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19 Management in the Newborn Period

Marc A. Levitt and Alberto Peña

Contents 19.1 Males . . . 289 19.2 Females . . . 290 19.3 . . . 292 References . . . 292

19.1 Males

Figure 19.1 shows the decision-making algorithm for the initial management of male patients with anorec- Fig. 19.1 Decision-making algorithm for male newborns with tal malformations. When one is called to see a new- anorectal malformations (ARM). U/S Ultrasound, PSARP pos- born male with an anorectal malformation, a thor- terior sagittal anorectoplasty, R/O rule out (Reprinted from ough perineal inspection must be performed. This Pediatric Surgery, 4th ed., Ashcraft, Whitfield & Murphy eds. usually gives the most important clues about the type Peña A, Levitt MA. and Cloacal Malforma- of malformation that the patient has. It is important tions, p 501, Elsevier Saunders, Philadelphia (2005), with per- not to make a decision about colostomy or primary mission from Elsevier.) operation before 24 h of life because significant intra- luminal pressure is required for the meconium to be forced through a orifice, which is the most reli- to determine the presence of associated spinal and able sign of the location of the fistula. If meconium is sacral anomalies. A spinal ultrasound in the newborn seen on the perineum, it is evidence of a perineal fis- period is a good screen for tethered cord and other tula. If there is meconium in the urine, a rectourinary spinal anomalies. An ultrasound of the abdomen will fistula is present. rule out the presence of hydronephrosis [1]. If the Radiologic evaluations do not show the real anat- baby has signs of a perineal fistula, an anoplasty can omy before 24 h because the is collapsed and be performed during the first 48 h of life without a does not yet have enough intraluminal pressure to protective colostomy (Fig. 19.2). If the baby’s clinical overcome the muscle tone of the sphincters that sur- condition warrants waiting to do surgery, such as for round it. Therefore, radiologic evaluations done too a premature baby or one with an associated cardiac early (before 24 h) will most likely reveal the false im- defect, dilatations of the fistula with a delayed repair pression of a “very high rectum.” is acceptable. Such a baby must be watched closely to During the first 24 h, the baby should receive intra- be sure that the colon is adequately emptying through venous fluids and antibiotics. The presence of associ- the fistula. After 24 h, if no meconium is seen on the ated defects should be investigated. These include car- perineum, a cross-table, lateral x-ray film with the diac conditions, , , baby in a prone position often shows the location of and urologic and spinal defects. An echocardiogram the distal rectum. If the gas in the rectum is located of the heart can be taken. A nasogastric tube is passed below the coccyx and the baby is in a good condition to detect the presence of esophageal atresia. An x-ray with no significant associated defects, depending on film of the lumbar spine and the sacrum are helpful the surgeon’s experience, a posterior sagittal opera- 290 Marc A. Levitt and Alberto Peña

Fig. 19.2 Newborn anoplasty Fig. 19.3 Radiograph of cross-table lateral x-ray

tion without a protective colostomy can be considered 19.2 Females (Fig. 19.3). If the rectal gas is seen located above the coccyx and the patient has meconium in the urine, Figure 19.4 shows a decision-making algorithm for or has significant associated defects, an abnormal sa- the initial management of female patients. As in crum, or a flat bottom, a colostomy is the safest ap- males, the perineal inspection is the most important proach, with postponement of the main repair for a step in the diagnosis and decision-making. The first subsequent operation [2]. The definitive operation 24 h should also be used to rule out important associ- can be performed as early as 4–8 weeks later provided ated defects, as described previously. the baby is gaining weight normally. Performing the The perineal inspection may disclose a single definitive repair early has important advantages for perineal orifice, which establishes the diagnosis of a the patient, including less time with an abdominal cloaca. The clinician should know that such patients stoma, less size discrepancy between the proximal have a high likelihood (90%) of having an associated and distal bowel at the time of colostomy closure, eas- urologic defect. The patient needs a urologic evalua- ier anal dilatation, and avoidance of psychological se- tion and the presence of hydrocolpos should be ruled quelae from painful perineal maneuvers. In addition, out by ultrasound. placing the rectum in the right location early in life, Babies with a cloaca should undergo a diverting and using it, may represent an advantage in terms of colostomy. It is important to perform the colostomy the potential for acquired local sensation [3]. proximally enough to allow for the future repair of A temptation to repair these defects without a pro- the malformation without interference from the co- tective colostomy always exists [4, 5]. Such a repair lostomy. The surgeon must leave enough redundant without colostomy is performed without adequate distal rectosigmoid to allow a pull-through and, if anatomic information about the specific type of de- needed, a vaginal reconstruction. fect. Catastrophic complications have been seen in During the opening of the colostomy, it is manda- patients in whom the surgeon did not have a distal tory to drain a hydrocolpos if present [7]. If the hy- colostogram, approached the patients posterior sagit- drocolpos is not large enough to reach the abdominal tally looking for the rectum, and during the search for wall above the bladder, it can be drained with a rubber the rectum, injured to important structures (urethra, tube. Because a significant number of these patients bladder, ureters, vas deferens, or seminal vesicles) oc- have two hemivaginas, the surgeon must be certain curred [6]. that the tube inserted into the hydrocolpos is drain- 19 Management in the Newborn Period 291

Fig. 19.4 Decision-making al- gorithm for female newborns with ARM. Urol. Urological (Re- printed from Pediatric Surgery, 4th ed., Ashcraft, Whitfield & Murphy eds. Peña A, Levitt MA. Imperforate Anus and Cloacal Malformations, p 502, Elsevier Saunders, Philadelphia (2005), with permission from Elsevier.)

ing both hemivaginas. Occasionally, the surgeon has resents the most common source of complications in to open a window in the vaginal septum in order to these patients. To decide to repair this malformation drain both with a single tube. The hydrocolpos can be primarily or to open a colostomy is a personal deci- so large that it may produce respiratory distress; vagi- sion that should be based on the experience of the nas that large may be drained by suturing the vaginal surgeon. Colostomy is still the most effective way to wall to the abdominal wall as a stoma. protect the pull-through. Drainage of the hydrocolpos will relieve the ure- Occasionally (less than 10% of the cases) there is teral obstruction, as the dilated compresses no visible fistula and there is no meconium coming the trigone. In rare cases, the bladder remains dis- out from either the perineum or the urinary tract, tended, and this may be evidence of particularly long, even after 24 h of observation. For this small group of or narrow common channel. In such circumstances, patients, a cross-table, lateral film is valuable. If the x- the baby may require a vesicostomy or a suprapubic ray shows the gas in the rectum located very near the cystotomy. During the same anesthetic, it is helpful to skin, it is likely that the patient has a perineal fistula. perform an endoscopy to try to determine the anat- If the patient has a blind rectum located about 1 or omy, particularly the length of the common channel, 2 cm above the skin, the patient probably suffers from which will help planning of the definitive operation. an imperforate anus with no fistula. One can con- The perineal inspection may show the presence of sider, in this case, performing a primary operation a perineal fistula, for which a primary anoplasty with- without a colostomy, depending on the surgeon’s ex- out a colostomy may be performed. Occasionally, the perience (many of these patients with no fistula also surgeon may have to care for a baby with severe asso- have Down syndrome) [8]. ciated defects or one who is very premature. If that is Patients with a rectovestibular fistula who are very the case, dilatation of the fistula facilitates emptying of sick or premature can have dilatations of the fistula to the colon, and a definitive operation can be planned allow decompression of the colon, with a repair to be for a future time. performed later in life. That repair can be done with The presence of a rectovestibular fistula is the most a protective colostomy or in a primary fashion, de- common finding in female patients. This malforma- pending again on the surgeon’s experience. tion can be repaired during the neonatal period with- When patients with rectovestibular are out a protective colostomy. A newborn pull-through repaired primarily in the newborn period, we rec- in such patients is ideal, but unfortunately also rep- ommend keeping them hospitalized for 5 days with 292 Marc A. Levitt and Alberto Peña: Management in the Newborn Period

nothing by mouth, and on parenteral nutrition. On and development of a microcolon with megarectosig- the other hand, when the patients are subjected to a moid may result when a higher colostomy is utilized. primary repair of a perineal fistula or rectovestibular Mechanical cleansing of the distal colon prior to the fistula later in life, strict preoperative bowel irrigation definitive repair is much less difficult when the colos- is vital in order to be sure that the intestine is com- tomy is located in the descending colon. In the case of pletely clean. We insert a central venous line and keep a large rectourethral fistula the patient may pass urine the patient hospitalized for 7–10 days with nothing by into the colon, whereas a more distal colostomy al- mouth, receiving parenteral nutrition. With this regi- lows urine to escape through the distal stoma without men, there have been no cases of perineal infection. significant absorption. If urine remains in the colon and is absorbed, metabolic acidosis may develop. Loop permit the passage of stool 19.3 Colostomy from the proximal stoma into the distal bowel, which produces urinary tract infection, distal rectal pouch A divided descending colostomy is ideal for the man- dilatation, and fecal impaction. Prolonged distention agement of anorectal malformations (Fig. 19.5). The of the rectal pouch may produce irreversible bowel completely diverting colostomy provides bowel de- damage, leading to a significant bowel hypomotility compression as well as protection for the final repair disorder and severe later in life. Loop co- of the malformation. In addition, this type of colos- lostomies are also prone to prolapse [11]. tomy facilitates the distal colostogram, which repre- A colostomy created too distal in the area of rec- sents the most accurate diagnostic study for deter- tosigmoid may interfere with the mobilization of the mining the anatomy of these defects [9]. rectum during the pull-through and is a common er- A descending colostomy has advantages over a right ror. or transverse colostomy [10, 11]. There is a relatively short segment of defunctionalized distal colon. Atro- phy of the bowel distal to a more proximal colostomy References

1. Peña A, Hong AR (1999) Anorectal malformations – the state of the art. Colon Rectal Surg 2:1–19 2. Shaul DB, Harrison EA (1997) Classification of anorectal malformations – initial approach, diagnostic tests and co- lostomy. Semin Pediatr Surg 6:187–195 3. Freeman NV, Burge DM, Soar JS, et al (1980) Anal evoked potentials. Z Rinderchir 31:22–30 4. Goon HK (1990) Repair of anorectal anomalies in the neonatal period. Pediatr Surg Int 5:246–249 5. Moore TC (1990) Advantages of performing the sagittal anoplasty operation for imperforate anus at birth. J Pedi- atr Surg 25:276–277 6. Hong AR, Rosen N, Acuña MF, Peña A, Chaves L, Rodri- guez G (2002) Urological injuries associated with the re- pair of anorectal malformations in male patients. J Pediatr Surg 37:339–344 7. Levitt MA, Peña A (2005) Pitfalls in the management of newborn cloacas. Pediatr Surg Int 21:264–269 8. Torres P, Levitt MA, Tovilla JM, Rodriguez G, Peña A (1998) Anorectal malformations and Down’s syndrome. J Pediatr Surg 33:1–5 9. Gross GW, Wolfson PJ, Peña A (1991) Augmented-pres- sure colostogram in imperforate anus with fistula. Pediatr Fig. 19.5 Ideal colostomy (Reprinted from Atlas of Surgical Radiol 21:560–563 Management of Anorectal Malformations, Peña A. Colostomy, 10. Wilkins S, Peña A (1988) The role of colostomy in the p 19, Springer-Verlag, Inc. (1989), with permission of Springer management of anorectal malformations. Pediatr Surg Int Science and Business Media.) 3:105–109 19 Management in the Newborn Period 293

11. Peña A, Krieger M, Levitt MA (2005) Colostomy in ano- 13. Peña A (1989) Colostomy. In: Peña A (ed) Atlas of surgical rectal malformations: a procedure with serious but pre- management of anorectal malformations, Springer-Ver- ventable complications. J Pediatr Surg 41:748–756 lag, New York, pp 26–44 12. Peña A, Levitt MA (2005) Imperforate anus and cloacal malformations. In: Ashcraft KW, Holcomb W, Murphy JP (eds) Pediatric Surgery 4th edn. Elsevier Saunders, Phila- delphia, pp 501–502