Anorectal Malformations

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[size=20px]Which of the following is most accurate regarding the embryology of anorectal malformations (ARM)? A Most ARM are a result of failure of the rectum to continue caudal migration down to the perineum. B Most ARM are a result of failure of fusion of lateral cloacal folds. C A cloaca is the result of a persistence of the embryological cloaca. D Most ARM are a result of early embryonic perturbation of the cloacal membrane. E Most ARM are a result of prenatal exposure to teratogens.[/size]

[size=20px]A six-year old boy had repair of imperforate anus with rectobladder fistula as an infant. He has experienced persistent fecal incontinence despite adjustments in his laxative and enema regimens. An antegrade continent stoma is recommended. In advising the parents, which complication of an antegrade continent stoma that would require surgical intervention is most likely to occur ? A failure to resolve incontinence B stoma prolapse C stoma leak D stomal stricture E parastomal herniation[/size]

[size=20px]A newborn infant boy has imperforate anus. He is twelve hours old. He has hypospadias and a flat rocker bottom. Meconium was noted in the urine suggesting a urinary tract fistula or high lesion. Which of the following will best help to prevent chronic renal failure in this patient with an anorectal malformation? A early division of the rectourinary fistula B avoiding urinary tract procedures during colostomy C complete work up of the genitourinary tract with long term follow-up D empiric use of prophylactic antibiotics E posterior sagittal anorectoplasty[/size]

[size=20px]A former 38-week gestational age infant girl with a cloacal malformation underwent a divided sigmoid colostomy in the newborn period. Now eight months of age she presents for possible future surgical intervention. A previous cystoscopy and vaginoscopy showed a common channel with a length of one centimeter. The next operation for this patient with cloaca is A no further operation should be recommended. B closure of sigmoid colostomy alone. C laparoscopic urogenital mobilization. D perineal total urogenital mobilization and anorectovaginourethroplasty. E abdominoperineal mobilization of the vaginal, urinary and anal opening.[/size]

[size=20px]Which of the following statements regarding the management and long term outcome of an infant born with cloaca is true? A the incidence of renal insufficiency is less than 15% B the incidence of menstrual obstruction is less than 15% C the incidence of associated gynecological anomalies is less than 15% D vaginoscopy should be performed E decompression of hydrocolpos should be postponed until definitive reconstruction[/size]

[size=20px]A one day old term female is found to have an imperforate anus. She has a smooth ovoid mass in her lower abdomen and over the course of the day develops difficulty urinating. An echocardiogram is normal. A bladder ultrasound shows a distended bladder with a cystic mass behind it. The best next step in management of this patient with an anorectal malformation is A invertogram lateral pelvic radiograph. B upper gastrointestinal series. C cystovaginoscopy. D diagnostic laparoscopy. E diverting colostomy.[/size]

[size=20px]A six-year old male born with imperforate anus and a rectourethral fistula underwent repair during infancy via a posterior sagittal anorectoplasty. He was initially successfully managed with daily enemas but they have become increasingly difficult to administer. The most appropriate management of this patient with a difficult to manage bowel program is A continued daily retrograde enemas. B appendicostomy for antegrade irrigations. C ileostomy. D colostomy. E excision of a megarectum.[/size]

[size=20px]A four-year old boy underwent anoplasty for anorectal malformation with rectourethral fistula. He presents to clinic after being lost to follow-up. Mom reports constant leakage of stool in his diaper. Physical exam reveals a slightly distended abdomen, good position of the neoanus with no stricture and poor gluteal muscles. An abdominal radiograph shows a significant amount of stool and an abnormal sacrum. A contrast enema shows a very dilated rectosigmoid that is full of stool. What is the most appropriate initial management in this child with encopresis after repair of an anorectal malformation? A large volume saline enemas B high dose laxatives C…

[size=20px]A six-month old is undergoing colostomy closure after a posterior sagittal anorectoplasty. Which of the following statements regarding mechanical bowel preparation is correct? A decreases the risk of anastomotic leak B decreases length of hospital stay C promotes early feeding post-operatively D has no effect on surgical site infections (SSI) E must be done as an inpatient[/size]

You are called to see a 36-week old newborn with the anatomy pictured below. [size=20px]Meconium is passing from the prolapsed bowel and urine is noted from the right and left lateral portions of the mucosal mass. Abdominal ultrasound shows two morphologically normal kidneys, two ovaries, two fallopian tubes and two hemiuteri. At the time of operation which of the following is appropriate for this patient with this abdominal wall defect? A appendectomy B ileostomy C use cecal plate for bladder augmentation D use hindgut for vaginal reconstruction E incorporate cecal plate and distal hindgut into fecal stream[/size]

First one with correct answer with justification win. [size=20px]A one-year old girl has experienced chronic constipation since birth. Anorectal exam reveals an anal opening that appears well positioned but is very narrow on digital exam and will only accommodate a number 11 Hegar dilator. An abdominal radiograph reveals a very large fecaloma and a scimitar sacrum. The next best step to recommend to the parents of this child with constipation is A rectal irrigations. B magnetic resonance imaging. C voiding cystourethragram. D rectal biopsy. E posterior sagittal anal repair.[/size]

First one with correct answer with justification win. [size=20px]You are asked to see a 38-week gestation, 2800-gram birth weight newborn boy with no visible anus. He is in no distress but his overall muscular tone is decreased and he has the typical facial features and palmar creases of trisomy 21. The abdomen is mildly distended, soft and nontender. You confirm that there is no anal opening. In this boy with trisomy 21 the most likely type of anorectal malformation is an imperforate anus with A a perineal fistula. B a rectal to bladder neck fistula. C a recto bulbar urethral fistula. D a recto prostatic urethral fistula. E no fistula.[/size]

[center]Treatment of Anorectal malformation according to Talmud, 2000 years ago. [/center]