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Psychosexological counseling in patients affected by bladder exstrophy-epispadias complex BEEC
Le malformazioni urogenitali complesse: la presa in carico chirurgica e psicologica del bambino-adolescente e della famiglia
Bladder exstrophy-epispadias complex (BEEC) represents
a spectrum of genitourinary malformations concerning
male and female sexes. It needs different surgical
operations to obtain, primarily, secure abdominal wall
closure, achieving urinary continence with preservation
of renal function, and, finally, adequate cosmetic and
functional genital reconstruction. The psychological feature
and the sexual aspects of this pathology have important
effects on the patient’s and their family’s past. So,
the multidisciplinary approach, which involves psychologist
and surgeon upfront, permits: to completely answer
patients’ and relatives’ questions, to give emotional and
sexual education, emphasizing the expression and the
verbalization of the emotions, and to give people concerned
useful tools to face the BEEC in a more conscious
way. By treating the pathology in a multidimensional
way, it is possible to accompany the patients and their
families along the developmental steps, optimizing therapeutic
intervention benefits
Qualitative questionnaire on the psychosocial wellbeing of mothers of children with BEEC
Introduction
The bladder exstrophy–epispadias complex (BEEC) represents a spectrum of malformations that affect the anatomical and functional structure of the urogenital system. The parents of patients affected by this condition are subject to particularly stressful situations, such as worrying about their child's health, long hospital stays, concerns about the health and constant need for personal care for their children, that can profoundly compromise the quality of family life.
Objective
The objective of this explorative qualitative study is to evaluate the social situation and the psychological strategies implemented by the mothers of children between 6 and 10 years of age who are affected by BEEC.
Study design
Fourteen mothers of children aged 6–10 years and affected by BEEC (9 boys and 5 girls) were interviewed. Data on the mothers' experiences were collected through semi-structured interviews (Table).
Results
The qualitative analysis of the interviews showed that participants described experiences that were characterised by emotions such as fear and anger. Each mother had implemented a different and, sometimes, dysfunctional strategy in order to cope with the complex situation of the son/daughter. The aspects that most clearly emerged from mothers' descriptions were (1) the traumatic situation at the birth of the baby, (2) the sense of embarrassment concerning the pathological condition as the child was growing and the consequent sense of isolation of the mother, and (3) the fluctuation of feelings towards the multidisciplinary staff, which was sometimes seen as an important source of help and some other times as too destabilising and not helpful at all.
Discussion
The study provided some insight into the psychological and social conditions experienced by mothers of children with BEEC, which could serve as a basis for developing multidisciplinary teams with greater awareness about families living with this condition and better timing in addressing their needs.
Conclusions
Mothers of children with BEEC show emotional and social difficulties. This is a crucial aspect to consider when planning a multidisciplinary approach to the treatment/therapy, especially considering that children examined in this study are approaching adolescence
Psychosexual development management of bladder exstrophy epispadias in complex patients
Introduction
Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase.
Objective
The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC. The main goal of the intervention program was to educate the adolescents and re-frame how they see themselves or feel about themselves, especially in relation to BEEC. In particular it was predicted that the program could: (1) improve the perception of pleasure concerning the body, particularly regarding the genital area, giving proper and specific information on pleasure, masturbation and medical history of BEEC; and (2) elicit a more relational-affective perspective on sexuality.
Study design
13 adolescent patients took part in the 1-year program. The effects of the intervention program were verified through a test-retest methodology using Sexuality Evaluation Schedule Assessment Monitoring (SESAMO).
Results
The results showed that participants changed their attitude in several psychosexual areas, more specifically: psycho-environmental situations, body experience, areas of pleasure, medical and sexual history, and motivation and conflict areas.
Conclusions
This study demonstrated, for the first time, that a targeted program may significantly improve the psycho-sexual condition of adolescents with BEEC. In particular, this reserch showed that adolescents need to be able to discuss and tackle topics of a psychological and sexual nature, as well as receive understandable answers that can be put into practice in their everyday lives. The study had several methodological limitations, especially owing to the limited number of participants, the absence of a follow-up period of a few months after the intervention, and the overall exploratory nature of the program.
Discussion
This intervention methodology may be considered a first attempt at improving the self-esteem of adolescents with BEEC, by contrasting forms of psychological difficulties in order to improve the quality of life of these young people
Estrofia Vescicale, Epispadia
il complesso estrofla vescicale-epispadia rappresenta un gruppo di malformazioni genito-urinarie che
vanno dall’epispadia all’Estrofia Vescicale (EV) classica, all’estrofia cloacale. Rappresenta una delle condi
zioni più difficili in urologia pediatrica. Oltre ai più o meno numerosi e complessi interventi chirurgici ne
cessati per correggere le gravi malformazioni di vescica e genitali, potranno insorgere nel tempo problemi
secondari relativi alla funzione renale, alla continenza urinaria e fecale, alla fertifità, allo sviluppo psicoso
ciale e psicosessuale. E' auspicabile quindi che il paziente estrofico sia seguito, dalla nascita alla pubertà, in
Centri dedicati alla cura di questa rara patologia
Surgery illustrated - Focus on details preperitoneal repair of inguinal hernia
From 2007 to date, fi ve boys with bladder exstrophy underwent this pre-emptive treatment of inguinal hernia at our institution. None has developed a recurrence after a median (range) follow-up of 29 (5 – 46) months. This approach avoids any manipulation of the inguinal canal, which is an advantage per se, but may be even more important in children with bladder exstrophy given the relatively high risk of hernia recurrence reported [ 1,2 ] . Furthermore, the presence of a pelvic diastasis makes the pelvic dissection of the cord easier than in normal children with a closed pelvic ring. The same procedure can also possibly be performed in children with a congenital inguinal hernia undergoing other procedures that require dissection of the perivesical space, e.g. ureteric re-implantation or ureterocoele repair
Onlay on albuginea: Modified onlay preputial island flap urethroplasty for single-stage repair of primary severe hypospadias requiring urethral plate division
OBJECTIVES:
To describe a modified onlay preputial island flap (OIF) urethroplasty to also be used in patients requiring urethral plate transection for penile straightening.
METHODS:
A total of 14 cases with primary severe hypospadias underwent the modified OIF urethroplasty as follows. After skin degloving, the corpora cavernosa were dissected ventrally. The urethral plate was mobilized and, if curvature >30° persisted, transected just proximally to the coronal sulcus. A pedicled preputial island flap was elevated and placed as an onlay to create the neourethra. It was sewn to the margins of the urethral plate proximally and distally. In contrast, in the portion of the shaft without a plate, it was sewn directly to the albuginea of the corpora cavernosa with 2 parallel suture lines. Glanuloplasty and skin closure followed as in standard onlay repairs.
RESULTS:
After a median follow-up of 7 months (range 5 to 27), 3 patients (21%) developed complications requiring additional surgery (1 simple fistula, 1 partial urethroplasty breakdown, and 1 ballooning of the neourethra). None of the remaining patients presented with residual curvature or voiding problems, according to the parental report.
CONCLUSIONS:
The results of the present preliminary experience suggest that OIF urethroplasty can also be performed in cases requiring urethral plate transection. It can be accomplished by suturing the preputial flap directly to the albuginea of the corpora cavernosa. Although we acknowledge that a larger number of cases and longer follow-up are necessary, we believe this technique should be incorporated into the armamentarium of hypospadiologists
When a lymphatic malformation determines a bowel volvulus: Are clinical status and images always reliable?
INTRODUCTION: An acute abdomen in the form of small-bowel volvulus could be a presentation of a lymphatic malformation in childhood.
CASE PRESENTATION:
A 5year old male was admitted to our Institute for an acute abdomen. Clinical aspects and radiological images were not specific for a certain diagnosis. Laparotomy revealed a big soft mass, with a milky content, completely involving about 50cm of ileus with a partial volvulus of the intestinal loop. A complete mass excision and also a bowel involved resection were performed. After a histological examination, a lymphatic malformation was diagnosed.
DISCUSSION:
The diagnosis of a mesenteric lymphatic malformation could be intraoperative and a complete resection should be the treatment of choice. Sometimes it could be necessary to perform an involved bowel tract resection in the case of volvolus with ischemia.
CONCLUSIONS:
Paediatricians and surgeons should bare in mind that an intrabdominal lymphatic malformation may present as a nonspecific an acute abdomen caused by a bowel volvolus and diagnosis may not be so simple preoperativel
Painful orgasm in an adolescent after seminal-sparing cystoprostatectomy: A puzzling symptom
An 18-year-old boy, followed up after seminal-sparing cystectomy for bladder rhabdomyosarcoma, presented complaining of recurrent episodes of left scrotal/inguinal pain arising after orgasms. Full work-up ruled out disease recurrence, but showed enlarged seminal vesicles. Ligation of the vas deferens was unsuccessful. The patient was started on α-blockers to reduce vas contractions with improvement of symptoms. The possible pathophysiology and treatments of this symptom are discussed
[Fistulas of the lower urinary tract in children]
A lower urinary tract fistula consist in an abnormal connection between bladder, urethra and adjacent abdominal organs or skin. There are several types of urinary fistulas in paediatric age and they may be congenital or acquired. Etiology may be due to embriological defects, infectious processes, malignant tumours, pelvic irradiation as well as complications following surgical procedures, especially postsurgical repair of hypospadia or epispadia. Clinical presentation depends on the type of fistula and diagnosis is based on signs, symptoms and radiological or endoscopic examinations. We performed PubMed research using terms such as lower urinary fistulae, urology and paediatrics and we consulted medical texts. We reviewed selected articles and used the relevant ones to perform our study concentrating on classification, diagnosis and treatment of different types of fistulas. Paediatric lower urinary fistulas are an uncommon pathology, but the knowledge of their etiology and classification is important to recognise them and lead the physician to an appropriate treatment, which is surgical in most cases
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