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References

295
urinary tract. If the patient has short colon, obvi­ously the urologist cannot use the colon or even small bowel for the augmentation of the bladder, but rather must think in all the possibilities such as gastric augmentation. If the patient has a normal- sized colon, then the urologist can be more liberal to use a piece of bowel that he wants for the bladder augmentation.
Concerning the surgical management of the colorectal component of the malformation, as well as the genitalia reconstruction, the surgeon must follow the same principles recommended in the management of cloacas and cloacal exstro­phies (see Chap. 16 ).
The functional prognosis of these patients seems to be similar or better than in cases of cloa­cal exstrophy. Therefore, we put together the results of covered cloacal exstrophy and cloaca exstrophy in Tables 17.1 and 17.2 of this chapter.
Our experience includes 28 cases and was reported in 2013 [ 67 ] .
References
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2. Swan H, Christensen SP (1953) Exstrophy of the clo-
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3. Von Geldern CE (1959) The etiology of cloacal exstro-
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4. Rickham PP (1960) Vesico-intestinal fi ssure. Arch
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5. Soper RT, Kilger K (1964) Vesico-intestinal fi ssure.
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6. Spencer R (1965) Exstriphia splanchnica (exstrophy
of the cloaca). Surgery 57:751–766
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cloaca: report of a case treated surgically. Pediatrics 35:687–692
8. Tank ES, Lindenauer SM (1970) Principles of man-
agement of exstrophy of the cloaca. Am J Surg 119(1):95–98
9. Fonkalsrud EW, Linde LM (1970) Successful man-
agement of vesicointestinal fi ssure: report of two cases. J Pediatr Surg 5(3):309–314
10. Markland C, Fraley EE (1973) Management of infants
with cloacal exstrophy. J Urol 109(4):740–743
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Exstrophy of the cloaca. Am J Dis Child 125(6):879–883
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12. Jeffs RD (1978) Exstrophy and cloacal exstrophy. Urol Clin North Am 5(1):127–140
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15. Rieu PN, de Vries JD, Gardeniers JW, Bakker-Niezen SH (1991) Vesico-intestinal fi ssure: a multidisciplinary problem. Tijdschr Kindergeneeskd 59(2):58–64
16. Agugua NE (1991) Cloaca exstrophy. West Afr J Med 10(2):190–193
17. Smith EA, Woodard JR, Broecker BH, Gosalbez R Jr, Ricketts RR (1997) Current urologic management of cloacal exstrophy: experience with 11 patients. J Pediatr Surg 32(2):256–261
18. Radhakrishnan J (1998) Double-barrelled colovagi­noplasty in a patient with cloacal exstrophy variant. J Pediatr Surg 33(9):1402–1403
19. Carey JC, Greenbaum B, Hall BD (1978) The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). Birth Defects Orig Artic Ser 14(6B):253–263
20. Evans JA, Darvill KD, Trevenen C, Rockman­Greenberg C (1985) Cloacal exstrophy and related abdominal wall defects in Manitoba: incidence and demographic factors. Clin Genet 27(3):241–251
21. Keppler-Noreuil KM (2001) OEIS complex (omphalocele- exstrophy-imperforate anus-spinal defects): a review of 14 cases. Am J Med Genet 99(4):271–279
22. Meglin AJ, Balotin RJ, Jelinek JS, Fishman EK, Jeffs RD, Ghaed V (1990) Cloacal exstrophy: radio­logic fi ndings in 13 patients. AJR Am J Roentgenol 155(6):1267–1272
23. Loder RT, Dayioglu MM (1990) Association of con­genital vertebral malformations with bladder and cloacal exstrophy. J Pediatr Orthop 10(3):389–393
24. Cohen AR (1991) The mermaid malformation: cloacal exstrophy and occult spinal dysraphism. Neurosurgery 28(6):834–843
25. Morioka T, Hashiguchi K, Yoshida F, Matsumoto K, Miyagi Y, Nagata S, Yoshiura T, Masumoto K, Taguchi T, Sasaki T (2008) Neurosurgical manage­ment of occult spinal dysraphism associated with OEIS complex. Childs Nerv Syst 24(6):723–729
26. Suson KD, Colombani PM, Jallo GI, Gearhart JP (2013) Intracranial anomalies and cloacal exstrophy–is there a role for screening? J Pediatr Surg 48(11):2256–
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27. Molenaar JC (1996) Cloacal exstrophy. Semin Pediatr Surg 5(2):133–135
28. Vasudevan PC, Cohen MC, Whitby EH, Anumba DO, Quarrell OW (2006) The OEIS complex: two case reports that illustrate the spectrum of abnormalities and a review of the literature. Prenat Diagn 26(3):267–272
29. Yang F (2007) OEIS complex: a case report. J Diagn Med Sonog 23(1):13–18. doi:
30. Tiblad E, Wilson RD, Carr M, Flake AW, Hedrick H, Johnson MP, Bebbington MW, Mann S, Adzick NS
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(2008) OEIS sequence – a rare congenital anomaly with prenatal evaluation and postnatal outcome in six cases. Prenat Diagn 28(2):141–147. doi:
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32. Howell C, Caldamone A, Snyder H, Ziegler M, Duckett J (1983) Optimal management of cloacal exstrophy. J Pediatr Surg 18(4):365–369
33. Diamond DA, Jeffs RD (1985) Cloacal exstrophy: a 22-year experience. J Urol 133(5):779–782
34. Cywes S (1986) Cloacal exstrophy: a long term follow- up with faecal continence. Pediatr Surg Int 1:186–191
35. Gearhart JP, Jeffs RD (1991) Techniques to create urinary continence in the cloacal exstrophy patient. J Urol 146(2 (Pt 2)):616–618
36. Ricketts RR, Woodard JR, Zwiren GT, Andrews HG, Broecker BH (1991) Modern treatment of cloacal exstrophy. J Pediatr Surg 26(4):444–448
37. Hendren WH (1992) Ileal nipple for continence in cloacal exstrophy. J Urol 148(2 Pt 1):372–379
38. Babut JM, Boster D, Lotan G, Mhidia A, Fremond B (1993) Cloacal exstrophy: what therapeutic approach for what result? Pediatrie 48(3):259–263
39. Lund DP, Hendren WH (1993) Cloacal exstro­phy: experience with 20 cases. J Pediatr Surg 28(10):1360–1368
40. Husmann DA, Vandersteen DR, McLorie GA, Churchill BM (1999) Urinary continence after staged bladder reconstruction for cloacal exstrophy: the effect of coexisting neurological abnormalities on uri­nary continence. J Urol 161(5):1598–1602
41. Mathews R, Jeffs RD, Reiner WG, Docimo SG, Gearhart JP (1998) Cloacal exstrophy – improving the quality of life: the Johns Hopkins experience. J Urol 160(6 Pt 2):2452–2456
42. Levitt MA, Mak GZ, Falcone RA Jr, Peña A (2008) Cloacal exstrophy – pull-through or permanent stoma? A review of 53 patients. J Pediatr Surg 43(1):164–168. doi:
43. Sawaya D, Goldstein S, Seetharamaiah R, Suson K, Nabaweesi R, Colombani P, Gearhart J (2010) Gastrointestinal ramifi cations of the cloacal exstro­phy complex: a 44-year experience. J Pediatr Surg 45(1):171–175. doi:
44. Reiner WG, Gearhart JP (2004) Discordant sexual identity in some genetic males with cloacal exstro­phy assigned to female sex at birth. N Engl J Med 350(4):333–341
45. Reiner WG, Kropp BP (2004) A 7-year experience of genetic males with severe phallic inadequacy assigned female. J Urol 172(6 Pt 1):2395–2398
46. Sobel V, Imperato-McGinley J (2004) Gender identity in XY intersexuality. Child Adolesc Psychiatr Clin N Am 13(3):609–622
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47. Reiner WG (2005) Gender identity and sex-of-rearing in children with disorders of sexual differentiation. J Pediatr Endocrinol Metab 18(6):549–553
48. Soffer SZ, Rosen NG, Hong AR, Alexianu M, Peña A (2000) Cloacal exstrophy: a unifi ed management plan. J Pediatr Surg 35(6):932–937
49. Sacher P, Hirsig J, Gresser J, Spitz L (1989) The importance of oral sodium replacement in ileostomy patients. Prog Pediatr Surg 24:226–231
50. Husmann DA, McLorie GA, Churchill BM, Ein SH (1988) Management of the hindgut in cloacal exstro­phy: terminal ileostomy versus colostomy. J Pediatr Surg 23(12):1107–1113
51. Silver RI, Sponseller PD, Gearhart JP (1999) Staged closure of the pelvis in cloacal exstrophy: fi rst descrip­tion of a new approach. J Urol 161(1):263–266
52. Hurwitz RS, Manzoni GA, Ransley PG, Stephens FD (1987) Cloacal exstrophy: a report of 34 cases. J Urol 138(4 Pt 2):1060–1064
53. Mitchell ME, Brito CG, Rink RC (1990) Cloacal exstrophy reconstruction for urinary continence. J Urol 144(2 Pt 2):554–558
54. Batinica S, Gagro A, Bradić I, Benjak V (1991) Cloacal exstrophy: a case report. Eur J Pediatr Surg 1(6):376–377
55. Lee RS, Grady R, Joyner B, Casale P, Mitchell M (2006) Can a complete primary repair approach be applied to cloacal exstrophy? J Urol 176(6 Pt
1):2643–2648
56. Lund DP, Hendren WH (2001) Cloacal exstrophy: a 25-year experience with 50 cases. J Pediatr Surg 36(1):68–75
57. Rosenkrantz JG, Bailey WC, Dumars KW Jr (1964) Incomplete exstrophy of the cloaca. J Urol 91:549–552
58. Johnston JH, Koff SA (1977) Covered cloacal exstrophy: another variation on the theme. J Urol 118(4):666–668
59. Komura M, Tsuchida Y, Honna T, Kamii Y, Kitahara S, Ishizone S (1993) Completely covered cloacal exstrophy: recognition of a new clinical sub-entity. Pediatr Surg Int 8(2):157–161
60. Boemers TML, de Jong TPVM, Rövekamp MH, Bax NMA, van Gool JD (1994) Covered exstrophy associated with an anorectal malformation: a rare variant of classical bladder exstrophy. Pediatr Surg Int 9(5,6):438–440
61. Chadha R, Sharma A, Bagga D, Mahajan JK (1998) Pseudoexstrophy associated with congenital pouch colon. J Pediatr Surg 33(12):1831–1833
62. Lowentritt BH, Van Zijl PS, Frimberger D, Baird A, Lakshmanan Y, Gearhart JP (2001) Variants of the exstrophy complex: a single institution experience. J Urol 173(5):1732–1737
63. Oshita M, Okazaki T, Lee KD, Lane GJ, Yamataka A (2007) Complete covered cloacal exstrophy. Pediatr Surg Int 23(10):1029–1031
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65. Mallmann MR, Reutter H, Müller A, Boemers TM, Geipel A, Berg C, Gembruch U (2014) Prenatal diagnosis of covered cloacal exstrophy. Fetal Diagn Ther 36(4):333–336
66. Goldfi scher ER, Almond PS, Statter MB, Miller G, Arensman RM, Cromie WJ (1997) Omphalopagus twins with covered cloacal exstrophy. J Urol 157(3): 1004–1005
67. Bischoff A, Levitt MA, Breech L, Peña A (2013) Covered cloacal exstrophy–a poorly recognized con­dition: hints for a correct diagnosis. J Pediatr Surg 48(12):2389–2392. doi:
10.1016/j.jpedsurg.2013.08.011

General Principles for the Postoperative Management of Patients with Anorectal Malformations

1 8

18.1 General Care

As discussed in each one of the specifi c anorectal malformations, patients operated with a protec­tive colostomy that underwent a posterior sagittal repair, without opening the abdomen, are usually fed the same day of the operation. The pain that these patients suffer from is minimal. We try to stay away from strong pain medications, such as morphine. The patients feel comfortable once they feel the presence of the parents. We keep them in the hospital for 48 h in order to adminis­ter intravenous antibiotics. We do not have cases of infections after a posterior sagittal anorecto­plasty, performed with a protective colostomy. When the patient is discharged home, we ask them to come back to the clinic 2 weeks after the operation, to start the process of anal dilatations. Occasionally, patients suffer from vomiting after the operation; we believe that it is related with the anesthesia, since we did not perform any abdomi­nal surgery.
When we perform a posterior sagittal anorecto­plasty without a colostomy as described in the chapter on bowel preparation, the patients receive GoLYTELY® gastrointestinal tract. They also receive a PICC line or central venous catheter in order for them to receive parenteral nutrition. We tell the family that the patient will remain with nothing by mouth for
1
(Polyethylene glycol/electrolytes) Braintree Laboratories,
Braintree, MA., USA
1
; in other words, we clean the entire
a period of 7–10 days after the operation; we explain that he or she will not be hungry because we will be providing parenteral nutrition. After 7 days from the procedure, we look at the perineum of the patient. If everything looks well and it seems to be healing nicely, we let them eat. On the other hand, if we have any doubts or we see dehiscences of 1, 2, 3, or 4 sutures, either in the anoplasty, in the perineum, or in the posterior sagittal incision, we continue the period of fasting for 2 or 3 more days, take the patient to the operating room, and resuture the dehiscent areas. Very rarely, we have patients that suffer from a complete dehiscence of the anoplasty without a colostomy. In those cases, we perform a colostomy and wait until the wound is healed, not less than 3 months, in order to reoperate.
Patients who underwent a posterior sagittal anorectoplasty in addition to a laparotomy usu­ally start having po feeds 2–4 days after the oper­ation. That period of time depends very much on the degree of ileus that the patient suffers from, which is related with the manipulation of the bowel during the laparotomy. Sometimes, we operate on patients who had previous multiple operations, and we spend long periods of time in the operating room taking down all the peritoneal adhesions. The longest operation that we per­formed so far, nonstop, has been 18 h in a patient with a cloacal exstrophy in which we were able to repair the rectal component, the vagina, and the urinary tract. Those patients obviously would remain with nothing by mouth for longer periods
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, DOI 10.1007/978-3-319-14989-9_18, © Springer International Publishing Switzerland 2015
299
300
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
of time because they have a prolonged ileus. We followed the general guidelines that most sur­geons follow-up after complicated laparotomies.
Babies subjected to long operations (longer than 6 h) always go to the intensive care unit. We have seen a tendency for them to suffer from respiratory arrest postoperatively.
Occasionally, we operate on patients that came to us with a loop colostomy. As we previ­ously discussed in the chapter of colostomies, some loop colostomies are totally diverting, and we see that the entire stool goes into the stoma bag. However, when those loop colostomies retract a little bit, they pass stool into the distal bowel. When that happens, after the operation, to avoid contamination, there are several maneuvers that may prevent fecal contamination. One can be the introduction of a Foley catheter in the distal colonic limb, with the balloon infl ated for several days, to avoid the passing of stool. Another one is to place a purse string suture in the distal stoma with a heavy, long-term, absorbable suture to pre­vent the spillage of stool into the distal limb.

18.2 Local Care

We do not restrain the movements of our patients. We do not tie them to the bed. We do not keep the legs together. We let them walk around and move in any way they want. However, we encourage the mother to use a double diaper, to serve as a cushion for the bottom. Also, we prevent the patients from jumping and sitting in a rough way after the operation. Because the posterior approach is basically painless, the patients start jumping around, and sometimes they injure themselves. We keep the double diaper for 1 month. We like to use antibiotic ointment on the posterior sagittal incision, the anoplasty, and the area of the genitalia, in cases of female malfor­mations. However, we use this ointment only for 5 days, because we have seen that after that, the patient has a tendency to suffer from fungus infection in the perineum.
We do not believe that the urine contributes to producing dehiscences. In female babies, with a rectovestibular fi stula or rectovaginal fi stula, we
do not leave a Foley catheter. In cloaca patients, the type of urinary diversion that we use postop­eratively depends on the type of cloaca that the patient has (see Chap. 16 ). We leave a Foley cath- eter for 2 weeks in a case of a posterior sagittal anorectovaginourethroplasty and total urogenital mobilization with a short common channel. When the patient has a common channel longer than 3 cm and we enter into the abdomen, we pre­fer to leave a suprapubic tube particularly if we believe that the patient will be able to urinate nor­mally, before 3 months after the operation. When the patient has severe kidney damage, hydrone­phrosis, megaureters, and complex malforma­tions and we believe that it is going to take longer than 3 months for them to pass urine normally, we prefer to leave a vesicostomy.
In male patients with rectourinary fi stulas, we leave a Foley catheter for 1 week. After a week, the patient comes to the clinic, our nurses pull the catheter out early in the morning and ask the par­ents to take the baby to walk around and drink a lot of fl uids, to be sure that the patient is passing urine normally before sending him home. Occasionally, the patient shows signs consistent with burning of the urethra during the voiding episodes; we encourage them to take more fl uids and eventually the symptom disappears. It is extremely unusual to see patients in urinary retention after a technically correct posterior sag­ittal operation. However, if the patient has a poor sacrum, tethered cord, and a very high malforma­tion, we can anticipate that, occasionally, they may have urinary retention. Under those circum­stances, we leave the Foley catheter for a few more days and remove it again. If we already know that the patient has neurogenic bladder, then sometimes we leave a suprapubic tube, in order for our colleague urologists to evaluate the bladder postoperatively with a urodynamic study.

18.3 Anal Dilatations

Prior to the repair of anorectal malformations, the parents of our children receive a piece of paper describing our protocol of anal dilatations (Fig. 18.1 ).
18.3 Anal Dilatations
301
Fig. 18.1 Anal dilatation.
( a ) Protocol. ( b ) Size of dilators according to age
a
14 days post-operative the surgeon will determine the initial size of the Hegar dilator. He will demonstrate how to pass the dilator.
The parents must perform the dilatation twice a day, passing the dilator and leaving it inside for 30 seconds.
Dilatations must be performed before meals.
The baby must be restrained, keeping the knees against the chest.
The size of the dilator must be increased every week, until reaching the desired size, according to the patient’s age.
Once final size has been reached, dilatations must continue twice a day until the dilator passes easily. At that point, the parents must start tapering the frequency of dilatations, guided by the following sequence:
Once a day for one month
Every other day for one month
Every third day for one month
Two times a week for one month
Once a week for one month
Anal Dilatation Protocol
Once a month for three months
Size of Dilators According to Age
b
Age
0 – 4 months
4 – 8 months
8 – 12 months
1 – 3 years
3 – 12 years
> 12 years
(Hegar numbers represent millimeters in diameter)
We encourage the parents of our patients to read the protocol and memorize it, before coming to the clinic 2 weeks after our procedure.
We have been in national and international meetings, in which some surgeons question the need to perform anal dilatations in patients with anorectal malformations. In fact, some surgeons have expressed a negative impression about the use of dilatations, because of the potential psy-
Hegar #
#12
#13
#14
#15
#16
#17
chological trauma produced in the children, due to the pain that we provoke with these dilatations.
As an answer for criticisms related with our protocol of anal dilatations, we always explain that patients subjected to pull-throughs follow­ing old, rather rude, grotesque surgical tech­niques end up having an anoplasty that actually looks like a colostomy (Fig. 18.2 ). We agree
302
Fig. 18.2 Grotesque, prolapsed anoplasty. Does not
require dilatations
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
Fig. 18.3 Postoperative appearance of a technically cor-
rect anoplasty, in a patient with good sphincter mecha­nism. The anus is closed. The patient needs anal dilatation
that in the type of repair, the patients do not need dilatations. However, in patients that are subjected to technically correct surgical tech­niques to repair anorectal malformations, par­ticularly if the patient has good sphincter mechanism and the rectum is placed within the limits of the sphincter, at the end of the proce­dure, as can be seen in the pictures that we show after the surgical repair of each malfor­mation, the anus looks closed (Fig. 18.3 ). This is consecutive to the effect of the sphincter. This is seen in good anorectal malformations subjected to good repairs. If the patient is left like that with a colostomy, for several months, the anus is going to heal that way and is going to suffer from a stricture. We believe that what we do are not really dilatations, but we rather want the anus of the patient to heal with the rectum open, in the way we left it at the end of the procedure. When we do the anoplasty in the way that can be seen in diagrams and pictures in the chapters of this book, one can see that at
the end of the procedure, the anus has the size of a normal anus for the age of the patient. However, when we remove the retention sutures that are holding the anus open, the anus closes by the effect of the sphincter mechanism that surrounds it. If we leave it that way, it is going to heal that way. That is the reason why we believe that it is necessary to follow our protocol of anal dilatations. We emphasized the importance of starting the dilatations 2 weeks after surgery, not earlier, not later. Earlier than that, we would be concerned about damaging the sutures; later than that, we would be concerned about fi nding already fi brosis that would make the dilatation process most diffi cult.
Diffi cult dilatations that produce a lot of pain and bleeding occur when the rectum that was pulled down suffered from ischemia, and in spite of the efforts of the doctors, those cases usually end up having a severe anal stricture.
We defi nitely oppose the routine followed by some surgeons, consisting of trying to avoid pain

18.4 Avoiding Constipation

303
to the patient and booking the anal dilatations under anesthesia once a week. The doctors do that to calm the anxiety of the parents. Under anesthesia, the patient does not complain, and as a consequence, the doctors tend to over-dilate the anus, producing injuries to the suture line. In the following days, the anus is not dilated; the inju­ries tend to heal, producing fi brosis. This is fol­lowed by another traumatic dilatation every week, with more fi brosis. In those cases, we see patients coming to our clinic with severe fi brosis that is impossible to dilate. Because of that, we are very strict about our protocol. In the clinic 2 weeks after surgery, we show the parents how to dilate the anus and we passed a dilator that the baby does not even feel. It is usually a no. 6 or no. 8 Hegar. We do that in order for the mother to gain confi dence performing dilatations. We show the father how to hold the baby with the knees against the chest, to really immobilize him/her in order to avoid showing the mother a “moving tar­get,” which would make the mother more anx­ious. With the baby completely immobilized, the mother passes the dilator well lubricated, leaves the dilator in place for 30 s, and repeats the maneuver twice in the morning and twice at night. We encourage the mother to do the dilata­tion before meals, to avoid the possibility of vomiting and aspiration. We specifi cally tell the parents to increase the size of the dilator by one size per week (1 mm per week) and continue increasing the size until they reach the size that is adequate for the age of the patient. Based on our previous experience, we believe that a normal anal size for a full-term newborn is a no. 12 Hegar dilator, for a 4-month-old baby a no. 13, for an 8-month-old baby a no. 14, and for 1-year­old baby a no. 15 Hegar. Older than that, usually we recommend to go up to no. 16 Hegar dilator (Fig.
18.1b ).
Once the parents reach the adequate size for the age of the patient, we encourage them to con­tinue doing the dilatations twice a day until the dilator goes easily and painlessly. At that point, the parents are supposed to start tapering the fre­quency of dilatations. In other words, they move from the routine of doing it twice a day to once a day, for a month, and then every other day for a
month, twice a week for a month, once every 2 weeks for a month, and then stop the dilatations. We are very emphatic when we explain to the par­ents about the importance of dilatations, and we explain that not following the routine of dilata­tions may provoke anal stenosis that would be impossible to dilate, and the patient may require an operation. When the anoplasty has been done correctly, when the blood supply of the distal rec­tum is good, and when the technique has been meticulous, the dilatations are not really painful.
Most of our patients subjected to primary repairs of anorectal malformations were only a few months old, and it is our experience that those patients really do not have pain during the anal dilatations.
When we reoperate patients that are older than 2 years old, dilatations become a problem, because the patient comes to us already trauma­tized from previous procedures and painful dila­tations. Under those circumstances, particularly when we know that the patients have a bad prog­nosis type of malformation and we are dealing with a reoperation, we actually tried to make the anus a little larger than normal in diameter, being aware that during the healing process, it will decrease a little bit in diameter but still will be adequate to pass stool. This facilitates the dilata­tions and avoids any pain. Older patients are very diffi cult to restrain in order to do dilatations, and it is a more traumatic experience.
18.4 Avoiding Constipation
Constipation is by far the most common sequela seen in patients born with anorectal malforma­tions and subjected to surgical procedures that include the preservation of the rectum. The resec­tion of the rectum in patients with anorectal mal­formations followed by a pull-through of colon certainly may avoid the problem of constipation, but for sure it will produce fecal incontinence. We keep insisting that the rectum represents the natural reservoir of the human being and there­fore must be preserved as much as possible.
Patients with anorectal malformations suffer from a hypomotility disorder of the rectosigmoid,
304
18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
which translates in constipation. The constipa­tion is more severe in patients with lower defects (recto-perineal fi stula, rectovestibular fi stula, and imperforate anus with no fi stula); these patients suffer from more constipation than patients with recto-bladder neck fi stulas. We do not know the reason for that. It is important to remember and to be more proactive and aggressive in preventing and managing the constipation of those patients that are at higher risk. When the patients have been with a colostomy, for long periods of time particularly if it is a transverse colostomy, with­out repair of the malformation, they come to us with a severe megarectum and sometimes fecally impacted, they suffer from more severe constipa­tion, and it is important to anticipate that to avoid aggravation of the problem.
Avoiding constipation must start when we open a colostomy; we insist on the importance of cleaning the distal colon at the time of the open­ing of the colostomy and to keep the colon col­lapsed from day one. In addition, we try to avoid transverse colostomies and try to repair the mal­formations as early as possible to avoid dilatation of the distal end.
We, as well as the parents, must be ready to deal with the problem of constipation after the colostomy is closed (in patients with colostomies) or after the main repair, when the repair is done without a colostomy. We have long conversations with the parents of our patients and try to make them paranoid against constipation. We tell them that constipation produces fecal incontinence. We know that it is actually overfl ow pseudoinconti­nence, but we try to make the parents aware of the importance of taking care of the constipation. In the chapter of bowel management, we described a group of patients that had a good repair, of a good prognosis type of malformation, but did not receive care for constipation; as a consequence, they suffer from severe fecal impaction, megarec­tum, and fecal overfl ow pseudoincontinence. We try to avoid that in our patients.
The parents take home a little glycerin enema to be used only if necessary. The golden rule is to be sure that the patient passes stool every day. In addition, the parents must develop the sense that the patient is really emptying the colon. Many
persons do not know that passing stool does not necessarily mean that the patient is not consti­pated. Sometimes patients can pass many bowel movements, but they do not empty, and therefore, they suffer from severe constipation. If the patient goes one entire day without a bowel movement, before the baby goes to sleep, the parents must give the small glycerin enema and must increase the amount of laxatives or will start the adminis­tration of laxatives if they have not given laxa­tives yet. We prefer to use senna derivative type of laxatives from day one, because it has a better and more controlled effect. Pediatricians in gen­eral prefer to use a different type of laxatives, such as lactulose. The problem with those laxa­tives is that they have a tendency to produce liq­uid stools, which is one of the enemies of patients with anorectal malformations. We do not know the amount of laxatives that every patient needs; we know that every patient needs a different amount, and we determine the amount by trial and error with each patient. As a routine, we order an abdominal x-ray fi lm 1 week after the colostomy closure or after the pull-through; sub­sequently, we order fi lms to be taken after 2 weeks and 1, 3, and 6 months. Even when the parents tell us that the patient is doing very well and they think that he is not constipated, by fol­lowing this routine, we detect many times severe constipation that the parents were unaware of and we can give laxatives to avoid that problem. Once we start with laxatives, we explain to the parents that that must be given on a permanent basis.
We explain to the parents that from the time the colostomy is closed and until the age of bowel control (2 1/2 to 3 years), their work and respon­sibility consists of: (a) Avoiding constipation (b) Trying to achieve regularity in bowel
movements
Patients with anorectal malformations suffer from a colonic (mainly rectosigmoid) hypomotil­ity disorder, and if we leave them alone, they tend to pass a small amount of stool through the day, not really emptying. That is why they need help to empty the rectosigmoid completely. In addition, many of them do not have the excellent regularity of the motility of the rectosigmoid of normal

18.5 Toilet Training

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human beings, who usually empty the rectosig­moid all at once, every day. After 1 year of age, we encourage the parents to give the patients only three meals per day and no snacks in between. By doing that, we try to produce a gastrocolic refl ex only three times a day and not seven times, like many children that have snacks in between. It is much easier to toilet train a child that has two or three bowel movements in a day at a predictable time. The opposite, a patient that suffers from epi­sodes of diarrhea followed by episodes of consti­pation is very diffi cult to toilet train.
We explain to the parents that the big enemies of our patients are two: A. Diarrhea B. Constipation
We try to keep the patients right in between. We want them to empty the rectosigmoid every day and have soft but formed stool. We do all that trial and error, and we educate the parents to achieve our goal.
18.5 Toilet Training
It is very important to determine the future func­tional prognosis, as early as possible in patients with anorectal malformations. When the patients belong to the “poor prognosis” group (poor sacrum, tethered cord, bladder neck fi stula), we do not encourage them to try to toilet train their children. We try not to create false expectations.
The toilet training in our patients with a good or reasonably good functional prognosis usually starts by the age of 2. If we achieve our goal of having our patients free of constipation with the use or without the use of laxatives and also if we achieve our goal of producing one to three bowel movements, at regular times, during the day at predictable times, then it is relatively easy to toi­let train the patients.
In cases with poor functional prognosis, we encourage the family to start our bowel manage­ment program by the age of 3. We arbitrarily selected the age of 3 because many children, at least in the United States, are in diapers even between 2 and 3 years of age. In addition, many parents send the children to school by the age of
3, and we believe that the child must go to school either toilet trained, like all other children, or completely clean in the underwear, because of the bowel management that we provide. We are very much against the idea of sending a child to school knowing that everybody is using normal underwear except our patient. We believe that that is not good from the psychological point of view.
For the patients that have malformations with a functional prognosis that is considered in the middle of the spectrum, in other words, patients that have about 50 or 60 % chance to have vol­untary bowel movements by the age of 3, such as cases with rectoprostatic fi stula, if the patient is not toilet trained by the time that the parents want to send him to school, we offer our bowel management program and explain to the parents that it will not be necessarily for life. Every year, the patient should come to our clinic, and we will try to stop the enemas (bowel manage­ment) and see how much bowel control the patient has, when subjected to a regime that includes giving three meals per day, laxatives, and fi ber with the purpose of making the stool bulky. If the patient does not gain bowel control, we encourage the parents to go back to the bowel management with enemas, for another year, and come back later, because every year we have more chances to toilet train the patient. Every year, the patient will be more cooperative and more interested in being clean with the management plan.
Concerning the urinary control, we can very much predict what kind of urinary function the patient will have, based on the original anatomy and the associated defects of the sacrum and tethered cord. A normal baby, at that age of 1 year old, most likely is not toilet trained for urine; however, it is very easy to observe that the baby passes urine in episodes, about every 3 or 4 h, with a good stream and then remains com­pletely dry in between voiding episodes. That patient has good functional prognosis, and we do not need a urodynamic study to know that he will have urinary control. On the other hand, if we see a 1-year-old baby dribbling urine constantly, obviously the patient will have problems with