Frequently Asked Questions (FAQ)
Surgeons, Nurses and Parents send us questions and or suggestions over the Internet or via regular mail. We will be adding from time to time to this section, some of those that we consider will be most helpful, controversial or interesting.
Q: A little girl with recto vaginal fistula. It may be more like a cloacal variant. Not sure...
KB is a little girl repaired with a PSARP in 1996. She has done pretty well overall. A solitary right kidney with dysfunctional ureter and her stooling pattern have been her main problems. I tried instituting early bowel management in her case but I suspect a tremendous motility problem. Her mother informs me that she has 20 stools per day on her BEST day and often has up to 50 or 60 stools per day. I have tried just about everything you ever taught me to manage this but nothing much has helped. Should I try a more aggressive enema program or is it time to consider a colostomy? Let me know what you think. MA MD
A: Dear M: In general a colostomy is at the bottom of my list of alternatives. Prior to that I would like to take her Bowel Management as a challenge and work closely with her & her Mom until she is completely clean. If they could come here I would be very happy to take care of her.
Q: Subject: The Malone procedure for colonic irrigations
A 17 yo with an imperforate anus and subsequent reconstruction has done well for the past 5 years with rectal irrigations every other day. He remains absolutely clean in between irrigations. He is currently a sophomore at College and finds the rectal washouts a bit embarrassing and came to me requesting information about the Antegrade enemas and the surgical implementation. From the recent papers, it is suggested that there is a significant complication rate(stoma dysfunction, stenosis, leakage). He does not have an appendix and his cecum is more toward the midline, periumbilical. DH MD, Hartford, CT, USA though pedsurg-L@UMDNJ.EDU
A: 10% of patients need revision because of stricture of the umbilical stoma. 10% need revision to tighten the valve because it leaked stool. Most patients are very happy.
Levitt MA, Soffer SZ & Peña A: Continent appendicostomy in the bowel management of fecally incontinent children. J. Pediatr. Surg. 32(11):1630-1633, 1997
Q: Subject: VATER SYNDROME forwarded by the Pull Through Network
I recently read your paper on explaining the Vater syndrome on the pullthrough news page. Thank you for this paper. To me and my wife it underlined a number of our thoughts as well as to put some aspects in perspective. Our first born(now approaching 5) is a typical VATER with all the abnormalities and some more, but with an excellent prognosis. Two problems however persist in our quest to improve her quality of life. Firstly of a physical nature - she is very susceptible to lung infections /coughing/ throat irritation. It is very difficult to establish which happens first, but inevitably things end up in infections in the lungs. Apparently her trachea has limited support structure causing a very hoarse sounding cough and great difficulty to get rid of mucus in the trachea. The secondly problem is of psychological nature, and seems to the first associated with the VATER to our experience. She has since about the age of four decided that she is actually a boy - and contrary to carefully explained evidence persists with the conviction. Clinical tests have shown her to be a girl - sonar exploration revealed some uncertainty about the existence of internal female organs although the vagina is shown clearly. She has a posterior anus, and some external vaginal obstruction but has little difficulty with her stools. We are a bit lost with regards to the first problem as all treatment seems to be reactive and totally unsatisfactory. Regarding the psychological problem, we are currently trying to establish what the basis for her conviction is via a child psychologist. Initial assessment points towards irrational thinking IE "men stand pain better and are more brave than women and for the pain I have gone through, it would be better for me to be a man". Generally we would like to link up with the VATER Association, but is uncertain how to and also seek advice, similar cases, experiences in order to tackle our child's problems more informed. Looking forward to hear from you. N & M R (South Africa)
A: It sounds to me that your daughter suffers from tracheomalacya (soft tracheal cartilages) and I'm sure that your pediatric surgeon has ruled out the possibility of a recurrent tracheoesophageal fistula. It impressed me the fact that she is 5 yrs old and there is 'uncertainty' about her internal genitalia and also the fact that she suffers from some form of 'external genitalia obstruction'. Based on my experience I believe that perhaps she was born with a defect called cloaca; underwent repair of her rectum and she still has a urogenital sinus (urethra and vagina together, opening in a single orifice). To expand on this, I would like to read a copy of her medical records and to see all her X-Ray films.
Q: Relapse prostatic rhabdomyosarcoma - 8 yr old boy with prostatic rhabdomyosarcoma confirmed by transrectal needle biopsy was treated by QT for 3 months. Had a prostatectomy after qt. Histology = no viable tumor. Kept on QT for six months and no RXT. CT-scan one year later showed liver nodule and possible tumor at prostatic area. 2nd look - liver nodule excised = Benign; no tumor at the prostatic region. Six months later after CMV infection nodules in the spleen; guided needle biopsy = only blood, no splenic tissue or tumor. Now because of dysuria a new Ct showed a tumor growth of about 3 cm at the prostatic region and persistent nodules in the spleen. As he had two operations by retropubic access I was thinking in operate by posterior approach, and leave the splenic nodules to be biopsied only after postop. QT. Do you agree with this treatment? Any other suggestions? FC MD - Brazil
A: I have done resections of rhabdomyosarcoma of the prostate via trans-anorectal. It provides an excellent approach. Prepared the bowel pre-op with Golytely in a strict manner, inserted a central line and obtained a consent for a colostomy. However, things went so well that I decided not to open the colostomy, kept the patient 10 days with nothing by mouth and parenteral nutrition. I was able to resect the entire posterior urethra with margins free of tumor. Preserved urethral junction with the bladder.
REPLY: Thank you for your E-mail. I operated the boy last week by posterior sagittal approach. The access was excellent it was possible to resect the tumor with 2.5 cm of urethra inside the mass, and to anastomose urethral stumps without tension. Instead of transect the rectum I did as you suggest us to do in re-operation for imperforate anus. I didn't perform a colostomy and keep the patient NPO for five days. So far he is OK. Thank you FC - Brazil
Q: "Surgical Considerations. RE: long narrow fistulae."
A: Real long narrow fistulae are extremely rare. Most times what looks like a narrow fistula is actually a perineal fistula with the rectum located very close to the perineum but compressed by the sphincter mechanism simulating a long narrow fistula. A distal colostogram done in a child with a normal anus and rectum (colostomy done for other reasons) may resemble a HIGH IMPERFORATE ANUS' due to the effect of the sphincter mechanism that keeps the anal canal closed. If a patient has a normal sacrum he/she should have normal bowel control. The rectum is intimately attached to the urethra, one must always put a catheter during the operation and be very careful while dissecting the anterior wall.
Q: I need ed your advise in this case !!! Post-op (10 th day) male patient with Imperforate anus and recto-urethral (bulbar) fistula. Uneventful operation (I was very happy with everything). He is eliminating urine (very little) leakage, generally when the patient is crying at the anterior perineum !!! I re-catheterize bladder with a Foley catheter and I was planning to let it in place for 10-15 more days. Any suggestion will be more than welcome LEP - Venezuela
A: Put a percutaneous suprapubic cystostomy tube and let it rest for one month. After that, perform a suprapubic cystourethrogram and tell me the result. In no leakage after one month, start clamping the tube and eventually take it out.
Q: Opinion as to what might be potential causes of incontinence after a Soave pull-through for Hirschsprung's:
late protracted presentation?
technique - very low colo-anal anastomosis?
leak - stricture?
technique - axonotmesis of pelvic enervation? TL 04/98
A: Those patients have a combination of poor sensation due to a damage to the anal canal and (perhaps more important) a very increased colonic motility due to loss of the natural reservoir (rectum).
Q: I wanted to get the current opinion, ideas, and/or recent experiences with PSARP in patients with cloacal exstrophy. I have a 12-month-old who has done very well with his urinary reconstructions and has good motor function of his lower extremities. His bottom is "moderately" flat, but his stools are very well formed. I thought I would offer a pull through and hope by using a vigorous bowel management program he would do well. Any input would be appreciated. (WDV)
A: The question of whether or not a cloacal exstrophy patient is a candidate for a pull through is primarily defined by the amount of colon the patient has. If there is an abundance of colon, a pull through is warranted, and perhaps, some could even be used for a bladder augmentation or a new vagina. If the colon is short or has been used for urinary reconstruction already, a pull through is a bad idea, and the patient should have a stoma for life. The difficult decision is when there is an intermediate length of colon. For those patients, we would recommend instituting a bowel management program through the stoma. If the patient can be kept clean for 24§ on a regular basis with bowel management, then they are a candidate for a pull through. If bowel management is not successful, then you will make them miserable with a pull through. We feel that the optimal age for attempting this is 2-4 years. Younger than that probably does not make much sense, since most 12 month old would much rather stool through a colostomy than receive an enema every day. The fact that your patient's stools are well formed is obviously a good sign. We agree that a PSARP is the best approach for the unusual cases in which the patient is to be reared as a male, but for females, an anterior sagittal approach has worked very well for us. The exposure is excellent. At the same time that you perform the pull-through, you can create (in genotypic males), or repair (in females) the vagina, close the bladder neck, augment the bladder and the create a Mitrofanoff continent diversion for intermittent catheterization. A. Peña, A. Hong
Q: We have a patient with prune belly syndrome who is 4 years old and is basically enema dependent. Would the ace procedure benefit this type of patient? Anyone with this type of experience? J M
A: The ACE procedure may help him. I consider very important to offer the ACE procedure to a patient ONLY after we have demonstrated that the enemas work and keep the patient clean. We have evidence that indicates that some times the ACE (Malone) procedure is performed without a previous demonstration that enemas work. Remember the ACE is only the creation of a different route of enema administration (1).
(1) Levitt MA, Soffer SZ & Peña A. Continent appendicostomy in the bowel management of fecally incontinent patients. J Pediatr Surg 32(11):1630-1633, 1997.
Q: Imperforate Anus + perineal mass + hypospadias forwarded from pedsurg-l@UMDNJ.EDU
A: PERINEAL LIPOMAS are common in anorectal malformations, particularly in cloaca cases. When they are located in the midline, they split the sphincter mechanism. One can resect the lipoma in a wedge fashion, protecting the sphincter located on both sides of the midline. Subsequently, after the lipoma is resected the sphincter is re-approximated on the midline. I don't think the skin of the lipoma will have good blood supply to act as urethral tissue. In addition, skin is not good as a long term replacement tissue for urethra. Suggestion: repair the hypospadias before the colostomy is closed, particularly when dealing with a perineal hypospadias.
Q: Tetralogy of Fallot + Cloaca from forwarded from pedsurg-l@UMDNJ.EDU
A: I agree that the tetralogy should be repaired before the cloaca. The precise determination of the genitourinary area pre-op is rather difficult and not vital. The priority in a baby with a cloaca, before the main repair us to be sure that the urinary tract is well drained; meaning: no hydronephrosis, no kidney infections, and therefore no kidney damage. With a ureterostomy and CIC you can achieve that. Also, one should not leave dilated vaginas without drainage because they become infected (pyocolpos) and permanent damaged. Make sure that the CIC is effective in draining the vagina; you may take a pelvic ultrasound before and after the catetherization is done by the mother. If not working well, she needs a vaginostomy. The idea of re-implanting the ureters is well taken. However, during the repair of the cloaca frequently we have to dissect between the vaginas and the bladder and most likely we may have to take down the re-implant, the scar of the re-implant may be in the way.
Q: What's the best dosage of Milk of Magnesia (MOM) for patients post repair of imperforate anus?
A: MOM (Milk Of Magnesia) should be adjusted by trial and error. Dose should be individualized. DO NOT GO BY THE BOOK if a patient was born with imperforate anus.
Q: How can you tell that a patient is expelling all the stool with the enema?
A: There are only two reliable ways to know whether or not the patient is emptying the rectum:
1- Rectal exam
2- X-Ray of the abdomen
However, usually parents develop a sense, just by observation and they can say whether the colon is clean or not. Dark, sticky stool, means constipation and therefore Pt. Needs more laxative. If in doubt, take an x-ray of the abdomen.
Q: Hirschprungs and Imperforate anus. McC - Belfast, N.Ireland
A: I am very skeptical about the diagnosis of Hirschsprung's disease and imperforate anus. My answer is not going to be 100% accurate because unfortunately, we do not have enough data. Basically, I can see that the literature mentions that Hirschsprung's disease seems to be frequently associate to imperforate anus. I now have the records of 1,160 patients born with imperforate anus operated on by me. On two occasions, I was told that two of my patients had Hirschsprung's disease. Both patients underwent a colostomy closure and because they suffered from constipation, the local doctors took a rectal biopsy which showed no ganglion cells and therefore, they went for an abdominal perineal resection and pullthrough. There is no question that the constipation problem is then taken care of but patients then become totally fecally incontinent. Even when those biopsies showed no ganglion cells, I do not necessarily believe that those patients have Hirschsprung's disease.
The reason: I have never seen patients with imperforate anus, constipation and enterocolitis. You know very well that a patient with Hirschsprung's disease not only suffers from constipation but they have many other functional disturbances. Those are very sick children and as a matter of fact, 50% of them died before the age of 5 if we do not treat them. Patients with anorectal malformations and constipation are not sick, they are impacted but they do not have the other functional problems that patients with Hirschsprung's suffer from, mainly enterocolitis. I can not deny that perhaps some patients have Hirschsprung's and imperforate anus but I suspect that there are many patients who have an over-diagnosis of Hirschsprung's disease. Most patients with anorectal malformations who underwent a procedure that included the preservation of the original rectum, will suffer from constipation and Megasigmoid. Most pediatric surgeons will take a biopsy and it makes sense to expect that many of those biopsies are going to show no ganglion cells when taken from a bowel which has been widely dissected and perhaps, suffered ischemia.
Q: What should I do to have my child treated by you at your hospital? Rmc., LA
A: Our protocol of procedures requires that you send copies of all medical records and x-rays to our office. After reviewing these records, we will contact you, indicating our plan and suggestions. In the event that your child needs to be treated in New York, we will arrange accommodations for the family and the patient, at The Ronald McDonald House, located within walking distance to our hospital.
P: Cu l es el procedimiento para referir una paciente con cloaca a su hospital en Nueva York:
R: Para una paciente que necesita una reparaci¢n de cloaca. Nos gustar¡a mucho ver o, mejor, hacer personalmente los siguientes estudios:
R-X del sacro y columna lumbar AP and lateral
Colostograma distal con MEDIO HIDROSOLUBLE
Colostograma PROXIMAL con MEDIO HIDROSOLUBLE
Cystograf¡a
Ultrasonido renal
Viendo estos estudios podr¡a contestar preguntas relativas al pron¢stico funcional de la paciente y el manejo subsiguiente. Si es necesario tratar al paciente en nuestro hospital, nosotros haremos reservaciones en el Ronald McDonald House, a pocos pasos del hospital, para hospedar el/la paciente y la familia.
Q: Subject: Imperfored anus and graciloplasty. My daughter was born in 1982 with a hight imperfored anus. She had a colostomy from her birh to 1984 , then she has been operated to make continuity with no effect on her continence. At 12 years old a repair of the anus failed because it was made without colostomy Six months later the last attempt of repair (under colostomy) was not very succesfull and she is still incontinent. She cannot handle with it (and doe not want enemas) and asked us for intervention. The doctor (in PARIS) proposes a graciloplasty with electrical stimulation. My daugher wants to know the chance of sucess for graciloloplasty and if there is a lot of experiences of such intervention on people with imperfored anus (with several op,rations on the anus) . She also wants to know the suits of the operation (difficulty of reeducation) and what happends in case of failure. Thanks . J and A F.(France)
A: The concept of "High Imperforate Anus" in a female is a conflicting, controversial one. The enormous, greatest majority of female patients that were classified as "High Imperforate Anus," in the past, in reality suffered from a persistent cloaca. In other words, urethra, vagina and rectum are fused together opening in a single orifice in the perineum. Most surgeons repaired the rectal part and left the patients with a urogenital sinus (vagina and urethra together). The patients remained asymptomatic until puberty, it then became obvious that the vagina was still attached to the urethra and the patient did not have two orifices (vagina and urethra), but rather only one and needs another operation. I am not saying that your daughter has that, but one must have a strong suspicion and therefore, she needs an examination under anesthesia to rule out that condition. Otherwise, there is no explanation for such a diagnosis as "High Imperforate Anus."
Graciloplasty is an old operation, consisting in the use of a muscle called gracilis that is a long one, located in our thigh. Somebody had the very good idea of surrounding the lower rectum with that muscle and then train the patient to contract that muscle to simulate the function of a new sphincter. That operation was abandoned because it didn't usually work. Recently, the procedure has been revived by a number of surgeons who believe that by connecting an electrical current to that new sphincter, it may develop a tone that will be able to prevent fecal incontinence. I personally remain skeptical about the results of that treatment. First of all, if one reads the results presented by the authors, they still have a significant number of patients in which the procedure doesn't work. Second, we don't know the long term effect of such operations. We must keep in mind that those patients have a foreign body (electrical device), which usually gives problems later in life. However, the main reason why I am skeptical about that procedure is because one must keep in mind that bowel control depends on three factors: a) sensation b) sphincter and c) colonic motility. That operation (dynamic graciloplasty) is only addressing one particular problem (sphincter) but not the other two. If a patient has no sensation, usually it is useless to have a sphincter. But more important perhaps, is the fact that there are two types of patients who suffer from fecal incontinence: a) those who suffer from incontinence and constipation and b) those who suffer from incontinence and tendency to diarrhea. The creation of that kind of new sphincters in an indiscriminate manner, without classifying these patients and identifying the type of incontinence that the patient has, may have severe secondary effects. In other words, creating this new sphincter in a patient that suffers from constipation is going to make the problem worse. The activation of the sphincter may hold the stool inside the rectum, but the deactivation of the sphincter doesn't mean that the patient will be able to empty the rectum and the patient may need an enema to empty the rectum. On the other hand, with an enema, we keep all of our patients completely clean without the need of a new sphincter. For all of those reasons, I remain cautiously skeptical about the results and I would prefer to wait a while before I made a conclusion about those procedures. At present time, what we are doing with patients with fecal incontinence is to follow a specific protocol. AP M.D.
Q: I had the pleasure of taking your last anorectal malformation course, and I wonder if you could give me your advice. This is a now 8 month old boy, 14 lbs, born with an anorectal malformation as part of a VATER complex (mild pulmonic atresia, solitary kidney), as well as hydrocephalus as part of a Chiari malformation. There has been no bladder dysfunction, and the child had a normal VCUG. The child was treated at birth by another surgeon with a loop sigmoid colostomy, which has been functioning well. The perineum is fairly well developed, with a relatively normal appearing gluteal crease, although the previous surgeon was not able to elicit much sphincter tone when stimulation was attempted at the time of colostomy. I studied the child with a colostogram with finding of (I believe) a rectovesical fistula. The dye went extremely easily into the bladder, and a short fistula joined the rectum to the bladder. I doubt this is a high prostatic fistula, but it is difficult to completely be sure. There was a small fecolith in the distal bowel, although the bowel did not appear grossly distended and seemed to be of good length. As well, the child has a very poorly developed sacrum, with a sacral ratio of approx 0.30. I am surprised there appears to be no bladder dysfunction. My question to you is how to properly counsel the family, given such as diffucult malformation with a poorly developed sacrum. I have initially told them that this case has a low chance of satisfactory functional bowel control, even with a technically satisfactory reconstruction. Is it worth it to pull-through this child, with aniticipation of initiating of an involved bowel program for long-term control? Any other information which would be of help to make a decision to reconstruct (or refer) the patient? HR MD Duke
A: Dear Dr. :Your question has been frequently presented to me in the past. Your observation concerning the poor sacrum and good urinary control is perfectly correct. It is amazing to see that male patients with anorectal malformations preserve urinary control even with an almost absent sacrum. Most of the male patients who suffer from urinary incontinence after an imperforate anus repair suffered some sort of dennervation or damage provoked by the operation. The overwhelming majority of male patients with imperforate anus (perhaps excluding those with complete absent sacrum) have urinary control unless we surgeons do something bad to them.
Female patients are a different story, because there, we deal with cloacas and those patients even with a good sacrum, may require intermittent catheterization 70% of the time when the common channel is longer than 3cm and 20% of the time when the common channel is shorter than 3cm. Cloaca patients suffer from some sort of myopathic bladder malfunction that incapacitates them to empty the bladder, but fortunately they do have a good bladder neck most of the time, and therefore, those patients remain completely dry when subjected to intermittent catheterization.
Concerning the bad prognosis for bowel function in your patient: Assuming that you are right in that your patient has a bladder neck fistula (and I believe that you are right) then your patient operated by me would have about a 15% chance of having voluntary bowel movements by the age of three. In addition, if the sacrum is very abnormal, the chances are even lower. Therefore, most likely, your patient is going to be fecally incontinent and yet I personally believe that it is worthwhile to repair the malformation and close the colostomy. The reason; these patients can have a very good quality of life when subjected to a bowel management program consisting in the use of an enema every day that keeps them completely clean for 24 hours. The overwhelming majority of patients insist that the quality of life achieved with the bowel management is far better than the one with a colostomy, particularly during the crucial years of the childhood. During the last 14 years I have done only 7 permanent colostomies, mainly in patients in whom the bowel management was not successful due to the fact that they suffer from severe diarrhea and we were incapable of keeping them clean with an enema everyday. Remember, even in patients with spina bifida, we don't open colostomies systematically because the bowel management gives them better quality of life.
Thus, at present time, the only time I indicate the opening of a permanent colostomy is in a patient that is incapable of forming solid stool. For instance, patients who are subjected to different kinds of colon resection for different reasons or suffer from intractable diarrhea; in those patients the bowel management is not successful. In other words, I still believe that patients with very poor prognosis, with no sphincter whatsoever, with absent sacrum, and even with myelomeningocele, they should receive the pull through and a colostomy closure, provided the bowel management is done correctly. The problem that I see is that most surgeons are not interested in learning how to implement the bowel management because it takes a significant amount of time and dedication. AP
Q: Subject: TCA - I presently have two patients with total colonic aganglionosis who are awaiting definitive operative therapy; our faculty is divided, vocally and viscerally, over what operation should be employed. I respectfully ask for the readership's opinion. BG MD, SUNY, Brooklyn, NY.
REPLY 1: Dear B, I would do reversed Rt. sided colon patch with preservation of the ileocecal valve, and ileum pullthrough as Duhamel's, it seems a good operation !!! Dr. BBK - Jordan
A: Concerning TOTAL COLONIC AGANGLIONOSIS, I have the feeling that we are not curing this condition and patients that are affected by this suffer a great deal. The anastomosis of terminal ileum to the anal canal produces, as expected, many bowel movements that provoke a terrible diaper rash. Surgeons had the idea that using a patch of aganglionic bowel to take advantage of the water absorption capacity of that piece of bowel , they could produce a reduction in number of bowel movements. That happens sometimes, but many other times, that patch produces a significant number of problems. These include: entero-colitis, retention of secretions and stool in the patch, and sometimes produces secretory diarrhea in the patch itself and in the rest of the bowel. We had experienced having to resect several of those different types of patches from patients that suffered from severe secretory diarrhea. This included a patient that lost a large volume of fluid that required 8 liters of electrolyte solution intravenously everyday to compensate with the losses. The moment we resected the pouch the patient improved dramatically.
At the present time, we treat these patients with a straight anastomosis between terminal ileum and anal canal. Considering the timing, there is no question that we can do it very early in life. At present time, it is fashionable to do it as early as possible and even without any sort of diversion. The surgeons that have done this, demonstrated that it is feasible but that does not mean that it is the best for the patient. I personally feel that this operation should be done after the patient is totally continent for urine.
These patients have a terrible diaper rash when that operation is done early in life. The quality of life is something that we frequently do not take into consideration and we do not discuss in our presentations. These patients will suffer enormously from severe rash that provokes even granulation tissue in the perineum. A baby actually doesn't care about having an ileostomy and lives happy with that. It is the parents and the surgeon who care about the baby having an ileostomy. Doing the operation after the patient is toilet trained for urine allows the patient to become toilet trained for stool much faster avoiding the diaper rash.
I would be happy to continue this discussion if anybody wants to participate. A Peña, M.D.
Q: I was concerned with your recipe for normal saline [for bowel irrigations] since it differed from our own. I therefore had our laboratory technician weigh and measure. Here are the results. One heaping teaspoon of salt was weighed 12 times and the range was 7.99 to 10.17 grams. One and three quarters teaspoon of salt had a range of 9.27 to 9.5 grams. If mixed in 1000 ml of water, and the two extremes in each series are eliminated, the percent solution is 0.914 ? 0.045 (for one heaping teaspoon) and 0.938 ? 0.008 for one and three quarters teaspoon. Normal saline is 0.9%. I therefore think the formula for home manufacture should be one heaping teaspoon in one quart of water. even though one and three quarters teaspoon would be more accurate. I do not think it should be one tablespoon which is usually three times one teaspoon. MK MD
A: Dear MK: Thanks for your valuable information. Those who have the time and dedication to check on details are closer to the truth. One important thing is that we frequently change the salt concentration of the enemas depending on the patients response. For instance, if it takes too long for the patient to empty his colon, we may double the salt concentration. If it provokes cramps then we lower the concentration.
Q: I would like to update you on TW [cloaca Pt. ,2 cm common channel, good sacral ratio] and also I have a few questions. She is now 16 mo, and doing well. We have been noticing that her stool frequency is 5-7 soiling a day then 2 to 3 days without a bm.... The other thing is that we have noticed she has nice firm stools and she doesn't expel them all the way out of her anus. (half stool is in and half is still out) It's not an extremely firm stool and she doesn't try to push it out...we usually push down on her low abdomen to "expel" it...and then there is sometimes poopy water that follows... Her diet is now table food...and we make sure she is very balanced in her diet... (constipating food w/ a laxative type food) to even out the process.. I was wondering what your input is on this....and if you have any suggestions. W&K W
A: Dear Parents: What you describe is a problem of constipation. When TW has many bowel movements in a day she is still constipated because she does not empty her rectum well. Give laxative food (see attachment recommended list) and if not enough, give senokot (contact my nurse 718-470-3576). Give only 3 meals; try to be as regular as possible.
Q: KB (baby girl) continues to provide us with the ups and downs that we encounter as surgeons. After I put her back on the bowel management program and had such remarkably good results, she started to have some problems. The surgeon who replaced me tried to examine KB's anus and found that she had developed a skin level stenosis. She had developed a significant prolapse after her initial PSARVUP (cloaca repair) that required a minor revision. She had completed her dilatation regimen and had been doing well. It now seems that she has this stricture/stenosis. The new MD started having Mom dilate once again but things are not going well. KB's behavior has been a major problem. My feeling is that she may need another revision. I am afraid that this far out the scar is too inelastic to be remodeled with a dilation program. Her mom is so frustrated with the problem that she is asking about colostomy and delaying any revision until she is older. I have advised her that we need to be very careful about letting a little girl dictate her surgical and medical care. If you have any comments or suggestions, please let me know. I look forward to hearing from you. MA MD
Follow-up information: It turns out that KB was been taken off the bowel management program started before. I have since been directing her to resume the program and she is actually doing much better. Her solitary kidney with dysfunctional ureter and her stooling pattern have been her main problems. I tried instituting early bowel management in her case but I suspect a tremendous motility problem. Mom informs me that KB has many stools per day. I have tried just about everything to manage this but nothing much has helped. Should I try a more aggressive enema program or is it time to consider a colostomy? MA MD
A: In general a colostomy is at the bottom of my list of alternatives. Prior to that I would like to take her Bowel Management as a challenge and work closely with her & her Mom until she is completely clean. If they could come here I would be very happy to take care of her. If the patient has a stricture and can not be dilated for whatever reason, I would do a small midline posterior anoplasty (Heineck-Mikuliks) and then re-start dilatations.
Q: My son NMcD had the Malone Procedure done in 1997. He was the first case to be done in this region and it was actually done through hiss cecum, but basically the rest of the procedure was the same as the Malone. Unfortunately our network of information is limited. He was born with a high imperforate anus and had no bowel management previous to the Malone. He is six years old and is very active. The procedure has provided him with a wonderful quality of life. The irrigation is done with 1000 ml of saline water and one pediatric fleet, it normally takes for it to get inside 20 minutes and then he sits on the toilet for an hour until he is cleaned out. The problem is that often for some reason the 1000 ml with fleet does not come out for hours and he is left with horrible stomach cramps and he throws up. Last week we had to go to emergency as the water and fleet did not come out for 36 hours and his pain was unbearable. It did make its way through finally. We went through x-rays, barium enemas to see if there was a blockage or any other problems l. There was none. We tried regulating his diet with bran etc and this made this situation worse. Our doctors say they have no idea why this is happening as everything looks fine. This is very frustrating as our son can be up to two hours in pain and then the irrigation just is not successful.
Have you come across this type of situation before? Is there any advice you can give us to help us to get the water through him quickly? We find if we do it faster than 20 minutes it just creates more cramping and does not solve the problem. We have tried adding mineral oil enemas with the regular enemas and I do not see a big improvement.
I look forward to hearing from you and I appreciate anything you can do for us. C & S McD - Dartmouth, Canada.
A: We insist that we never do a Malone operation unless we have already tried the bowel management. It is easier to do a Malone procedure than to implement the bowel management and that is why the easy way is to operate. But remember, the Malone procedure only provides a different route for administration of the enema. Your main problem seems to be bowel management. It would first be necessary to take a look at a contrast enema. When we implement a bowel management program, we do it over a period of one week by trial and error and taking x-ray films every day. If you give an enema through the Malone orifice and the rectosigmoid is impacted, it is expected that the patient will have terrible cramps and will not be able to evacuate. It is important first to dis-impact the colon since the bowel management should start with a clean colon. Therefore, the enema only removes the amount of stool that has been produced for 24 hours. As I mentioned, we implement this for a period of one week and we see the patient every day.
Q: Female patient, 12 year old "VATER" . TEF no problem. For recto-vestibular fistula unfortunately had a PSARP (I think) very unfortunately combined with NIXON flaps. 12 years later has awful appearance showing in particular, rigid scars anterior & posteriorly, rectal ectasia, and rectal exam reveals very short 'anal canal' leading to capacious rectum. The anal canal is very lax....gaping widely on exam. I suspect she requires: colostomy, redo PSARP with tapering of rectum and effectively tightening, placement within sphincter complex and lengthening of 'anal canal'. (combined of course with bowel management).J F Australia
A: My first concern would be to determine whether she has an anus that allows the passing of a #15 or #16 Hegar or your index finger. If that is the case, the next step would be to try to determine whether or not she is continent. In our experience, 95% of our patients that were born with vestibular fistulas and operated with a limited posterior sagittal ANORECTOPLASTY have bowel control. Assuming that she had a good operation I would give her a test consisting of the use of laxatives. Keep in mind that constipation provokes encopresis even in normal individuals and therefore, every time I see a patient that was born with a good prognosis type of defect, mega colon, severe constipation, and "fecal incontinence" our obligation is to rule out overflow pseudo-incontinence. We first dis-impact the patient with enemas and secondly, we keep increasing the amount of laxatives until we reach the amount that makes the patient evacuate the bowel every day, and at that point we determine whether the patient has bowel control or not. If the patient does not have bowel control, he is a candidate for bowel management. We recently published an article in The Journal of Pediatric Surgery on bowel management regarding the basis of our management (1).
On the other hand, if the patient has a stricture in the anus, and I cannot pass my finger, I try to determine the length of the stricture. That can be determined by passing a Foley catheter into the rectum, inflating the balloon, and then pulling on the Foley. That will give you the measurement of the length of the stricture. If it is just a narrow ring, we can treat that easily with a little posterior Y-V plasty. If the patient has a long, narrow stricture then he/she needs, as you mentioned, a colostomy and then a redo posterior sagittal ANORECTOPLASTY. After the reoperation is done, and assuming that the patient does well, you still have to go through the test of laxatives to determine whether she has bowel control or is fecally incontinent. If the patient has bowel control, she will need an enormous amount of laxatives, every day, to stay clean and to stay regular. We then give the patient the choice of resecting the sigmoid to reduce the amount of laxatives needed. On the other hand, if the patient is incontinent, we feel that it is not indicated to resect the sigmoid because it is much easier to manage a constipated patient incontinent with the bowel management.
1.- Peña A, Guardino K, Tovilla M, Levitt MA, Rodr¡guez G & Torres R: Bowel Management for fecal incontinence in patients with Anorectal Malformations. J Pediatr Surg 33(1):133-137, 1998)
Q: My son SP (Prostatic Fistula repaired) is doing well, although still on prunes (2 jars of stage 1 baby food prunes/day), propulsid (3x1.5ml/day), and sorbitol syrup (2x2t/day) to keep things moving. Here, a few issues we don't understand.
(1)Given what we can find in the literature (much of it yours), the web, and from the Pullthrough Network E-mail list. The biggest question we have is whether we should expect our son to always need laxatives, or whether he may be able to drop them eventually. If so, when and how does this happen? Suddenly or gradually? Is there a typical age where they aren't needed? Do your algorithms for the prognosis of continence w/out management include the use of daily medications such as laxatives - or are these algorithms based on diet alone? In my son's case, what part of his 60% chance of continence includes taking laxatives the rest of his life? Are there any tips on timing, quantity, or combinations you can provide? For example, given that the goal is one big poop a day, how do you achieve this w/ diet? Do you give laxative foods at every meal, or a whole bunch at one meal, or perhaps a lot at breakfast and a little bit at lunch and dinner? Should giving juice between meals help keep things moving or is juice between meals bad because it starts peristalsis? In general, is it better to try to control the diet to manage him, or just feed him like the rest of the family and add more prunes, sorbitol, or other laxatives to keep him moving? Is all dairy constipating or just high (dairy) fat foods? Eggs obviously don't have milk fat but are the included in the constipating dairy category? Is there a safe way to test the motility of the colon that is not dangerous and would help determine a child's motility? Should all children with anorectal defects and chronic constipation or diarrhea have their motility tested? It seems like trying to treat the symptoms of the problem w/out knowing the underlying cause would be less successful. For example, a child who is constipated because of slow motility would need laxatives to help push the poop to the rectum. But a child with good motility who has trouble emptying the rectum could use a suppository or enema. In the latter case, wouldn't the risks of laxatives be higher than the risks of the suppository? More to the point, is it a good idea to have my son's motility tested? In your opinion, what are the greatest risks associated with long term use of each type of laxative product - stool softeners, bulk forming products, lubricants, osmotic laxatives, and stimulant laxatives? Since there have been no studies done on long-term (greater than 6 months) regular use of any of these products and probably none will be financed any time soon, are you trying to compile clinical information about long term use of laxatives? Do you have or know of patients that have been taking any of these drugs for 20 years, 10 years, or 5 years? Are you aware of any possible side effects in these patients? Given the lack of information available, do you believe it may be safer to just do enemas? It seems like a saline enema would be less likely to cause major side effects. Would a sigmoid resection be an option to not have to take laxatives or take less for life? Seems like one operation (or even two or three) is less risky than drugs that we don't know the long term effects of. We understand that if too much colon is taken out it would cause diarrhea which would make continence even harder.
(2)From SURGERY OF INFANTS AND CHILDREN. Rectum and Anus Chapter by Paidas and Peña, pg. 1355: "A typical paste used for the diaper rash consists of vitamin A and D ointment, aloe, neomycin, Desitin, and Mylanta. Nystatin is added if the rash is the result of yeast." Is the nystatin in cream or powder form? What is the ratio of ingredients for the paste? Are there any commercial products w/ these active ingredients already mixed? J & K P
A: Most patients that need laxatives will need them for many years. I cannot say if it is for life because I have not followed the patients for more than 18 years. Some patients, we don't know why, may decrease the requirements for laxatives as time goes by. Perhaps, it is the fact that they become sensitive to a certain kind of food and the patient, himself or the family learns what the patient is sensitive to and by giving that type of food, they don't need laxatives.
The great majority of patients, on the other hand, need higher and higher amounts of laxatives as time goes by. After all, a laxative is a stimulant medication and in physiology when you take some sort of stimulant, you will need higher and higher amounts for it to keep working. Always, when you think in terms of laxatives, consider the fact that we order laxatives because we do not have a better alternative. The consequences of not taking a laxative when indicated are much worse then the secondary effects of the long term use of laxatives.
If a patient that has a 60% chance of having voluntary bowel movements, suffers from constipation and does not receive adequate treatment (laxatives), the 60% chance may become 0% because he will suffer from fecal impaction and overflow pseudo incontinence. There are very few tips that we can provide with regard to timing, quantity or combinations of food laxatives. We have a list of foods that have a tendency to produce laxative effects and another list of foods that tend to produce a constipating effect. More important than that list, is what I call individualities or idiosyncrasies. You will become the expert on your own son and will know exactly what type of food will provoke laxative effect and how many hours after the ingestion of that food the effect will be demonstrated. It is a long process of trial and error.
The goal, as you mentioned in your letter, is to provoke 1 or 2 big bowel movements in a day and nothing in between. Sometimes this is impossible. We suggest, just by common sense, that you have more chances to achieve that if you give 3 meals per day and no snacks. Every time we eat something, we produce a reflex called gastrocolic reflex meaning that the colon moves every time we eat something and therefore we try to give only 3 meals of the same quality and preferably at the same time of the day, every day. We recommend that laxatives be given at a single specific time everyday. Again, those are our recommendations. But you have to learn about the individual way your child responds.
In managing constipation, I personally don't believe that we should be very restrictive. I don't think that by giving rice, apples and bananas (which usually produce constipation) have much of an effect in the constipation problem. I rather believe in giving laxative food and laxative medication if necessary and let the child eat freely. You just put on the table what you know is laxative but you don't fight every meal with your child to make him eat something. Let him eat whatever he wants from a variety of laxative food and then you give medication if that food is not enough. Milk and fats in general produce a laxative effect, but we have seen patients in which they produce the opposite. You have to find out in your own patient.
Evaluation of colonic motility is still at a very primitive stage. Some doctors do manometrics of the entire colon but the results that they obtain don't tell us very much.
We look to the future and expect that the evaluation of the motility of the colon will become standard and will become accurate enough as to have a clinical meaning. At the present time, rectal manometry, contrast enemas and even colonic manometry (which just a few people do in the country) are not very accurate tests.
Contact my office for a referral to a Doctor to study your son's colonic motility. The problem is that he would not be able to answer those questions that you raised. He will tell you that the colon of your son doesn't move normally but he is not going to change the treatment that we propose.
I am surprised that you are asking about the use of an ointment for diaper rash. Your child should not have diaper rash if managed in the way we proposed. If you want to know more about the ingredients of those creams and ointments, please contact my office.
The main risk associated with the use of laxatives is to increase the laxative requirement as time goes by. But I insist, the risks of not taking a laxative in a patient that needs laxatives are much worse and eventually the patient may need higher doses of laxatives because the colon becomes dilated in cases of constipation. Remember: constipation produces a dilated colon and a dilated colon produces more constipation. If you want to know the potential secondary effects of each one of those laxatives, we could provide the medical literature, or you can consult the Physician's Desk Reference (PDR).
There was some concern about potential malignancy after the use of Senokot for many years. The government promoted research concerning that issue and recently they came to the conclusion that there is no risk in the long term use of Senokot. We don't have patients that have been taking laxatives for 20 years but we have some that have been taking for 10 years or 5 years and the only thing I can tell you is they may need more laxatives as time goes by. The use of enemas are not necessarily to replace laxatives. Enemas are used in patients who have demonstrated that they suffer from fecal incontinence. A saline enema is the most natural way to clean the rectosigmoid but many times it is not powerful enough to achieve its goal and we have to add phosphate or more salt. We cannot answer all of your questions except by saying that we never heard of a patient that had a demonstrated cancer or other problem because of the use of laxatives.
A sigmoid resection is indicated to reduce the amount of laxatives and sometimes to eliminate the use of laxatives; but I only recommend that in patients that have demonstrated bowel control. If the patient has no bowel control and you do a sigmoid resection, then the bowel management consisting in the use of enemas is going to be much more difficult. Keep in mind that it is much more difficult to treat a patient with fecal incontinence and diarrhea than fecal incontinence and constipation.
We dream about the creation of an Institute for the investigation of colorectal problems in children; the job of the team who will work at that Institute, would be to try to answer all of your questions.
