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478
Fig. 26.43 Diagram
showing the rectum dissected and pulled down enough to resect 5 cm above the tumor
26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.44 Picture of the operative fi eld, showing the
rectum pulled over
Fig. 26.45 Picture of the operative fi eld. The rectum has
been opened to show the tumor

26.10 Presacral Masses

479
rectum mobilized, sutured with two layers of interrupted long-term absorbable sutures to the lower rectum above the pectinate line. Figure 26.44 shows a full mobilization of the rectum, immediately prior to the resection. Figure 26.45 shows the operative fi eld; the rec- tum has been opened to show the location of the tumor and to be sure that we leave a 5-cm mar­gin before we suture the upper rectum to the lower rectum. Figure 26.46 shows the operation completed. The rectum has been mobilized and sutured to the lower rectum.
Fig. 26.46 Diagram showing the operation completed.
Anastomosis done 2 cm above the pectinate line
26.10 Presacral Masses
Presacral masses in association with anorectal malformation were discussed in the chapter of perineal fi stula.
There are, however, cases of presacral masses with or without sacral defects, without anorectal malformation.
Our series of presacral masses includes over 50 cases, mainly dermoid, teratomas, lipomas, and anterior meningocele, but also includes one schwannoma and two malignant tumors. That is another reason why all the presacral masses must be resected. Patients with presacral masses and no anorectal malformations have mainly cystic teratomas. Figure 26.47 shows the MRI of the pelvis of an adult patient with giant cystic presa­cral teratomas without a sacral defect. The pres­ence of presacral masses without sacral defect has a much better functional prognosis in terms of bowel and urinary function.
There are many other patients that are born with presacral masses without anorectal malfor­mation. These types of cases are more diffi cult to detect. A typical example is a patient that suffers from, what the doctors believed, an infected pilo­nidal sinus. The patient is operated several times, only to fi nd that the abscess and drainage keep recurring. Under such circumstances, it is recommended to order an MRI of the pelvis,
a b
Fig. 26.47 MRI of a patient with giant cystic mature teratomas, without a sacral defect. ( a ) Transverse section.
( b ) Sagittal view
480
26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.48 Infected presacral dermoid, misdiagnosed as
a recurrent pilonidal cyst. The patient underwent seven previous failed operations
had a very large presacral mass that had become chronically infected.
Another one of our patients, a 19-year-old girl, suffered from constipation of unknown ori­gin for her entire life. She developed an abscess in the area of the sacrum, as well as manifesta­tions of meningitis and sepsis. She became extremely ill and was admitted to the hospital. The abscess was drained, and the sepsis and men­ingitis were controlled; the patient developed as sequelae a chronic draining sinus in the area of the coccyx. Figure 26.49 shows the external appearance of this patient. Figure 26.50 shows the same area, immediately after the resection of a very large presacral infected dermoid. The x-ray fi lm showed a hemisacrum. Patients who suffer from idiopathic constipation should have, as part of their evaluation, an x-ray fi lm of the sacrum, as well as an MRI of the spine and cord, to rule out the presence of presacral masses. Table III shows our series of presacral masses by histologic diagnosis, with and without associa­tion with anorectal malformation and with and without association of sacral abnormality.
Fig. 26.49 Chronic draining sinus in the sacral area, the
patient had an infected presacral dermoid
Fig. 26.50 External aspect immediately after the
operation
which may disclose the presence of a teratoma or dermoid that becomes infected and drains in the subcoccygeal area. Figure 26.48 shows a 6-year- old girl that had seven previous operations under the misdiagnosis of a pilonidal sinus; actually she

26.11 Surgical Technique

Most presacral masses are resectable using only the posterior sagittal approach without an abdomi­nal intervention. We are not including in this dis­cussion the cases of neonatal sacrococcygeal teratomas, which represent, we believe, a different entity, with special characteristics and important technical considerations. The posterior sagittal incision is done, as described multiple times in this textbook. It is very important for the surgeon to have a very precise anatomic diagnosis of the mass, with emphasis in the location, in terms of distance between the coccyx and the mass. Using a posterior sagittal approach, we can usually reach up to the level of sacral vertebra 2 or 3; above that, it would represent a serious technical challenge. The incision involves exactly the midline and divides the skin, the subcutaneous tissue, and the entire sphincter mechanism. The MRI study should alert us as to what we are going to fi nd after we divide the entire sphincter mechanism below the coccyx. If the mass is located above the

26.12 Posterior Sagittal Approach, Its Application in Cases with Hirschsprung’s Disease

481
Fig. 26.51 Intraoperative picture of the resection of a
retrorectal mass
coccyx, then we will identify, right away, the pos­terior rectal wall, and dissecting on top of the rectal wall, we will be able to fi nd the mass. On the other hand, sometimes the mass is attached to the poste­rior rectal wall below the coccyx, and therefore, we should look and expect the mass to be found as soon as we divide the sphincter mechanism. Figure
26.51 shows the operative fi eld of one of
these operations, where the mass can be seen in the deepest portion of the incision. We usually place several silk stitches in the mass in order to apply uniform traction to facilitate the dissection. We must be aware of the fact that the mass and the posterior rectal wall are intimately attached; basi­cally we have to shave the posterior rectal wall to separate the mass. In those congenital cases, in which the mass has been compressing the rectum in utero, the resection of the mass is sometimes not enough to deal with the problem, due to a stricture of the rectum. That stricture is fi brotic and should not be expected that it would regain a normal caliber after the mass has been resected. Our
experience shows that it is necessary to resect the narrow portion of the rectum and pull down the healthy- looking bowel. Sometimes there is a very giant dilated upper rectum that must be tapered in order to pull it down. Several patients came to us after they underwent the resection of the mass; they had been subjected to multiple anorectal dila­tations, with the expectation that the narrow por­tion of the rectum would improve which actually does not happen. Therefore, from day one, we sug­gest to resect that narrow portion of the rectum.
In those cases, in which the surgeon is able to make the diagnosis of anterior meningocele component of the mass, the patient should be seen by a pediatric neurosurgeon with experience in these kinds of problems. When the anterior meningocele is very small, sometimes we can simply respect it, knowing that it is there, and leave it in place. However, some neurosurgeons prefer to approach the patient from behind, through a laminectomy to close the communica­tion of the dura with the presacral mass. In the second stage, the surgeon may resect the poste­rior rectal component, of the meningocele, with­out being concerned about injuring the dura. Other neurosurgeons prefer to be present during the posterior sagittal resection of the mass and to close directly the dural defect. Usually, in addi­tion to the closure with nonabsorbable sutures, a patch of fat, muscle, or sometimes cartilage is used to reinforce the defect. We have never seen a case of chronic drainage of cerebrospinal fl uid consecutive to one of these operations.
26.12 Posterior Sagittal Approach,
Its Application in Cases with Hirschsprung’s Disease
In the chapter corresponding to Hirschsprung’s disease, we briefl y mentioned cases of reopera­tions that may represent a very serious surgical technical challenge, due to the fact that the patient suffered from dehiscence, infection, abscesses, and fi stulas that provoked a very signifi cant amount of fi brosis in the pelvis. These patients are particularly diffi cult to treat using a traditional transanal or abdominal approach. When con­fronted with that type of case, the surgeon must
482
ab
26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.52 Diagram showing a sagittal view of the pelvis
of a girl who underwent a failed attempted repair for Hirschsprung’s. There was a dehiscence of the anastomo-
consider the possibility of approaching the patient posterior sagittally. These patients are frequently referred to us with a patent anus, but an atretic rec­tum as a consequence of dehiscence of the pull­through. The area of fi brosis that produced the acquired atresia or stricture of the rectum is located in a place diffi cult to access in the pelvis. Trying to resect the lower rectum, preserving the anal canal, as well as pulling normal bowel down through a “cement type” of pelvis may prove to be extremely diffi cult. Figure
26.52a shows a diagram with a
sagittal view of a female patient who suffered from this kind of problem. There was a dehiscence of the anastomosis, producing a local abscess, severe scarring, and fi brosis. The reoperation for these complications should be done not before 6 months after the event. Figure 26.52b is a diagram of the completed repair. A distal colostogram is per­formed in order to see the precise location of the normal- looking upper bowel, as well as injection of contrast material from below, in order to mea­sure the gap between the proximal and distal bowel. A rectal examination allows the surgeon to realize that there is a lot of scar and therefore very diffi cult to attempt a traditional transanal or abdominoperineal approach. Under those circum-
sis, leaving the patient with an acquired rectal atresia and severe pelvic fi brosis. ( a ) Preoperative diagram. ( b ) Postoperative diagram
stances, the posterior sagittal approach represents a viable alternative. Something similar happens in patients who underwent a Soave type of pull­through, and, for unexplained reasons, the sur­geons left pieces of bowel mucosa attached to the muscular cuff; those islets of mucosa produce mucus, abscess, and fi stula formation and are extremely diffi cult to fi nd in a reoperation, particu­larly when trying to do it with a traditional approach. Again, the posterior sagittal approach gives much better exposure and more chances to fi nd those remnants of mucosa. We have found trapped pieces of bowel left in the pelvis produc­ing mucous and multiple fi stulas, sometimes draining into the perineum, sometimes into the vagina or through the urinary tract.

26.13 Vaginal Atresia with Normal Rectum

Patients suffering from vaginal atresia have a dilated, blind vagina located at different levels sometimes compressing the trigone of the blad­der. Depending on the specifi c distance between the blind lower part of the vagina and the
26.13 Vaginal Atresia with Normal Rectum
483
perineum, the surgeon must select the best pos­sible approach to mobilize the vagina down. If the vagina is considered not reachable through the perineum, one alternative that we have used is exploring the space between the urethra and rec­tum, trying to reach as high as possible, provided we feel safe and confi dent in not producing injury of the rectum or urethra. Once we have reached from below the point in which we feel it is not safe to continue, then the vagina can be mobi­lized through the abdomen with a laparotomy or laparoscopy. In the event of fi nding the blind vagina during the exploration between the ure­thra and rectum, but the mobilization becomes technically diffi cult, in a patient that has been subjected to a strict bowel preparation prior to the procedure, we can expand the incision trans­anorectally, as demonstrated in cases of urogeni­tal sinus with normal rectum. Provided the reconstruction of the rectum is performed metic­ulously, as we previously mentioned, the patients recover very well, even without the opening of a
colostomy. Figure 26.53a–f shows images related with one specifi c patient who came to our clinic with a very dilated blind vagina (hematocolpos and hematometra). With the bowel completely clean in order to have access to trans-anorectal approach, if necessary, we approached the patient through the perineum, and we were fortunate enough to fi nd the vagina and were able to mobi­lize the vagina down enough to reach the labia, without having to divide the rectum. However, we can easily divide the rectum, if necessary, if the procedure becomes technically demanding. Figure 26.53a shows the preoperative aspect of the introitus of this patient that only shows the urethral opening. There is no vaginal opening. Figure 26.53b shows the MRI of this patient. Figure 26.53c, d shows the appearance of the fi eld during the surgical exploration, looking for the vagina. Figure 26.53e shows that the vagina has been mobilized, and Fig. 26.53f shows that the vaginal opening has been successfully created.
Fig. 26.53 Vaginal atresia, hematocolpos, and hemato-
metra. ( a ) Introitus showing only the urethral opening. ( b ) MRI study showing the giant vagina. ( c ) Surgical explora- tion between the urethra and the rectum. Silk sutures
ab
applied on the vagina. ( d ) Vagina open. Observe old blood coming out. ( e ) Vaginal fully mobilized. ( f ) Finished operation
484
26 Posterior Sagittal Approach for the Treatment of Other Conditions
cd
ef
Fig. 26.53 (continued)

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Miscellaneous Conditions

2 7

27.1 Part I: Perianal Abscess and Fistula

27.1.1 Defi nition
Perianal fi stula is a fairly common condition in male infants. It consists in the presence of a tract that runs from one of the anorectal crypts toward the perianal skin (Figs. 27.1 and 27.2 ). This fi s- tula occurs as a consequence of an abscess previ­ously formed in the perianal area. We do not know precisely the incidence of this condition. It is extremely unusual to see this condition in female patients, as well as in older children. In pediatrics, this condition has very signifi cant differences when compared with perianal and
perirectal fi stulas in adults [ 13 ]. It is important to recognize these differences because of their therapeutic implications.
Perianal and perirectal abscesses and fi stula are fairly common in the adult population, but it occurs in forms and variants more severe and serious than in pediatrics [ 13 ]. The recom- mended treatments in adults also vary, depending on the type of fi stula (transsphincteric, transleva­tor, intrasphincteric). In pediatrics, we only see a benign form in babies (mainly males), and that is the type that we will discuss here.
Fig. 27.2 Perianal fi stula. Arrow shows the point of the
Fig. 27.1 Perianal abscess
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, DOI 10.1007/978-3-319-14989-9_27, © Springer International Publishing Switzerland 2015
future fi stula
487