Anorectal Malformations

THOUGHTS ON DISCLAIMER FOR TREATMENT SERVICES OF CONSUMER AT FULL AGE WITH ANOREXIA AND/OR BULIMIA NERVOZA AND DUE TO THE POSSIBILITY OF INVALIDITY THE RESULTS CONCERNING CONSUMER LAW
Assist. Prof. Özlem Tüzüner (Ph.D.)
ABSTRACT
Anorexia and bulimia are two mental illnesses examined under the umbrella of the so-called eating disorders. Anorexia is the corruption of body mass perception of the person who refuses to eat. Thus the body mass falls far below what is required. Bulimia, on behalf not to gain weight or lose weight, is eating two to three times than the normal human food at one time and inducing to vomit as self-conscious. In both two disorders, can be found other anxieties in addition to the fear of weight gain. Also they may be accompanied by the abuse of diuretic, laxative and other diet drugs accelerating metabolism. Strong tendency to self-slaughter, among anorexia and/or bulimia patients, there is high mortality rate. It is listed as causes of death in the medical literature: excessive hunger, excessive thirst, melting of the muscles including the heart muscle, endocrine disruption, interruption in nerve conduction, deviation in the appetite center, urea increase, fever based on extreme constipation rise and electrolyte imbalance.
Any anorexia and/or bulimia nervosa patient who comes to hospital with high risk of death, his/her declaration of the absolute rejection for treatment is null and void. Because of the high risk of death due to taking the step to self-destruct stage, from now on the anorexic and/or bulimic person, to realize the implications of that treatment is not possible. Through the principle of relativity in the power of discernment, the anorexic and/or bulumik person whose death approaches as a serious risk has no power to understand the consequences of not being treated.
When the anorexic and/or bulimic person is unconscious, under full age or limited by a court decision, there are legislations/provisions in Turkish Law, which serve the urgent medical intervention to those patients. However, about the minor and slightly conscious anorexia and/or bulimia nervosa patient who stepped into fatal stage, when his/her declaration of the absolute rejection for treatment is null and void, there occurs some important results welded the integrity rule as well as general provisions. As the disclaimer to treatment service of the anorexic and/or bulimic person is invalid, most important results occur from consumer law. The results concerning the consumer law, especially liability for compensation, are examined below in the comparative law perspective.
Keywords: Anorexia, bulimia, power of discernment, invalidity, integrity rule, obligation to save life, obligation to contract and liability for compensation

Colonic atresia and anorectal malformation are rare congenital anomalies individually. Few reports of the conditions combined in a single patient have been published in the literature. Neither colonic atresia, anorectal malformation or a combination of the disorders has previously been reported in the Haitian population.

This term is coined by Dr. David Smith in the 1960’s. Dysmorphology is the study of abnormalities of human form and the mechanism that cause these abnormalities. It is estimated that 1 in 40 or 2.5% of newborns have a recognisable malformation or multiple malformations at birth.

Anorectal malformations are associated with other anomalies among which vaginal malformations are occasionally encountered and may go unnoticed by the primary physician. Between January 1998 and December 2003, 563 cases of anorectal malformations were managed in Pediatric Surgery Department, Chittagong Medical College & Hospital, Chittagong, Bangladesh. Among these, five cases of rectovestibular fistula were associated with vaginal malformations. In this retrospective study age, physical findings, operative findings and procedures, outcome of operation and postoperative follow-up were evaluated. Mean age at presentation was 8.67 years (range 2 months to 17 years). All cases referred as rectovaginal fistula and vaginal anomalies (atresia to agenesis) were detected after thorough examination. Initial pelvic colostomy was done in all patients. Cases 1, 3 and 4 had distal vaginal agenesis and underwent posterior sagittal anorectovaginoplasty. In case 5, atretic vaginal duplication was found with didelphic hypoplastic uterus and absent left kidney. Case 2 (vaginal atresia) operated elsewhere is waiting for definitive surgery. Colostomy closure was done in four cases. The third patient had already married and conceived. Bowel habits are regular in all except the second patient. In females, a thorough understanding of anorectal malformations is necessary to identify the association with vaginal anomalies and awareness of this association will lead to earlier diagnosis and appropriate operative measures.

Introduction: Anorectal malformations include a wide spectrum of congenital defects of the anus, anal canal and rectum, which are often combined with urogenital tract defects. They are often associated with congenital anomalies of other organ systems such as genitourinary, cardiovascular, digestive, skeletal, etc. Objectives: This study aimed to review the patients with anorectal malformations, diagnosis, different types of associated anomalies and their frequency, modalities of treatment, results of treatment and complications. Materials and Methods: Since 2010, we have performed a combined retrospective and prospective analysis of a total of 76 patients with anorectal malformations which were diagnosed and treated at our clinic between 2005 and 2014. Results: Of 76 patients with anorectal malformations, 48 were male (63.16%) and 28 female (36.84%).

Purpose: The purpose of the study was to review a rare anomaly of anorectal malformations in girls, congenital anovestibular fistula (AVF) with normal anus, over a 12-year period. Materials and Methods: A total of 24 female patients of AVF with normal anus were treated in the

Cases of congenital double anus are very rare. The number of cases of hindgut duplication of all kinds is slightly higher, and some series include patients with genitourinary and genitalia duplications but with a single anus. However, double termination with tubular duplication is rarely heard of. We report on the case of a patient with 2 well-formed ani, total colon duplication, and 2 vaginas. D

This research explores the experiences of 18 gifted high school students career decision process which took 2 years 8 months. The students were from three different schools in İstanbul, and they had been taking career counseling in order to decide about their majors in their university years. The interactions with the students provided the data to answer the research questions, ''What are the difficulties, if any, to choose the right career path after the high school years, particularly the major that they want to study at the university?'' The data were gathered through in-depth interviews during the therapies including, the preparations of the documents for overseas applications which were recorded, transcribed verbatim, coded, and analyzed for patterns. The patterns became the prevalent themes for discussion in the findings and analysis.

OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed. Bowel function and LUTS were assessed by questionnaire. Patients with extensive sacral anomalies or meningomyelocele were excluded. RESULTS Of 89 patients (median age 15 years, range 5-29 years), MRI was available in 90% (n = 80; 40 male patients with RUF), and 80% of patients returned the questionnaire (n = 64; 31 male patients with RUF). Spinal cord anomalies were found in 34%, comprising a filum terminale lipoma in 30%, low conus ...

OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed. Bowel function and LUTS were assessed by questionnaire. Patients with extensive sacral anomalies or meningomyelocele were excluded. RESULTS Of 89 patients (median age 15 years, range 5-29 years), MRI was available in 90% (n = 80; 40 male patients with RUF), and 80% of patients returned the questionnaire (n = 64; 31 male patients with RUF). Spinal cord anomalies were found in 34%, comprising a filum terminale lipoma in 30%, low conus ...

Ano-rectal diseases are arrays of disease conditions occurring within the rectum and anal canal. Diseases of the rectum and anus are common and affect a large proportion of the population. It is thought that most patients with symptoms referable to the ano-rectum do not seek medical attention and many prefer to seek alternative therapy. Most ano-rectal diseases can be diagnosed by detailed history, physical examinations and when necessary ancillary investigations. Correct diagnosis is mandatory for proper management.

Anorectal malformations (ARM) are rare abnormalities that occur in approximately 1 in 3000 live births with around 40% of patients presenting with isolated forms. Multiple familial cases reported, suggest underlying genetic factors that remain largely unknown. The recurrence in relatives is considered rare, however transmission rates of ARM by affected parents have never been determined before. The inheritance pattern of ARM was investigated in our database of patients with isolated ARM. Within our cohort of 327 patients with isolated ARM we identified eight adult patients from eight families who had in total 16 children with their healthy spouse. Of these ten had ARM, resulting in a recurrence risk of approximately one in two live births (10 of 16; 62%). From 226 families with 459 siblings we found two affected siblings in five families. Hence, the recurrence risk of ARM among siblings is approximately one in 92 live births (5 of 459; 1.0%). Comparing the observed recurrence risk i...

Background: The occurrence of an anorectal malformation with Holt-Oram syndrome, microcephaly, and bilateral corneal opacity is rare and to the best of our knowledge has not previously been reported in the literature. Hence, there is a need to document our experience in this case and learn as much as possible from it. Case presentation: We present the case of a Nigerian female neonate with a postnatal diagnosis of syndromic anorectal malformation associated with Holt-Oram syndrome, microcephaly, and bilateral corneal opacity. The infant had successful staged correction of her anorectal malformation but developed a metastatic Wilms' tumor and died before other corrective procedures could be instituted. Conclusions: An anorectal malformation is here reported to occur with Holt-Oram syndrome, an association that has not been reported previously. To enhance the prognosis and quality of life of children with syndromic anorectal malformation, prenatal ultrasound monitoring of high-risk pregnancies and expertise in prenatal detection of congenital anomalies are invaluable in antenatal care.

Introduction: Anorectal malformations include a wide spectrum of congenital defects of the anus, anal canal and rectum, which are often combined with urogenital tract defects. They are often associated with congenital anomalies of other organ systems such as genitourinary, cardiovascular, digestive, skeletal, etc. Objectives: This study aimed to review the patients with anorectal malformations, diagnosis, different types of associated anomalies and their frequency, modalities of treatment, results of treatment and complications. Materials and Methods: Since 2010, we have performed a combined retrospective and prospective analysis of a total of 76 patients with anorectal malformations which were diagnosed and treated at our clinic between 2005 and 2014. Results: Of 76 patients with anorectal malformations, 48 were male (63.16%) and 28 female (36.84%). Anal stenosis was the commonest type (30.26%) followed by ARM without fistula (25.00%) and rectourethral fistula (18.42%). The most common associated anomalies were urogenital (49.12%), cardiovascular (21.05%), gastrointestinal (15.79%), and bone malformations (5.26%). 34 patients (44.74%) were treated primarily without colostomy. In 40 patients (52.63%) colostomy was performed after birth. PSARP was the procedure of choice in 76.92% of patients. 7 patients (9.21%) died in total. Constipation was present in 52.38% of all patients. Patients older than 3 years of age totally continent were 30.91%. Redo operations were performed in 5 patients (7.94%). Conclusion: Early diagnosis of anorectal malformations is very important. Some malformations such as anal stenosis are sometimes diagnosed beyond the neonatal age. Challenging problem still remains high percentage of children that suffer from fecal incontinence and constipation.

Purpose: Sexual dysfunction and impaired quality of life due to fecal incontinence are common after classic operations for anorectal malformations. We hypothesized that modern repairs may result in improved outcomes. Materials and Methods: Following ethical approval for this single institution cross-sectional study, all patients 16 years or older treated for rectourethral, vestibular or perineal fistula from 1983 onward were sent detailed postal questionnaires on sexual function and quality of life. Each respondent was age and gender matched to 3 controls randomly selected from the general population. Penoscrotal/gynecologic abnormalities were obtained from the records. Results: A total of 41 patients (62%) with a median age of 22 years participated in the study. Of the patients 20 were males with rectourethral fistula (prostatic in 60%), 10 were females with vestibular/perineal fistula and 11 were males with low malformations. Although experience of sexual relationships and orgasmic function were reported in comparable proportions to controls, age at coital debut was significantly delayed in all groups of patients (p 0.046). Erectile function was preserved after sagittal repair but absent ejaculations or azoospermia affected 3 males with rectourethral fistula (15%). Penoscrotal/gynecologic abnormalities affected 12% of patients. Overall quality of life scores were comparable to controls but a trend was found for lower scores on emotional items in males with rectourethral fistula (p ¼ 0.06) and for a negative effect on sexual life in females (p ¼ 0.03). Conclusions: While erectile and orgasmic function appear preserved after sagittal repair, further evaluation of fertility issues in males with rectourethral fistula is indicated. Larger multicenter studies are needed to confirm our findings.

Aphallia or penile agenesis is very rare congenital anomaly of unknown cause occurring 1 in 30 million live births. Very little has been written in literature about aphallia. There is absent phallus and urethra may open abnormally in perineum or into rectum posing various surgical, social and psychological implications as the child grows. We are presenting 03 cases of aphallia with associated congenital anomalies such as unilateral renal agenesis, bilateral undescended testes, anorectal malformation and rectovasical fistula.

Cases of congenital double anus are very rare. The number of cases of hindgut duplication of all kinds is slightly higher, and some series include patients with genitourinary and genitalia duplications but with a single anus. However, double termination with tubular duplication is rarely heard of. We report on the case of a patient with 2 well-formed ani, total colon duplication, and 2 vaginas.

The aim of this paper is to present the case of a female patient, 14 years of age, who developed a megarectum and overflow of fecal incontinence as a consequence of chronic and severe constipation, after the surgical repair of her vestibular fistula at the age one. The huge rectum was resected than dissected down to the pelvic floor and a new anus was reconstructed via a posterior sagittal anorectoplasty with excellent results.

Purpose: Sexual dysfunction and impaired quality of life due to fecal incontinence are common after classic operations for anorectal malformations. We hypothesized that modern repairs may result in improved outcomes. Materials and Methods: Following ethical approval for this single institution cross-sectional study, all patients 16 years or older treated for rectourethral, vestibular or perineal fistula from 1983 onward were sent detailed postal questionnaires on sexual function and quality of life. Each respondent was age and gender matched to 3 controls randomly selected from the general population. Penoscrotal/gynecologic abnormalities were obtained from the records. Results: A total of 41 patients (62%) with a median age of 22 years participated in the study. Of the patients 20 were males with rectourethral fistula (prostatic in 60%), 10 were females with vestibular/perineal fistula and 11 were males with low malformations. Although experience of sexual relationships and orgasmic function were reported in comparable proportions to controls, age at coital debut was significantly delayed in all groups of patients (p 0.046). Erectile function was preserved after sagittal repair but absent ejaculations or azoospermia affected 3 males with rectourethral fistula (15%). Penoscrotal/gynecologic abnormalities affected 12% of patients. Overall quality of life scores were comparable to controls but a trend was found for lower scores on emotional items in males with rectourethral fistula (p ¼ 0.06) and for a negative effect on sexual life in females (p ¼ 0.03). Conclusions: While erectile and orgasmic function appear preserved after sagittal repair, further evaluation of fertility issues in males with rectourethral fistula is indicated. Larger multicenter studies are needed to confirm our findings.