Case of Urethral Hypoplasia in Prune Belly Syndrome Successfully Managed With Vesicoamniotic Shunts and a Progressive Augmentation by Dilation Urethra Anterior Procedure.

Urethral atresia is a rare but clinically significant cause of congenital lower urinary tract obstruction. Initial management options include urinary diversion until definitive urethral reconstruction or progressive urethral dilation. Given the overall rarity of the condition, there are no evidence-based guidelines for the immediate and long-term management of urethral atresia, and clinical practice varies widely. We present an illustrative case managed with progressive urethral dilation alongside urinary diversion to highlight key factors in shared clinical decision making. Ultimately, pooled multi-institutional long-term outcomes data are needed to better guide practice for these patients and their families.

Prevalence of soft tissue calcifications in the maxillofacial region: a radiographic study

Aim: To evaluate the prevalence of soft tissue calcifications in orofacial region and their panoramic radiographic characteristics using digital panoramic radiographs among patients reporting to a tertiary dental hospital. Methods: 1,578 digital panoramic radiographs were retrieved from the archives and scrutinized for the presence of calcifications. Soft tissue calcifications were recorded according to age, gender, site (left or right). Data were analysed using Chi-square and Fisher's exact test using SPSS software and a p < 0.05 was considered statistically significant. Results: Among the total number of radiographs, calcified carotid artery (34.3%), calcified stylohyoid ligament (21%), tonsillolith (10.3%), phlebolith (17.6%), antrolith (6.3%), sialolith (5.9%), rhinolith (2.5%) and calcified lymph nodes (1.9%) were identified. The most commonly observed calcifications were calcification of carotid artery and stylohyoid ligament and the least commonly observed calcifications were rhinolith and calcified lymph node. A statistically significant association of the presence of calcifications of carotid artery and stylohyoid ligament on the left and right side was observed in females and tonsillolith on the right side in males (p-value < 0.05). Considering the gender and age group, the occurrence of antrolith among males and rhinolith among females of young-adult population, tonsillolith among the males, calcified carotid artery and stylohyoid ligament among the females of middle-aged population was found to be significant. Conclusion: Soft tissue calcifications are often encountered in dental panoramic radiographs. Our study revealed that the soft tissue calcifications in orofacial region were more common in women and were found to be increased above 40 years of age

Embarazo Gemelar con Discrepancia Morfologíca. Complejo Malformación y Obstrucción Uretral: diagnóstico prenatal / Twin Pregnancy with Morphologic Discrepancy. Complex Malformation and Urethral Obstruction: prenatal diagnosis

Presentamos un caso de embarazo gemelar, monocorial-bianmiotico con discrepancias morfológicas, diagnóstico de malformación y obstrucción uretral prenatal y diagnóstico postnatal de Síndrome de Prune-Belly. Feto A: Dilatación macroscópica del sistema colector con vejiga grande con paredes finas, hidroureter bilateral , dilatación de la uretra, parenquima renal aumentado de tamaño. Feto B: sin alteraciones morfológicas. Postnatal se observó en feto A abdomen flácido y pastoso, ano imperforado, megavejiga e insuficiencia renal

We present a case of twin pregnancy, monocorial-bi-mixtic with morphological discrepancies, diagnosis of malformation and prenatal urethral obstruction and postnatal diagnosis of Prune-Belly syndrome. Fetus A: Macroscopic dilation of the collecting system with large bladder with fine walls, bilateral hydroureter, dilation of the urethra, renal parenchymal increased in size. Fetus B: without morphological alterations. Postnatal was observed in fetus at flaccid and pasty abdomen, imperforated anus, megavejiga and renal failure.

Pregnant patient with prune belly syndrome: case report.

Prune belly syndrome is a rare congenital disease of unknown etiology that is present in one in every 40 thousand live births, and predominantly affects males, at a ratio of 4:1. In males, it presents with anomalies in the urinary system, absence of abdominal muscles, bilateral cryptorchidism, and infertility. In women, the syndrome has variable presentations, but fertility is preserved. Searching the medical literature, we found only one case of prune belly syndrome in pregnant women. Therefore, the patient in this report is the second case. She was primiparous, 25-years-old, with no abdominal muscles, severe congenital kyphoscoliosis, and pulmonary restriction. Elective cesarean section was performed at 37 weeks of gestation due to maternal risk of uterine rupture by transverse presentation and fetal risk of intrauterine growth restriction. The pre-anesthetic approach defined that general anesthesia might have more risks for the patient due to severe maternal lung disease compared to ultrasound-guided locoregional anesthesia. During prenatal care, there were some maternal complications, such as asthma exacerbations, abdominal pain, and constipation. The newborn was born small for gestational age and this can possibly be explained by maternal restrictive lung capacity. The newborn presented with Apgar score 8/9 and tachypnea, but improved after two hours of life.

Study of Penis Structure and Development in Fetus With Prune Belly Syndrome.

OBJECTIVE: To evaluate the influence of prune belly syndrome (PBS) on the development of penises in human fetuses. METHODS: We studied 39 human fetuses (including 4 with PBS) aged 11-22 weeks post conception. We measured the length and width of the free portion and penis root and the penis total length (PTL). In 21 fetuses of the control group and in 3 with PBS we analyze the penile with histochemical and immunohistochemical methods. The total penile area, area of corpora cavernosa, area of corpus spongiosum, and thickness of tunica albuginea were measured by stereological method. Means were compared using the Kolmogorov-Smirnov and ANOVA test (P <.05). RESULTS: We did not observe difference in PTL (P = .999) when we compared the control group (4.69-29.77 mm, mean = 16.2 mm; SD = 6.34 mm) with the PBS (12.23-23 mm, mean = 16.16; SD = 4.99). The total penile area (P = .002), the area of corpora cavernosa (P = .023) and the area of corpus spongiosum (P = .004) had a significant increase when comparing the PBS with the control group. CONCLUSION: In fetuses with PBS there is no change in the length of the penis but we observed a low rate of growth in all the other histologic parameters analyzed, suggesting that PBS impacts the penile development.

Modern management of and update on prune belly syndrome.

Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS are sporadic and have a normal karyotype, with 95% patients being male. In the last decade, mutations in known genes that regulate embryonic genitourinary myogenesis have been identified and with increasing knowledge of these critical genes involved in bladder maldevelopment, advances can be made in genetic counseling. A multidisciplinary approach is necessary and individualization of care is recommended according to phenotypic severity. Some patients require abdominal and urinary tract reconstruction while others require as little as bilateral orchiopexies. Major treatment objectives are: preservation of renal function and upper urinary tract; polyuria management; adequate bladder emptying; improvement of corporal image and quality of life; preservation of fertility and adequate sexual function. Long-term surveillance of the urinary tract is essential up to adulthood, because functional dynamics can change over time.

The role of intra-abdominal pressure in human testicular migration

ABSTRACT Objectives: This review aims to study the role of the abdominal wall in testicular migration process during the human fetal period. Materials and Methods: We performed a descriptive review of the literature about the role of the abdominal wall in testicular migration during the human fetal period. Results: The rise in intra-abdominal pressure is a supporting factor for testicular migration. This process has two phases: the abdominal and the inguinal-scrotal stages. The passage of the testis through the inguinal canal occurs very quickly between 21 and 25 WPC. Bilateral cryptorchidism in Prune Belly syndrome is explained by the impaired contraction of the muscles of the abdominal wall; mechanical obstruction due to bladder distention and structural alteration of the inguinal canal, which hampers the passage of the testis during the inguinoscrotal stage of testicular migration. Abdominal wall defects as gastroschisis and omphaloceles are associated with undescended testes in around 30 to 40% of the cases. Conclusions: Abdominal pressure wound is an auxiliary force in testicular migration. Patients with abdominal wall defects are associated with undescendend testis in more than 30% of the cases probably due to mechanical factors; the Prune Belly Syndrome has anatomical changes in the anterior abdominal wall that hinder the increase of intra-abdominal pressure which could be the cause of cryptorchidism in this syndrome.

The role of intra-abdominal pressure in human testicular migration.

OBJECTIVES: This review aims to study the role of the abdominal wall in testicular migration process during the human fetal period. MATERIALS AND METHODS: We performed a descriptive review of the literature about the role of the abdominal wall in testicular migration during the human fetal period. RESULTS: The rise in intra-abdominal pressure is a supporting factor for testicular migration. This process has two phases: the abdominal and the inguinal-scrotal stages. The passage of the testis through the inguinal canal occurs very quickly between 21 and 25 WPC. Bilateral cryptorchidism in Prune Belly syndrome is explained by the impaired contraction of the muscles of the abdominal wall; mechanical obstruction due to bladder distention and structural alteration of the inguinal canal, which hampers the passage of the testis during the inguinoscrotal stage of testicular migration. Abdominal wall defects as gastroschisis and omphaloceles are associated with undescended testes in around 30 to 40% of the cases. CONCLUSIONS: Abdominal pressure wound is an auxiliary force in testicular migration. Patients with abdominal wall defects are associated with undescendend testis in more than 30% of the cases probably due to mechanical factors; the Prune Belly Syndrome has anatomical changes in the anterior abdominal wall that hinder the increase of intra-abdominal pressure which could be the cause of cryptorchidism in this syndrome.

Seudo síndrome prune belly / Pseudo Prune-belly syndrome

Introducción: El síndrome prune belly es una rara y compleja anomalía congénita y el seudo prune belly es aún más raro y en muchas ocasiones no se diagnostica como tal. Objetivo: Describir un paciente con las características de este síndrome. Presentación del caso: Feto de sexo masculino que en la semana 23 del embarazo el ultrasonido prenatal detectó dilatación pélvica bilateral y se propuso la interrupción del embarazo que no fue aceptada por los padres. Al nacer se observa criptorquidia bilateral, el ultrasonido renal a las 34 horas de nacido confirma dilatación pélvica bilateral severa, se indica profilaxis con cefalexina, y a los cuatro dias de nacido es enviado a consulta de nefrología. Al llegar a consulta se observa la ausencia de testículos en ambas bolsas escrotales y el ultrasonido abdominal a los 16 dias confirma dilatación pélvica renal derecha severa y uréter dilatado en su tercio superior con 6 mm de diámetro y ureteropielocaliectasia severa del lado izquierdo; no se visualiza testículo derecho y el izquierdo en canal inguinal mide 7 × 10 mm. La gammagrafía estática demostró hipocaptación marcada del radiofármaco por el riñón izquierdo y disminución de la función renal relativa y en la uretrocistografía miccional se encontró dilatación vesical y reflujo vesicoureteral con ureterohidronefrosis bilateral. Conclusiones: El seudo síndrome prune belly presenta alteraciones complejas del tracto urinario que son las que establecen el pronóstico en estos casos(AU)

Introduction: Prune belly syndrome is a rare and complex congenital anomaly and the pseudo prune belly is even rarer and often not diagnosed as such. Objective: To describe a patient with the characteristics of this syndrome. Presentation of the case: Male fetus that in the 23rd week of pregnancy it was detected a bilateral pelvic dilatation in the prenatal ultrasound; and it was proposed the termination of pregnancy which was not accepted by the parents. At birth it is observed bilateral cryptorchidism; the renal ultrasound at 34 hours after birth confirmed bilateral severe pelvic dilation, it is indicated prophylaxis with cephalexin, and at four days after birth, the newborn is remitted to the Nephrology consultation. In the consultation it was noticed the absence of scrotal sacs in both testicles and the abdominal ultrasound at 16 days after birth confirmed severe right renal pelvic dilatation and dilated ureter in its upper third with 6 mm of diameter and severe uretero-pielocaliectasy in the left side; it is not visualized the right testicle and the left in the inguinal channel measured 7 × 10 mm. Static scintigraphy showed marked hypocaptation of the radiopharmaceutical by the left kidney and decreased relative renal function, and in the voiding cystourethrogram it was found bladder dilation and vesicoureteral reflux with bilateral ureterohydronephrosis. Conclusions: The pseudo prune belly syndrome presents complex abnormalities of the urinary tract that are those which establish the prognosis in these cases(AU)

Trasplante renal en paciente con pared abdominal compleja: síndrome de abdomen en ciruela pasa / Kidney transplant in a patient with a complex abdominal wall: Prune belly syndrome

Introducción. Se presenta un paciente con falla renal crónica secundaria a hidroureteronefrosis bilateral, con antecedente de síndrome de abdomen en ciruela pasa (prune belly), en quien se realiza trasplante renal. El objetivo de este reporte de caso es exponer una anomalía congénita, con baja incidencia mundial y en menor proporción asociado a trasplante renal, con énfasis en la dificultad para la técnica quirúrgica dada por la hipoplasia de los músculos de la pared abdominal. Métodos. Revisión de Historia clínica, Consentimiento Informado. Búsqueda de la literatura.Caso clínico. En cirugía se identifica ausencia de músculos de la pared abdominal y fibrosis de las venas Iliaca externa y común derecha. Se realiza abordaje bajo técnica de Gibson contralateral, con trasplante renal sin complicaciones. Presentó adecuada evolución en seguimiento ambulatorio. Discusión. El síndrome de abdomen en ciruela pasa (prune belly) es una anomalía congénita de baja incidencia, con pocos reportes asociados a trasplante renal. El reto quirúrgico está dado por la ausencia de músculos de la pared abdominal, que aumentan los riesgos por posibles complicaciones, como la lesión de los vasos epigástricos inferiores. Para el cierre de pared abdominal en trasplante renal, se describe en dos planos, que en este caso se realiza sobre fascia muscular y piel. Aun siendo un reto, el paciente presenta adecuada evolución postoperatoria

Introduction. We present a patient with chronic renal failure secondary to bilateral hydroureteronephrosis, with a history of prune belly syndrome, who underwent a kidney transplant. The objective of this case report is to expose a congenital anomaly, with a low worldwide incidence and to a lesser extent associated with kidney transplantation, with emphasis on the difficulty for the surgical technique due to the hypoplasia of the abdominal wall muscles.Methods. Review of clinical history, Informed Consent. Literature search.Clinical case. Absence of abdominal wall muscles and fibrosis of the external iliac veins and right common veins were identified intraoperatively. An approach was performed under the contralateral Gibson technique, with uncomplicated kidney transplantation. He presented adequate evolution in outpatient follow-up.Discussion. Prune belly syndrome is a low-incidence congenital anomaly, with few reports associated with kidney transplantation. The surgical challenge is given by the absence of abdominal wall muscles, which increases the risks due to possible complications, such as injury to the inferior epigastric vessels. For the closure of the abdominal wall in kidney transplantation, it is described in two planes, which in this case is performed on muscle fascia and skin. Despite being a challenge, the patient has adequate postoperative evolution

Follow up of Patients with Eagle-Barett (Prune Belly) Syndrome Treated with Single-Stage Abdominoplasty and Total Genitourinary Tract Reconstruction / Seguimiento de pacientes con síndrome de Eagle-Barret (prune belly) tratados con abdominoplastia en un solo tiempo y reconstrucción total del tracto genitourinario

Introduction The Prune Belly Syndrome (PBS) is the deficiency or congenital hypoplasia of the abdominal muscles accompanied with disorders of the urinary tract. The surgical treatment for the uropathy of the PBS seeks to correct the anatomical defects that induce damage to the urinary tract, including the improvement of the bladder emptying. The correction of the abdominal wall defect must be considered as an important part of the treatment. Objective To describe of the most relevant clinic characteristics and outcomes of a group of eight patients with PBS managed with abdominoplasty and reconstructive urologic surgery. Methods Retrospective review of the medical charts of patients with PBS at Hospital Universitario San Ignacio, Hospital Militar Central of Bogotá, Clinica Infantil Colsubsidio and Fundación Oftalmológica Ardila Lulle of Bucaramanga, (Colombia) that were managed with reconstructive surgery of the urinary tract and Monfort technique abdominoplasty (between 2006 and 2016) by one of the authors (JPN). The evaluated variables included: phenotypic appearance, renal function, bowel movements, and urinary tract infection (UTI) episodes. These variables as well as the abdominal wall aspect, were evaluated postoperatively. Results There were 11 patients identified with PBS between 2006 and 2016. Eight (8) of them underwent reconstructive urologic surgery, orchidopexy and Monfort technique abdominoplasty. After the procedures, none of the patients presented deterioration of the renal function, and they reported a better quality of the stools according to the Bristol scale; there were also less episodes of UTI. The phenotypic aspect of the abdominal wall was qualified as satisfactory in all cases. Conclusions Reconstructive urologic surgery and abdominoplasty in patients with PBS is an excellent choice of management that reduces the number of UTIs, improves bowel movements according to the Bristol scale, and preserves the renal function. This management approach also improves the phenotypic aspect of the abdominal wall. All of this leads to higher survival rate and better quality of life.

Introducción El Síndrome de Prune Belly (SPB) es la deficiencia o hipoplasia congénita de los músculos abdominales acompañada de desórdenes del tracto genitourinario. El manejo quirúrgico de la uropatía del SPB busca corregir los defectos anatómicos que inducen daño al tracto urinario, incluyendo la mejora del vaciamiento vesical. La corrección del defecto de la pared abdominal debe considerarse como una parte importante del manejo. Objetivos Describir las características clínicas más relevantes y el desenlace de un grupo de 8 pacientes con SPB manejados con abdominoplastia y cirugía urológica reconstructiva. Materiales y Métodos Revisión retrospectiva de las historias clínicas de los pacientes con SPB del Hospital Universitario San Ignacio, Hospital Militar Central de Bogotá, Clínica Infantil Colsubsidio, y Fundación Oftalmológica Carlos Ardila Lulle de Bucaramanga (Colombia), manejados con cirugía reconstructiva de las vías urinarias y abdominoplastia tipo Monfort entre los años 2008 y 2016 por uno de los autores (JPN). Las variables incluidas fueron: aspecto fenotípico, función renal, hábito intestinal y características de las deposiciones, y episodios de infección urinaria. El desenlace de estas variables y el aspecto de la pared abdominal fueron evaluados en el postoperatorio. Resultados se identificaron 11 pacientes con SPB entre 2006 y 2016. Ocho (8) fueron sometidos a cirugía reconstructiva de las vías urinarias, orquidopexia y abdominoplastia tipo Monfort. Después de los procedimientos, ninguno de los pacientes presentó deterioro de la función renal; los pacientes refieren mejor calidad de las deposiciones según la escala de Bristol; se presentaron menos episodios de infección de vías urinarias. El aspecto fenotípico fue calificado como satisfactorio en todos los casos. Conclusiones La cirugía urológica reconstructiva acompañada de abdominoplastia en pacientes con SPB es una excelente forma de manejo que reduce el número de infecciones urinarias, mejora la calidad del hábito intestinal según la escala de Bristol, y conserva la función renal. Este manejo mejora además el aspecto fenotípico de la pared abdominal. Todo esto lleva a una mayor sobrevida y mejor calidad de vida.

Do prune-belly syndrome and neural tube defects change testicular growth? A study on human fetuses.

BACKGROUND: There are no reports comparing testicular volume between normal fetuses, fetuses with prune-belly syndrome (PBS), and fetuses with anencephaly. The study hypothesis was that PBS and especially anencephaly alter the testicular volume during the human fetal period. AIM: The objective of the study was to compare the testicular growth in fetuses with anencephaly, with PBS, and without anomalies. STUDY DESIGN: This is a morphometric study of human fetuses. Seventy testes from fetuses without anomalies aged 11-22 weeks post-conception (WPC), 30 testes from fetuses with anencephaly aged 13-19 WPC, and eight testes from fetuses with PBS aged 13-16 WPC were studied. Testicular length, width, and thickness were evaluated with the aid of computer programs (Image Pro and ImageJ) (Figure). The fetal testicular volume was calculated using the ellipsoid formula: Testicular volume (TV) = [length × thickness × width] × 0.523. The Shapiro-Wilk test was used to ascertain the normality of the data and to compare quantitative data between normal fetuses vs. fetuses with anencephaly, while the Kruskal-Wallis test was used to assess gender and laterality differences. Simple linear correlations (LCs) were calculated for testicular volume according to fetal age, weight, and crown-rump length. RESULTS: All 108 testes studied were abdominal. The right (p = 0.0310) and left (0.0470) testicular volumes were significantly smaller in fetuses with anencephaly than those in the control group. The linear regression analysis indicated that the right and the left testis volume in the control group (right: r2 = 0.6665; left: r2 = 0.6707) and PBS group (right: r2 = 0.9937; left: r2 = 0.9757) increased with fetal age (p < 0.0001). This analysis also indicated that the testicular volume in fetuses with anencephaly did not increase with fetal age (right: r2 = 009816; left: r2 = 0.07643). DISCUSSION: This article is the first to report testicular volume correlations with fetal parameters in fetuses with anencephalic and fetuses with PBS. Significant alterations were observed in testicular growth in the anencephalic group compared with the control group, and it was also observed that the bilateral cryptorchidism in PBS does not alter the testicular development and growth during the fetal period. The unequal WPC distribution between fetuses with PBS, fetuses with anencephaly, and controls and the small sample size are limitations of this study. Further studies should be performed to confirm this study's findings. CONCLUSIONS: Testicular growth is slower and does not show significant correlations with fetal parameters in fetuses with anencephalic. Significant differences in testicular development in fetuses with PBS was not observed.

Pfeiffer Syndrome Type 3 and Prune Belly Anomaly in a Female: Case Report and Review.

Background: Pfeiffer syndrome (PS) is an autosomal dominant entity characterized by craniosynostosis, broad thumbs, and preaxially deviated great toes. It is classified in three types depending on the severity. Type 1: Mild to moderate severity, Type 2: Severe presentation with cloverleaf skull, and Type 3: Severe craniosynostosis with prominent ocular proptosis. Association of Pfeiffer syndrome (PS) types 2 and 3 with "prune belly" anomaly has been reported in two non-related patients, one PS type 2 and one PS type 3. Case Report: we report the second case of PS type 3 in a female neonate with "prune belly" anomaly and prenatal exposure to Parvovirus B19. Conclusions: We suggest that the "prune belly" anomaly and others abdominal wall defects as omphalocele and scar-type defects may be included as a feature in PS type 2 and 3.

Síndrome de eagle: reporte de caso / Eagle syndrome: case report / Síndrome de eagle: relato de caso

Resumen El síndrome de Eagle se considera como la elongación del proceso estiloides con osificación del ligamento estilohioideo o sin él, que se extiende entre el hueso temporal y el hioides. El diagnóstico puede ser realizado principalmente con tomograña computarizada (TC) o radiografía panorámica, por su puesto sin dejar de lado la variedad de signos y síntomas, como dolor cervical, sensación de cuerpo extraño en la faringe, limitaciones en la apertura bucal, dificultad para la fonación, disfagia, otalgia, tinnitus, trismo y movilidad reducida del cuello. Ya identificada la elongación, el paciente podrá recibir un tratamiento conservador o quirúrgico dependiendo de la severidad de los síntomas. El manejo conservador incluye fisioterapia, infiltración con anestésicos locales o corticosteroides y el tratamiento con fármacos antiinflamatorios, anticonvulsivos o antidepresivos. El manejo quirúrgico consiste en fracturar el proceso estiloides bajo anestesia general, a través de abordajes transorales o transcervicales. A continuación, expondremos información pertinente sobre el síndrome de Eagle y posteriormente presentaremos un caso clínico de una paciente con dicho síndrome del servicio de Cirugía Oral y Maxilofacial del Hospital Militar Central.

Abstract Eagle syndrome is considered as the elongation of the styloid process with or without ossification of the stylohyoid ligament, which extends between the temporal bone and the hyoid. The diagnosis can be made mainly with computed tomography (CT) or panoramic radiography, of course without neglecting the variety of signs and symptoms, such as cervical pain, foreign body sensation in the pharynx, limitations in oral opening, difficulty in phonation, dysphagia, otalgia, tinnitus, trismus and reduced neck mobility. Once the elongation has been identified, the patient may receive conservative or surgical treatment depending on the severity of the symptoms. Conservative management includes physiotherapy, infiltration with local anesthetics or corticosteroids and treatment with anti-inflammatory, anticonvul sant or antidepressant drugs. Surgical management consists in fracturing the styloid process under general anesthesia, through transoral or transcervical approaches. Next, we will present pertinent information about the Eagle syndrome and then we will present a clinical case of a patient with said syndrome of the Oral and Maxillofacial Surgery service of the Hospital Militar Central.

Resumo A síndrome de Eagle considera-se como o alongamento do processo estiloide com ossificação do ligamento estilo-hioideo ou sem ele, que se estende entre o osso temporal e os hioides. O diagnóstico pode ser realizado principalmente com tomografia computadorizada (TC) ou radiografia panorâmica, obviamente sem deixar de lado a variedade de sinais e sintomas, como dor cervical, sensação de corpo estranho na faringe, limitações na abertura bucal, dificuldade para a fonação, disfagia, otalgia, zumbido, trismo e mobilidade reduzida do pescoço. Já identificado o alongamento, o paciente poderá receber um tratamento conservador ou cirúrgico dependendo da gravidade dos sintomas. O tratamento conservador inclui fisioterapia, infiltração com anestésicos locais ou corticosteroides e o tratamento com fármacos anti-inflamatórios, anticonvulsivos ou antidepressivos. O tratamento cirúrgico consiste em fraturar o processo estiloide sob anestesia geral, por meio de abordagens intraorais ou transcervicais. A seguir, exporemos informação pertinente sobre a síndrome de Eagle e, posteriormente, apresentaremos um caso clínico de uma paciente com essa síndrome do serviço de Cirurgia Oral e Maxilofacial do Hospital Militar Central.

Collagen I and III in women with diastasis recti.

OBJECTIVES: Interest in elucidating the etiology of hernias has encouraged countless studies of musculoaponeurotic structures in individuals with and without hernias. Studies of hernia patients have firmly demonstrated a correlation between hernias and collagen alterations in their fascia. Diastasis recti is an increased width of the abdominal midline that is exclusively composed of interlacing aponeurotic expansions of the anterolateral abdominal muscles. The condition is common among women undergoing abdominoplasty, and many factors, not only mechanical, play a role. The goal of this study is to evaluate and compare collagen type I and III levels in the midline fascia of women with and without diastasis recti to report their possible influence on this condition. METHODS: This is a case-control study nested within a surgical cohort of 18 women with diastasis recti and 18 women without the condition (cases and controls, respectively). Fascia from the midline of the abdominal wall was collected and analyzed through immunohistochemistry using polyclonal antibodies to collagen type I and III. RESULTS: Both type I and type III collagen were less abundant in women with diastasis recti than in those without the condition, and the difference was statistically significant (p<0.001). CONCLUSION: Low collagen type I and type III levels in the midline of the abdominal wall may play a key role in the development of diastasis recti.

Structural study of the bladder in fetuses with prune belly syndrome.

AIMS: To study the bladder structure of fetuses with prune belly syndrome (PBS). METHODS: We studied three bladders obtained from three male fetuses with PBS and seven bladders from seven male fetuses without anomalies. Each bladder was dissected and embedded in paraffin, from which 5 µm thick sections were obtained and stained with Masson's trichrome (to quantify connective tissue and smooth muscle) and picrosirius red with polarization (to observe collagen). Immunohistochemistry with tubulin (Tubulin, beta III, Mouse Monoclonal Antibody) was applied to observe the bladder nerves. The images were captured with an Olympus BX51 microscope and Olympus DP70 camera. The stereological analysis was done with the Image Pro and Image J programs, using a grid to determine volumetric densities (Vv). Means were statistically compared using the Mann-Whitney test (P < 0.05). RESULTS: Quantitative analysis documented that smooth muscle fibers were significantly smaller (P = 0.04) in PBS fetuses (9.67% to 17.75%, mean = 13.2%) compared to control group (13.33% to 26.56%, mean = 17.43%). The analysis of collagen fibers showed predominance of green in the control group, suggesting collagen type III presence, and predominance of red in the in PBS fetal bladders, suggesting collagen type I presence in this group. The qualitative analysis of the nerves with immunohistochemistry with tubulin showed predominance of nerves in the control group. CONCLUSION: The bladder in PBS had lower concentrations of smooth muscle fibers, collagen type III, and nerves. These structural alterations can be one of the factors involved in urinary tract abnormality such as distended bladder in patients with PBS.

Collagen I and III in women with diastasis recti

OBJECTIVES: Interest in elucidating the etiology of hernias has encouraged countless studies of musculoaponeurotic structures in individuals with and without hernias. Studies of hernia patients have firmly demonstrated a correlation between hernias and collagen alterations in their fascia. Diastasis recti is an increased width of the abdominal midline that is exclusively composed of interlacing aponeurotic expansions of the anterolateral abdominal muscles. The condition is common among women undergoing abdominoplasty, and many factors, not only mechanical, play a role. The goal of this study is to evaluate and compare collagen type I and III levels in the midline fascia of women with and without diastasis recti to report their possible influence on this condition. METHODS: This is a case-control study nested within a surgical cohort of 18 women with diastasis recti and 18 women without the condition (cases and controls, respectively). Fascia from the midline of the abdominal wall was collected and analyzed through immunohistochemistry using polyclonal antibodies to collagen type I and III. RESULTS: Both type I and type III collagen were less abundant in women with diastasis recti than in those without the condition, and the difference was statistically significant (p<0.001). CONCLUSION: Low collagen type I and type III levels in the midline of the abdominal wall may play a key role in the development of diastasis recti.

De las bases embriológicas a la clínica en el síndrome de Prune Belly / From the embryological basis to the clinical Prune Belly syndrome

El síndrome de Prune Belly es un trastorno congénito, que obedece según lo reportado actualmente a una base genética. Está caracterizado por la siguiente triada: deficiencia en grados variables de la musculatura abdominal, criptorquidia bilateral y anomalías del tracto urinario. Se identifican dos variantes del síndrome, una mortal y otra compatible con la vida(AU)

Prune Belly syndrome is a congenital disorder that is due, as currently reported, to genetic basis. It is characterized by the following triad: deficiency of abdominal muscles in varying degrees, bilateral cryptorchidism and urinary tract anomalies. Two variants of the syndrome are identified, a deadly one and another compatible with life(AU)

Suspected Urine Leak in a Pediatric Renal Transplant Patient With Prune Belly Syndrome.

Patients with prune belly syndrome usually have tortuous ureters, which can cause difficulty in the interpretation of renal scan used to evaluate possible urine leak after renal transplant. We reported a renal scan finding in a pediatric renal transplant patient with prune belly syndrome. The radioactivity in the dilated ureter, which was lateral to the renal transplant, appears to be urine leak.