children

Article
Percutaneous Anorectoplasty (PARP)—An Adaptable,
Minimal-Invasive Technique for Anorectal
Malformation Repair
Julia Küppers 1 , Viviane van Eckert 1 , Nadine R. Muensterer 1 , Anne-Sophie Holler 1 , Stephan Rohleder 2 ,
Takafumi Kawano 3 , Jan Gödeke 1 and Oliver J. Muensterer 1, *

1 Department of Pediatric Surgery, Dr. von Hauner Children’s Hospital, Ludwig-Maximilians-University

Medical Center, 80337 Munich, Germany; [email protected] (J.K.);
[email protected] (V.v.E.); [email protected] (N.R.M.);
[email protected] (A.-S.H.); [email protected] (J.G.)
2 Department of Pediatric Surgery, Johannes-Gutenberg-University Medical Center Mainz,
55131 Mainz, Germany; [email protected]
3 Department of Pediatric Surgery, Kagoshima University, Kagoshima 890-8520, Japan;
[email protected]
* Correspondence: [email protected]; Tel.: +49-894-4005-3101

Abstract: Background: Anorectal malformations comprise a broad spectrum of disease. We de-

veloped a percutaneous anorectoplasty (PARP) technique as a minimal-invasive option for repair
of amenable types of lesions. Methods: Patients who underwent PARP at five institutions from
2008 through 2021 were retrospectively analyzed. Demographic information, details of the oper-
ative procedure, and perioperative complications and outcomes were collected. Results: A total
Citation: Küppers, J.; van Eckert, V.;
of 10 patients underwent the PARP procedure during the study interval. Patients either had low
Muensterer, N.R.; Holler, A.-S.;
Rohleder, S.; Kawano, T.; Gödeke, J.;
perineal malformations or no appreciable fistula. Most procedures were guided by ultrasound,
Muensterer, O.J. Percutaneous fluoroscopy, or endoscopy. Median age at PARP was 3 days (range 1 to 311) days; eight patients were
Anorectoplasty (PARP)—An male. Only one intraoperative complication occurred, prompting conversion to posterior sagittal
Adaptable, Minimal-Invasive anorectoplasty. Functional outcomes in most children were highly satisfactory in terms of continence
Technique for Anorectal and functionality. Conclusions: The PARP technique is an excellent minimal-invasive alternative for
Malformation Repair. Children 2022, boys born with perineal fistulae, as well as patients of both sexes without fistulae. The optimal type
9, 587. https://doi.org/10.3390/ of guidance (ultrasound, fluoroscopy, or endoscopy) depends on the anatomy of the lesion and the
children9050587 presence of a colostomy at the time of repair.
Academic Editors: Robert Bergholz
and Thomas Franz Krebs Keywords: anorectal malformation; percutaneous; anorectoplasty; ultrasound; fluoroscopy; endoscopy;
keyword; perineal fistula; Down syndrome
Received: 7 March 2022
Accepted: 19 April 2022
Published: 21 April 2022

Publisher’s Note: MDPI stays neutral 1. Introduction

with regard to jurisdictional claims in
Anorectal malformation affects around 1:5000 liveborn infants and comprises a wide
published maps and institutional affil-
spectrum of conditions concerning the distal anus, rectum, as well as urogenital tract. Half
iations.
of all anorectal malformations are considered low anorectal malformations [1–3].
In 1982, Peña and de Vries introduced the posterior sagittal anorectoplasty (PSARP),
which has become the standard of open repair [1,4]. The procedure, however, is associ-
Copyright: © 2022 by the authors.
ated with a relatively high risk of dehiscence, wound infection, stricture, and long-term
Licensee MDPI, Basel, Switzerland. continence issues [3,5,6]. Some forms of anorectal malformations may be amenable to a
This article is an open access article less invasive repair in which the neorectum is reconstructed through the intact sphincter.
distributed under the terms and For high lesions, including rectoprostatic and rectovestibular fistulas, the laparoscopy-
conditions of the Creative Commons assisted anorectoplasty (LAARP) procedure, first introduced by Georgeson et al. in 2000,
Attribution (CC BY) license (https:// has become routine in many centers around the world [7]. Lower lesions, however, such
creativecommons.org/licenses/by/ as rectoperineal fistulas in boys, and imperforate anus without a fistula, do not require or
4.0/). benefit from laparoscopy [8].

Children 2022, 9, 587. https://doi.org/10.3390/children9050587 https://www.mdpi.com/journal/children

Children 2022, 9, 587 2 of 11

We therefore devised a minimal-invasive percutaneous procedure that avoids opening

of the pelvic floor or splitting the sphincter, the so-called PARP. Over the course of more
than a decade, this procedure has undergone several important modifications. The aim of
this study is to retrospectively describe and evaluate all patients who underwent a PARP
procedure in terms of feasibility, effectiveness, complications, and outcome.

2. Materials and Methods

2.1. Study Design
This is a retrospective analysis of all patients who underwent the PARP procedure at
5 different institutions from 2008 through 2021. In order to be eligible for inclusion, the
patients had to be either male with a typical perineal fistula or of either sex without a fistula.
All other forms of anorectal malformation were excluded.

2.2. Ethics
The study was approved by the ethics board of the Ludwig Maximilian University
Faculty of Medicine (registration number 22-0141). The parents or caregivers gave their
explicit and written consent on having their child operated on using this novel method.
Alternatives were described and offered. The potential risks and benefits were discussed
in detail.

2.3. Operative Technique

2.3.1. PARP without Image Guidance (nPARP)
The percutaneous anorectoplasty (PARP, Figure 1, Video S1: Description of the PARP
procedure without image guidance in a newborn male with anorectal malformation and a
rectoperineal fistula) is applicable in patients born with pure imperforated anus covered by
a skin tag in the form of a bucket handle. A Wangensteen–Rice invertogram radiograph
confirms the presence of a low imperforated anus. The bucket handle is dissected off
the underlying tissue in a modified lithotomy position. Subsequently, the bucket handle
is divided and resected. The posterior skin is excised in a wedge-type fashion. Then,
the muscle complex is located at the center of the neo-anus using an electronic muscle
stimulator. Once the sphincter complex has been detected, a needle or an eight French
dilator is passed through the sphincter complex into the rectal pouch. A guidewire is
passed through the rectal pouch to secure the tract. Once secured, the tract is dilated
sequentially from eight to twenty French. At that point, the guidewire and dilator are
removed. Following the evacuation of meconium, Hegar dilators are used to dilate the tract
further to approximately eleven millimeters
  or an age-appropriate size. Finally, retractors
    are inserted. The rectal mucosa is retracted down to the level of the skin and is then sewn to
the skin
  using interrupted polyglactin sutures resulting in a cosmetically pleasing, inverted,
 and orthotopic anus with an intact sphincter complex.

 
Figure 1. Screenshot of Video S1. Typical perineal fistula with bucket‐handle in a boy 
Figure 1. Screenshot of Video S1. Typical perineal fistula with bucket-handle in a boy.
 
Children 2022, 9, 587 3 of 11

2.3.2. Ultrasound-Guided PARP (uPARP)

When performing an ultrasound-guided percutaneous anorectoplasty (uPARP), the
Children 2022,
2022, 9,
9, xx FOR
FOR PEER
previously
PEER REVIEW
REVIEW
described PARP operative technique is complemented by real-time ultrasound
Children 33 of
of 10
10
imaging of the perineum in the operating room to localize the rectum and muscle complex
(Figure 2).

Figure 1.
Figure 1. Ultrasound
Ultrasound image
image during
during uPARP
uPARP showing
showing the
the guidance
guidance of
of the
the needle
needle (arrow)
(arrow) towards
towards the
the
Figure 2. Ultrasound image during uPARP showing the guidance of the needle (arrow) towards the
meconium-filled rectal
meconium-filled rectal pouch.
pouch.
meconium-filled rectal pouch.

2.3.3. Fluoroscopy-Guided
2.3.3.
2.3.3. Fluoroscopy-Guided (Interventional)
Fluoroscopy-Guided (Interventional)PARP
(Interventional) PARP(iPARP)
PARP (iPARP)
(iPARP)
During aaa fluoroscopy-guided
During
During fluoroscopy-guided (interventional)
fluoroscopy-guided (interventional) percutaneous anorectoplasty (iPARP),
(interventional) (iPARP),
the patient
the
the patient is
patient is placed
is placed in
placed in aaa prone
in proneposition
prone position with
position with the
with thebuttocks
the buttocks elevated,
buttocks elevated, much
elevated, much like
much like during
like during aaa
during
conventional
conventional posterior
posterior sagittal
sagittal anorectoplasty
anorectoplasty (PSARP).
(PSARP). The The
conventional posterior sagittal anorectoplasty (PSARP). The fluoroscopy unit is fluoroscopy
fluoroscopy unit unit
is is posi-
posi-
positioned
tioned
in a in a cross-table
cross-table lateral lateral configuration.
configuration. The The
center center
of the of the
muscle muscle
complex complex
tioned in a cross-table lateral configuration. The center of the muscle complex is identified is is identified
identified using
using
an an electronic
electronic
using an electronic
stimulator.stimulator.
A needle
stimulator. A is
A needle is advanced
advanced
needle is advanced through
throughthrough
the center theofcenter
the center of the
the sphincter
the sphincter
of sphincter
into the
into the
the air-filled
air-filled
into air-filled
rectal pouch rectal
rectal pouch
under
pouch under fluoroscopic
fluoroscopic
fluoroscopic
under guidance
guidanceguidance and the
and the guidewire
and the guidewire
guidewire
is advanced is advanced
is advanced
through
through
the
through the
needlethe needle
(Figure
needle (Figure
3a,b). 2a,b).
A 122a,b).
(Figure A 12
mm balloon
A 12 mm
mm balloon
dilator dilator is
is advanced
balloon dilator is advanced
over the needle
advanced over
overandthe
thetheneedle
tract
needle
is dilated
and the (Figure
tract is 3c,d).
dilated Thereafter,
(Figure the
2c,d). mucosa
Thereafter, is retracted
the mucosa down
is to the
retracted
and the tract is dilated (Figure 2c,d). Thereafter, the mucosa is retracted down to the skin skin
down using
to hooks
the skin
and
usingsutured
hooks circumferentially
and sutured as described
circumferentially for
as the PARP
described
using hooks and sutured circumferentially as described for the PARP above. above.
for the PARP above.

Figure 3.
Figure
Figure 2. Technique
2. Technique of
Technique of iPARP.
of iPARP.The
iPARP. Theneedle
The needleand
needle andguidewire
and guidewireare
guidewire areintroduced
are introducedinto
introduced intothe
into therectum
the rectum through
rectum through
through
the
the sphincter
sphincter complex
complex (a)
(a) under
under fluoroscopic
fluoroscopic guidance
guidance (b).
(b). A
A balloon
balloon dilator
dilator is
is advanced
advanced
the sphincter complex (a) under fluoroscopic guidance (b). A balloon dilator is advanced over the over
over the
the
guidewire (c)
guidewire (c) to
to dilate
dilate the
the tract
tract (d).
(d).
guidewire (c) to dilate the tract (d).

2.3.4. Endoscopically-Guided
2.3.4. Endoscopically-Guided PARP
PARP (ePARP)
(ePARP)
An endoscopically-guided
An endoscopically-guided percutaneous
percutaneous anorectoplasty
anorectoplasty (ePARP,
(ePARP, Video
Video S2:
S2: Descrip-
Descrip-
tion of
tion of the
the ePARP
ePARP procedure
procedure inin aa 66 month
month old
old girl
girl with
with Down
Down syndrome
syndrome who
who had
had aa trans-
trans-
verse colostomy in an outside hospital) requires a previous colostomy and is thus
verse colostomy in an outside hospital) requires a previous colostomy and is thus per- per-
Children 2022, 9, 587 4 of 11

 
2.3.4. Endoscopically-Guided PARP (ePARP)
    An endoscopically-guided percutaneous anorectoplasty (ePARP, Figure 4, Video S2:
 
Description of the ePARP procedure in a 6 month old girl with Down syndrome who had
 a transverse colostomy in an outside hospital) requires a previous colostomy and is thus
performed in children without a fistula, usually patients with Down syndrome. The patient
is placed supine in a way that allows a distal colonoscopy from the mucous fistula. A
fluoroscopy unit is placed to allow cross-table lateral imaging. At the blind end of the
colon, a typical star-shaped scar is always detected and marks the center of the future tract
(Figure 5). The center of the muscle complex is identified from the outside with a stimulator
and a needle is advanced through the sphincter complex into the rectum under X-ray and
endoscopic guidance. A guidewire is placed. Then, a twelve-millimeter balloon dilator is
inserted over the guidewire and inflated to dilate the tract. After the balloon is deflated, the
tissue tract can be inspected endoscopically to the outside. Subsequently, the endoscope is
retracted back inside. The next step involves bringing the rectal mucosa down to the skin.
This is accomplished by introducing two sharp hooks, one anteriorly and one posteriorly,
which gently retract the mucosa. From the outside, circular stay sutures are placed on
the mucosal sleeve. The exact placement of the sutures can be verified endoscopically.
Children 2022, 9, x FOR PEER REVIEW 4 of 10
Thereafter, a colocutaneous anastomosis is performed using circular braided absorbable
sutures. Correct placement of the sutures can be verified endoscopically to confirm that the
mucosa circularly anastomoses with the skin. This is important   to prevent stricture. Finally,
is placed supineareincut
a way thata allows a distal colonoscopy from
endthe mucous
Figure 1. Screenshot of Video S1. Typical perineal fistula with bucket‐handle in a boy 
the stay sutures leaving watertight anastomosis. At the of the fistula.the
procedure, A
fluoroscopy
 
neo-anus unit is placed
is calibrated using to allow cross-table
a ten-millimeter lateral
Hegar imaging. At the blind end of the
dilator.
colon, a typical star-shaped scar is always detected and marks the center of the future tract
(Figure 3). The center of the muscle complex is identified from the outside with a stimu-
lator and a needle is advanced through the sphincter complex into the rectum under X-
ray and endoscopic guidance. A guidewire is placed. Then, a twelve-millimeter balloon
dilator is inserted over the guidewire and inflated to dilate the tract. After the balloon is
deflated, the tissue tract can be inspected endoscopically to the outside. Subsequently, the
endoscope is retracted back inside. The next step involves bringing the rectal mucosa
down to the skin. This is accomplished by introducing two sharp hooks, one anteriorly
and one posteriorly, which gently retract the mucosa. From the outside, circular stay su-
tures are placed on the mucosal sleeve. The exact placement of the sutures can be verified
endoscopically. Thereafter, a colocutaneous anastomosis is performed using circular
braided absorbable sutures. Correct placement of the sutures can be verified endoscopi-
cally to confirm that the mucosa circularly anastomoses with the skin. This is important
 
to prevent stricture. Finally, the stay sutures are cut leaving a watertight anastomosis. At
Figure
the end Screenshot
4. of of Video S2.
the procedure, theTrans-neoanal
neo-anus is endoscopic
calibratedview
usingof a
the anastomosis being
ten-millimeter con-structed.
Hegar dilator.
Figure 4. Screenshot of Video S2. Trans‐neoanal endoscopic view of the anastomosis be‐
ing constructed 

Figure 5.
Figure 3. Star-shaped
Star-shapedend
endofofthe rectal
the pouch
rectal marking
pouch the the
marking future tracttract
future towards the sphincter
towards com-
the sphincter
plex (*).
complex (*).

2.4. Data Acquisition

The data were retrospectively collected from operative reports and hospital records.
Pertinent demographic information, comorbidities, operative time, type of PARP, periop-
erative and postoperative complications, as well as short- and long-term outcome were
extracted into a database.
 
Children 2022, 9, 587 5 of 11

2.4. Data Acquisition

The data were retrospectively collected from operative reports and hospital records.
Pertinent demographic information, comorbidities, operative time, type of PARP, periop-
erative and postoperative complications, as well as short- and long-term outcome were
extracted into a database.

3. Results
3.1. Patients
During the study interval, a total of 10 patients were included. Eight of those patients
were male. Half of the patients presented with anorectal malformation with a perineal
fistula; the other half did not exhibit an appreciable fistula. Only three patients did not show
comorbidities. Three patients were diagnosed with Down syndrome, one patient suffered
from VACTERL, and one patient presented with Currarino triad, Spina bifida, as well as
congenital heart disease. Furthermore, two patients were born prematurely, one of whom
experienced a pneumothorax and underwent chest tube placement preoperatively. This
patient was later diagnosed with Duchenne muscular distrophy. Moreover, four patients
had received a colostomy prior to the PARP procedure. The median age for colostomy
placement was 1.5 days (range 0 to 2) (Table 1).

Table 1. Patient demographics and data (VACTERL—vertebral, anorectal, cardiac, tracheo-

esophageal, renal, limb anomalies).

Type of Anorectal Age (Days) at

Patient Sex Year Comorbidities Colostomy
Malformation Colostomy
1 Male 2008 Perineal fistula, bucket handle None No -
Perineal fistula
2 Male 2010 None No -
(pinpoint at raphe)
3 Female 2011 No fistula Down syndrome No -
32-week prematurity, left
4 Male 2011 Perineal fistula pneumo-thorax and chest No -
tube placement
36-week prematurity,
5 Male 2015 Perineal (scrotal) fistula diagnosed later No -
with Duchenne
Currarino triad, Spina bifida,
6 Male 2015 Perineal fistula No -
congenital heart disease
7 Female 2015 No fistula Down syndrom Yes 3
8 Male 2017 No fistula None Yes 3
9 Male 2020 No fistula Down Syndrome Yes 1
10 Male 2021 No fistula VACTERL association Yes 2

3.2. Operations
Overall, ten percutaneous anorectoplasties were performed between 2008 and 2021.
The median age at the PARP was three days (range 1 to 311 days). The median operative
time amounts to approximately 60 min (range 25 to 183). The OP times of the final two
PARP procedures were not included in this calculation as patients underwent multiple
concomitant surgical procedures. Apart from the initial two percutaneous anorectoplasties
without image guidance, the procedures were generally guided: one uPARP, three iPARPs,
and four ePARPs were performed (Table 2).
Children 2022, 9, 587 6 of 11

Table 2. Operative data and complications. (* Total operative time includes ePARP and other
procedures, namely, cystoscopy, esophagoscopy, Kimura-lengthening of upper esophageal pouch.
§ Operative time includes cystoscopy).

Intraoperative and Perioperative

Patient Age at PARP (Days) Operative Time (Minutes) Type of PARP
Omplications
1 2 25 No image guidance None
Injured urethra or second fistula,
2 2 183 No image guidance converted to psarp, urethra
repaired, colostomy performed
3 3 68 uPARP None
4 1 57 iPARP None
5 1 109 iPARP None
6 3 44 iPARP None
7 225 51 ePARP None
8 77 62 ePARP None
9 168 236 * ePARP None
10 311 111 § ePARP None

3.3. Complications
There was one complication in the second child who was operated on without image
guidance. The procedure was initiated in the supine position with the legs raised and hips
flexed. Preoperatively, a Foley bladder catheter was placed, but it could not be advanced all
the way and no urine was obtained. It was left in place without inflating the balloon. After
punctuation of the rectum and dilation, the Foley catheter was visible through the rectum,
prompting us to abort the procedure. The patient was turned prone, prepped, and draped.
Then, a posterior sagittal anorectoplasty (PSARP) was performed. Subsequently, the Foley
catheter was removed, a new catheter was placed through the urethra under vision into
the bladder, and the bulbar urethral opening, where the first catheter had passed into the
rectum, was repaired using interrupted resorbable sutures. No other peri- or postoperative
complications were noted in this series (Table 2). There were no wound infections.

3.4. Outcomes
The median follow-up lasted approximately 16 months (range 0 to 43. Table 3). Two
out of ten patients dealt with constipation postoperatively, one of which required oral
macrogol (polyethylene-glycol) treatment. None of the patients suffered from incontinence
following the PARP procedure. Four patients required further dilations. Overall, outcomes
were highly satisfactory in most patients in terms of functionality and continence.

Table 3. Postoperative data and outcomes (y—years, m—months).

Age at Last
Patient Constipation Incontinence Dilations Additional Comments
Follow-Up
Potty trained at 2 years,
1 2y3m No No No
functionally normal
Short-term well, long-term lost
2 2m - - Yes
to follow-up
Needed macrogol, otherwise no
3 1y3m Yes No Yes
problems in the follow-up time period
4 1y8m No No No Started potty training
No problems, normal stooling pattern,
5 3y9m No No No general hypotonia due to duchenne
muscular dystrophy in toddlerhood
Died of congenital heart disease
6 6m No No Yes
at 6 months
Children 2022, 9, 587 7 of 11

Table 3. Cont.

Age at Last
Patient Constipation Incontinence Dilations Additional Comments
Follow-Up
Colostomy takedown at 9 months of
7 2 y 10 m No No No age, normal spontaneous defecation
pattern 1× per day
Colostomy performed at the umbilicus,
8 9m No No No
no issues with stooling, no medications
9 1 y 11 m No No No Started potty training
10 8m Yes No Yes Too early to evaluate continence

4. Discussion
This is the largest case series on percutaneous anorectoplasty to date. Over the
course of the last decade, the technique has undergone evolution using additional image
guidance, to the point where it can be safely performed and recommended for certain
anorectal malformations.
Despite the heterogeneous pattern of anorectal malformations (ARMs) [1], posterior
sagittal anorectoplasty (PSARP) has been the main approach for repair across the board.
The drawback of PSARP is the division of the sphincter in two halves through the midline,
with later reconstruction [1,9]. Despite the argument that this allows accurate visualization
of anatomical structures, thus allowing the most accurate surgical correction and preser-
vation of blood vessels and nerve structures, current studies in the literature increasingly
consider that the invasiveness of this method may not be necessary in certain cases [2,10,11].
Laparoscopy can assist with repair of high forms of ARMs while leaving the sphincter
intact [7,10], although the intuitive hypothesis that this could improve the functional
prognosis in terms of decreasing cases of fecal incontinence and constipation has not yet
been conclusively confirmed [9,10,12,13]. Nevertheless, the laparoscopic, sphincter-sparing
approach indeed has been shown to significantly reduce postoperative wound complica-
tions and hospital stay [9,10,12,14]. Since wound complications have a negative impact
on functional prognosis, the advantage of minimally invasive techniques is increasingly
evident [1,3,4,9,10,12,14,15]. Other approaches to reduce wound complications such as
preoperative bowel preparation, prolonged postoperative fasting and antibiotics, as well as
application of a vacuum-assisted pump have also been described with varying degrees of
success [4,16–18]. While laparoscopy is useful for high forms of anorectal malformations, it
is not as helpful for low lesions.
To date, there are only a few reports describing minimal invasive techniques for
low lesions. Pakarinen et at. described the “Transanal Endoscopic-Assisted Proctoplasty
(TEAPP)” [2,11]. They performed a sigmoidostomy in seven patients with ARM without a
fistula in term of a staged surgical approach. Via colostomy, the absence of a fistula was
confirmed (high-pressure colostogram) before implementing the TEAPP procedure. A
retrograde endoscopy through the sigmoid mucous fistula was performed to visualize the
termination of the rectum. In case a low malformation was confirmed by using translumina-
tion of the endoscope light from the rectum to the anal dimple within the external sphincter,
correction via TEAPP was performed (successful in four of the seven patients). The rectum
was incised from below and the neoanus was created under endoscopic visual control,
similar to the ePARP procedure described in our report. They suggested that this technique
allows anatomical reconstruction of the anorectum, by placing the anorectum within the
sphincter complex under endoscopic control [11]. In this study, the TEAPP procedure was
aborted and converted to a PSARP in three of the seven recruited patients, mainly because
transillumination could not be positively confirmed. The question of transillumination
raises the question of the maximal distance between skin and pouch in those without a
fistula that is repairable by ePARP. In our series, the maximal distance was 3 cm. Using
the hooks, it was still feasible to bring the mucosa down to the anus without difficulties
for anastomosis. Nevertheless, the distance between the pouch and the skin may be a
Children 2022, 9, 587 8 of 11

limitation of the PARP technique, making it applicable only to low-type lesions where the
mucosa can be retracted downward and anastomosed to the skin. This approximation,
however, results in a nicely inverted skin rosette and may prevent prolapse, which we have
not seen as a complication in our series.
While another option may be to perform a limited perineal skin incision to access
the distal rectal pouch under direct vision, we believe that using ultrasound, radiography,
or endoscopy allows us to penetrate through the center of the sphincter complex with
a needle, limiting dissection and associated damage, much like during the laparoscopic
approach for higher lesions.
In contrast to the generally accepted concept that even in low forms of ARMs without
fistula there is intimate contact between the rectal blind sac and the posterior urethra [1],
Pakarinen et al. describe the midpoint of the distal rectal termination to be right above the
anal site within the sphincter muscle complex and not intimately related to the urethra.
This finding may disprove the argument that the close relationship between the rectum
and the urethra justifies the need for PSARP in low forms of ARMs [2,11].
The results regarding the percutaneous anorectoplasty procedure (PARP) described
in this article show comparable advantages to the TEAPP procedure. The minimally
invasive approach may help avoid potential complications associated with PSARP in
select, eligible patients. Furthermore, the high success rate in our study (90%; only one
patient was converted to a PSAPR procedure) indicates that suitable cases can be reliably
identified preoperatively.
In contrast to TEAPP, the PARP procedure allows, in addition to minimal invasive
correction of patients with low ARMs, the correction of male patients with a perineal fistula
(anocutaneous, rectoperineal outside the sphincter complex). These types of malformations
are currently still recommended to be reconstructed by posterior sagittal anoplasty [19].
However, there is evidence suggesting that overall functional outcome is comparable after
minimally invasive anoplasty and PSARP for perineal fistula in boys [20]. Additionally,
in contrast to the TEAPP, the ePARP in our series is performed not only using endoscopic
guidance, but under concomitant fluoroscopic control. In our opinion, this is essential for a
safe, precise reconstruction of the anorectum.
Obviously, the ePARP procedure requires a prior colostomy for antegrade endoscopy,
but also for the preoperative exclusion of an occult rectourethral or rectovesical fistula by
high-pressure distal colostogram [19]. However, the iPARP procedure does not require
a colostomy and therefore may be an option when the invertogram clearly shows the
blind-ending rectum and there are no signs of a fistula.
Relevant complications of colostomies in newborns include wound complications,
prolapse, leakages, parastomal hernias, or bowel obstruction [21]. Therefore, colostomies
should be avoided if possible, particularly in males with perineal fistulas. Another ar-
gument in favor of a one-stage procedure is the so-called “brain–defecation reflex” that
may remain intact following the “use it or lose it” principle [22,23]. Finally, there is
evidence of one-stage procedures affording similar outcomes compared to multi-stage
procedures. This raises the question whether liberal placement of a colostomy is generally
warranted [9,16,24].
In our series, only one perioperative complication occurred during the PARP proce-
dure, namely, the presence of the Foley catheter in the rectum upon visualizing the rectum
from the perineum. The unanswered question remains whether the posterior urethra was
injured during the procedure or whether the patient had a low rectourethral fistula in
addition to the perineal fistula in the first place. According to the literature, such H-type
anorectal malformations have an incidence of around 3 percent [25], ranging from 0.1 to
16 percent [26]. Therefore, pediatric surgeons should have a high index of suspicion when
performing any of these procedures. Conversion to a PSARP in our case 2 afforded the
patient a good outcome. Surgeons attempting a PARP procedure should maintain a high
index of suspicion for rectourethral fistulae and should convert to PSARP if there is any
indication that anatomy is not as preoperatively suspected. In our case, the patient did not
Children 2022, 9, 587 9 of 11

have a micturating cysturethrogram, which would have been helpful. To ensure patient
safety, accurate preoperative evaluation of the underlying anatomy and, accordingly, the
selection of the appropriate surgical technique is crucial. This refers to the level of the
ARM, the relation of the rectal pouch to the muscle complex as well as the evaluation of a
rectogenitourinary communication [1,2,19]. These aspects may be estimated by a lateral
pelvic radiograph, ultrasound, cystoscopy, or micturating cystourethrogram (MCUG), even
though the results of these examinations may be inaccurate in some cases [2,10,27].
There were no complications during the ePARP procedures throughout our study. In
our opinion, the ePARP procedure, including employing intraoperative fluoroscopy, offers
the safest technique, especially in cases where preoperative diagnostics have not provided
complete clarity regarding the exact type of ARM. The relevance of accurate preoperative
diagnostics also applies to perioperative guidance. Using a percutaneous technique without
some kind of image guidance (nPARP) has a high potential risk of creating false tracks and
causing complications in neighboring structures such as the urethra, as seen with patient
number 2 in this series. We therefore do not recommend performing the nPARP procedure.
Functional outcomes in most children were highly satisfactory in terms of continence
and functionality, with only two cases of constipation and four patients with the need of
anal dilations. We are aware that the follow-up in this study was too short to draw any
conclusion concerning long-term functional outcomes. However, there is evidence showing
that long-term results in low malformations are good in most patients if perioperative
complications are prevented [1,2,19]. Furthermore, long-term follow-up of these patients
in terms of functionality remain controversial and is generally influenced by confounding
factors, including a high incidence of associated anomalies [20,28].
The study is limited by its relatively small sample size, the retrospective design and
heterogeneous population. Furthermore, the technique was implemented by multiple
surgeons. However, all surgeons had comparable experience in the field of pediatric
surgery and had discussed the exact technique prior to the interventions. These factors
mean that our data can only demonstrate a trend and that, so far, no precise statement can
be made about certain secondary endpoints, such as the operating room time.
This is the first study investigating the clinical outcome after PARP procedure as well
as describing the different options of visual guidance. The PARP procedure seems to offer
a safe and individually tailored minimally invasive surgical approach to avoid unnecessary
invasive surgery in eligible patients. Prospective studies with larger populations are needed
to confirm these findings.

Supplementary Materials: The following videos can be downloaded at: https://www.mdpi.com/

article/10.3390/children9050587/s1, Video S1: Description of the PARP procedure without image
guidance in a newborn male with anorectal malformation and a rectoperineal fistula, Video S2:
Description of the ePARP procedure in a 6-month-old girl with Down syndrome who had a transverse
colostomy in an outside hospital.
Author Contributions: J.K., V.v.E., N.R.M. and A.-S.H. compiled the pertinent information and
drafted the manuscript. S.R., T.K., J.G. and OM performed the procedures, contributed to the
evolution of the procedure, and reviewed the manscript. O.J.M. developed the procedures, recorded
the data on all procedures, produced the videos, and finalized the manuscript for publication. All
authors have read and agreed to the published version of the manuscript.
Funding: This research received no external funding.
Institutional Review Board Statement: The study was conducted in accordance with the Declaration
of Helsinki and approved by the Ethics Committee of the Ludwig Maximilian University Medical
Center Munich (protocol code: 22-0141 and date of approval: 4 March 2022).
Informed Consent Statement: Informed written consent was obtained from all parents to have their
children operated on using the respective new techniques.
Data Availability Statement: All data on which this publication is based are available from the
corresponding author (OM) upon reasonable request.
Children 2022, 9, 587 10 of 11

Conflicts of Interest: The authors declare no conflict of interest.

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