged from 3 weeks to 5 years. Twenty-five patients had a low and 3 had a high takeoff of the vagina from the urogenital sinus. Of these children 22 (78.5%) required further vaginal reconstructive procedures to achieve a normal vaginal outlet. Of the 22 patients requiring further repair 18 have undergone vaginal reconstructive procedures with an initial success rate of 72.2%. These long-term data suggest that if vaginal reconstructive surgery is performed in infancy details, such as placement of the perineal flap well into the vagina and careful followup of the vaginal outlet, are mandatory for success. If there is evidence of outlet narrowing, periodic dilation will be needed to prevent vaginal stenosis and further vaginal surgery.