Safety & Cost-Effectiveness of Primary Neonatal Posterior Sagittal Ano-recto Plasty (PNPSARP)

Background: Early repair of Anorectal malformation (ARM) within 6 months may be one of the factors that improve fecal continence. Delayed and multiple-stage repairs require dilatation, strict bowel preparation, fasting and total parenteral nutrition (TPN). PNPSARP requires neither bowel preparation nor parenteral nutrition. We believe it can be achieved within 72 hours of life. Aim: To evaluate the safety, feasibility and cost effectiveness of PNPSARP within 72 hours of life versus delayed or multiple-stage repair of vestibular and perineal stula. Material & Methods: A retrospective study was carried out of all newborns with ARM at our institute between August 2016 and August 2019. PNPSARP within 72 hours of life was compared with delayed or multiple-stage repair. Neither bowel preparation nor parenteral nutrition was required in the PNPSRP group. Perioperative complications and costs were evaluated. Results: Eight PNPSARP were compared with 7 delayed or multiple stage repair over the study period. Four babies (50%) were operated at day 1 post delivery in the PNPSARP group. Mean operative time (MOT) was 109 minutes (68-155). Mean follow up period was 22 months (12-36). One girl with a vestibular stula had wound infection. This group had a good outcome with no morbidity, high satisfaction rate and low costs in comparison to delayed or multiple-stage repair. Conclusions: PNPSARP for perineal and vestibular stula with supportive ancillary services in the rst few days of life appears to be safe and cost effective with minimal morbidity. PNPSARP is now the standard technique in our unit.


Background
Management of perineal and vestibular stula by delayed primary repair depends on clinical circumstances with programmed anal dilatation and bowel preparation. Multiple stage repair with colostomy, PSARP and closure of colostomy is another modality. Colostomy morbidity includes bleeding, prolapse, retraction and obstruction. These two modalities carry high cost, multiple operations, multiple admissions, psychosocial impact on parents and infants plus cosmetic outcome.
The main aim of primary neonatal repair of vestibular and perineal stula is to minimize multiple surgical trauma and to avoid the potential morbidities of colostomy 1 . Early repair of ARM within 6 months of life is one of the factors that improve fecal continence 2,3,4 . Some surgeons perform primary repair, many dilate with delayed repair, and many carry out an initial colostomy depending on their own clinical circumstances 2,3 . Delayed repair of perineal and vestibular stula requires pre-operative dilatation of the stula, strict bowel preparation, fasting and TPN. We believe it could be accomplished within 72 hours of life under antibiotic cover with early post-operative oral feeding. We have been carrying out neonatal primary repair in the rst few days of life for perineal and vestibular stula since 2003 with good outcome. PNPSARP is a demanding procedure, which requires a multidisciplinary team approach. It reduces stress on the parents, trauma to the baby, hospital admissions, number of procedures, cost and improves parent satisfaction. It also avoids a colostomy with its associated psychosocial impact.
Aim: To evaluate the safety, feasibility and cost effectiveness of PNPSARP within 72 hours of life versus delayed primary or multiple-stage repair of vestibular and perineal stula.

Material & Methods
After ethical approval from the IRB hospital ethical committee Reference Ethical (REC 225), the parents' consented to be involved in the study. A retrospective comparative study was carried out of all newborns who underwent PNPSARP within 72 hours of life for vestibular and perineal stula at our institute from August 2016 to August 2019.
Patients were divided into 2 groups: Group I who underwent PNPSARP in the rst 72 hours of life and Group II who underwent delayed or multiple-stage repair. Urine analysis, sacral x-ray, abdominal ultrasound and echocardiography were performed shortly after birth to rule out other congenital anomalies 1 . Preoperatively Gentamicin, Metronidazole and Ampicillin were given. Group I had neither bowel preparation nor TPN. Group II patients initially underwent colostomy and/or dilatation for vestibular and perineal stula. Informed consent was taken. After endo-tracheal intubation and caudal anesthesia, Foley catheter was inserted and patient positioned prone with bottom up. All patients underwent PSARP as described by Pena. Surgical loupes and Pena stimulator were used to identify the super cial parasagittal muscle bers and the "muscle complex" which is divided exactly at the midline in all cases 2 . The rectum mobilized and fully separated from vagina in case of the vestibular stula.
Perineal body was reconstructed. Anoplasty was performed in the middle of the muscle complex. Glue or opsite spray was applied at suture line to avoid wound contamination.
Milk feeding commenced on the rst postoperative day. The "neo-anus" was gently dilated in the third postoperative week. Laxative was not given routinely post repair. Parents were instructed for Hegar's dilatation guidelines.
Patients were followed regularly in the clinic to assess the outcome. Subjective data were collected from the outpatient clinic, chart review and patient satisfaction questionnaire. We compared both groups regarding cost effectiveness of surgical procedures, number of admissions and length of hospital stay (LOS). Quantitative measurement of outcome could be done only to measure the cost effectiveness.

Results
15 patients underwent repair of ARM during the period of the study. PNPSARP was performed for 8 patients while 7 patients underwent multiple stage or delayed primary PSARP. In group II; 3 out of 7 were girls with vestibular stula and had multiple-stage repair. Four boys with perineal stula had delayed repair. Seven patients (46%) were male and 8 patients (54%) were female. 7 cases (46%) had rectovestibular stula and 8 (54%) had perineal stula. Six babies (40%) had associated anomalies with cardiac and urinary systems being the most common. None of our patients has sacral deformities. In group I four babies (50%) were operated on day 1 post delivery, two (25%) on day 2, one (12.5%) on day 3 and one on day 4 (12.5%). Mean operative time (MOT) was 109 minutes (68-155). Mean follow up period was 26 months (12-36). One girl (12.5%) had super cial wound infection which was treated conservatively. All patients in Group I were breast-fed post-operatively and this was continued for a period of 3-6 months at home. Two patients had constipation, which responded to oral laxatives in one patient and enema in the second patient. Dilatation was continued in perineal stula patients for 4-6 months and followed by a delayed PSARP. This was preceded by the administration of bowel preparation.
Patients were given antibiotics, and kept NPO for two days pre-operatively, and ve days post-repair with TPN. Girls with vestibular stula in group II had a multiple stage repair (colostomy, PSARP and closure of colostomy). Group I had good outcomes with no scar ( gure1). There was a high satisfaction rate amongst parents and lower cost compared to Group II ((Figure2&3). Seven out of eight parents in Group I were satis ed in comparison to one out of seven in Group II. Dissatisfaction was mainly due to a longer waiting time for the de nitive surgical repair plus colostomy issues (Figure4). There was no signi cant statistical difference due to the small sample size.

Discussion
Management of ARM has gone through many modi cations during the last three decades. PSARP is considered now the gold standard repair after well understanding of the anatomy of anal sphincter complex by Pena in 1982 4,5,6 . ARM patients should have the best functional outcome with early e cient meticulous surgical repair plus management of associated anomalies 7,8 .
Pena suggested primary repair of low-lying rectum after a period of regular dilatation 5 . Our series is representing only those babies who had PNPSARP for vestibular and perineal stula in the rst 72 hours of life without any preparation. PNPSARP is labeled as a demanding procedure which requires special technical skills. We agree with Nagdeve et al that it is easier to operate on a virgin neonatal plane as tapering of the rectal pouch is not required 11 . The separation of the rectum from the hypertrophied vagina is easier due to the effect of the maternal hormones 12 . PNPSARP is now our approach in all types of ARM except recto-vesical stula as it saves the patient 1-2 operations and 2-3 admissions. We gained the technical skills required for PNPSARP by developing experience over time and regularly auditing our work. We use surgical loupes for easier dissection.
Some surgeons do primary repair, many dilate and delay the repair, and many do an initial colostomy depending on their own clinical circumstances 2,3 . Delayed repair of ARM requires dilatation, strict bowel preparation, fasting and TPN. Financial advantage and psycho-social resistance of colostomy popularized primary repair over the staged repair especially in developing countries 9,10 .
The driving forces for PNPSARP are parents and babies stress, multiple anesthesia, multiple admissions and high cost. Financial advantage and psycho-social resistance of colostomy popularized PNPSARP over the staged repair especially in developing countries and further multi centers studies are required to support it, Although stoma diversion decreases the risk of infection and dehiscence in PSARP 2,6,13 , it carries a risk of morbidities 13 . Only one girl with a vestibular stula had wound infection in group I. The infection was super cial and treated conservatively without any consequence. She had straightforward PSARP, but unfortunately she was delivered elsewhere and started feeding before transfer to our institute.
We have since modi ed our policy to exclude those who had commenced feeding.
Meticulous PNPSARP appears to provide the "best utilization of all existing resources" and might result in a better continence 17,18 , although some authors disagreed 19 . Constipation was encountered in 60% of published series 20 . In our study, content cannot be evaluated due to the short follow up period. The good outcome of PNPSARP is attributed to the accumulation of experience and the comprehensive ancillary services. We think that we must keep moving forward to operate our babies as early as possible, and hopefully without colostomy even for high lying rectum 8,11,18 , provided we evaluate our cases in an objective way, apply a strict auto-criticism and long term follow up. We found that PNPSARP can be done safely by our trainees under supervision of experienced surgeons with supportive ancillary services. We suggest adding this procedure to the pediatric surgery training curriculum as it is not more challenging than tracheo-esophageal stula repair. For patient safety the surgeon must take into consideration, his experience, the medical status of the patient and the surrounding circumstances on making a decision for PNPSARP. Our results suggest that PNPSARP is safe and cost effective.

Conclusions
PNPSARP for perineal and vestibular stula is safe with minimal morbidity, good parent satisfaction and low cost provided that the supportive ancillary services are available. PNPSARP is now the standard technique in our unit except for bladder neck stula, and we are recommending it as an excellent approach for ARM repair. Longer follow up for fecal and urinary incontinence is required to complement our ndings.

Declarations
Ethics approval and consent to participate Con ict of Interest Statement Author 1, declares that she has no con ict of interest.
Author 2, declares that he has no con ict of interest Author 3, declares that he has no con ict of interest Author 4, declares that he has no con ict of interest Page 6/12 Author 5, declares that he has no con ict of interest Author 6, declares that he has no con ict of interest Author 7, declares that she has no con ict of interest Statement of Ethics: All procedures performed in studies were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards and approved by KFAFH IRB.

Author Contributions
Corresponding Author: Drafting the work