tóm tắt luận án 24 trang tiếng anh nghiên cứu ứng dụng phẫu thuật nội soi một đường rạch điều trị bệnh hirschsprung

Application of single-incision laparoscopic surgery for the treatment of Hirschsprung''''s disease in children.. Starting from that actual situation, to contribute to research on the effe

MINISTRY OF EDUCATION AND TRAINING MINISTRY OF HEALTH

HANOI MEDICAL UNIVERSITY

DAO DUC DUNG

RESEARCH ON THE APPLICATION OF SINGLE-INCISION LAPAROSCOPIC ENDORECTAL PULL-THROUGH FOR HIRSCHSPRUNG'S DISEASE

Specialism : Surgery Code : 9720104

ABSTRACT OF THESIS

HANOI – 2024

The thesis has been completed at

HANOI MEDICAL UNIVERSITY

The thesis will be presented to a board of university examiners and reviewer lever at Hanoi Medical University on … 2024

This thesis can be found at:

National Library of Vietnam

Library of Hanoi Medical University

THE LIST OF WORKS HAS PUBLISHED AND

RELATED TO THE THESIS

1 Dao Duc Dung, Bui Duc Hau, Pham Duy Hien Application

of single-incision laparoscopic surgery for the treatment of

Hirschsprung's disease in children Journal of Medical

Research 2023;162(1):198-205

2 Dao Duc Dung, Bui Duc Hau, Tran Anh Quynh, Le Quang

Du, Hoang Huu Kien, Le Hoang Long, Pham Duy Hien Single-incision laparoscopic surgery for the treatment of

Hirschsprung's disease in neonates Journal of Medical

Research 2023;165(4):148-156

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INTRODUCTION

Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the submucosal and myenteric plexus of the intestinal wall The aganglionic segment begins at the internal anal sphincter and extends upward to varying degrees Most cases of HD are diagnosed in infancy Surgery is the principle of treatment with many different methods, of which the three most commonly applied techniques are Swenson, Soave, and Duhamel surgery The goal of surgery is to remove the aganglionic colon and reconstruct the digestive tract by bringing the normal ganglionic intestine down and anastomose to the anal canal, preserving sphincter function In 1995, Georgeson first reported the application of laparoscopic surgery to treat HD, showing superior effectiveness compared to open surgery In 1998, De la Torre described the endorectal technique for aganlionic colectomy without abdominal exploration To date, the two most commonly used surgical routes are laparoscopic and transanal surgery

Classic laparoscopic surgery uses three or more trocars placed in different positions in the abdominal wall to perform surgery and, therefore, will leave surgical scars in the corresponding areas In order to further reduce trauma and have better cosmetic results, in 2010, Muensterer successfully applied Single-incision laparoscopic endorectal pull-through (SILEP) for HD This surgery only uses a single skin incision for the trocar instead of many sites like conventional surgery and thus will only leave a small scar or even no scar when the incision passes through the navel In Vietnam, in the period 2013-

2015, SILEP was researched with a national-level project by the research team

of the National Children's Hospital successfully applied to some diseases in children include HD However, the national-level project only selected children under 12 months old with an aganglionic segment limited to the sigmoid colon and did not evaluate bowel function for children over three years old Since then, there has been no idea of SILEP treating HD, so the issue of expanding indications for older children or with aganglionic segments above the sigmoid colon needs to be researched

The view of pediatric surgeons worldwide regarding the treatment of

HD is early surgery, one-stage surgery with minimally invasive methods Early surgery in the neonatal period is still discussed Although surgery in infants has some advantages over older children, such as less colon dilatation, thin abdominal wall, and easy view of the surgical field, assessing postoperative complications along with bowel function compared with children beyond newborn age, it will need to be further clarified to answer the question of choosing an age for surgery in the infancy or not Up to now, research on the application of SILEP to treat HD in pediatric patients is still limited, and the results still have many problems that need to be improved What cases is this

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method indicated for, what are the advantages and disadvantages of this technique, and what are the short-term and long-term results? These are issues that need attention Starting from that actual situation, to contribute to research

on the effectiveness of treatment of HD using minimally invasive surgical

techniques, the project "Research on the application of single-incision

laparoscopic endorectal pull-through for Hirschsprung’s disease" was

carried out with two goals:

1 Application of single-incision laparoscopic surgery protocol in treating Hirschsprung's disease in children

2 Evaluate the results of single-incision laparoscopic surgery in treating Hirschsprung's disease in children

Chapter 1 OVERVIEW 1.1 CHARACTERISTICS OF HD

1.1.1 Etiology and pathophysiology

HD is a congenital neurological disease, a disease of the neural crest The disease is caused by failure of proliferation, migration, differentiation, and/or survival of neural crest-derived cells, leading to varying lengths of aganglionic of the distal intestine HD has a complex genetic component with varying degrees of involvement in the cause of the disease Siblings with HD increase the risk of a child being born with the disease by about 3.6-7.8% About 30% of patients have chromosomal and/or congenital disabilities, and children with Down syndrome are 40 times more likely to have the disease

In HD, due to the absence of the enteric nervous system, the aganglionic intestine has frequent contractions and no peristalsis, accompanied by the proliferation of adrenergic nerves The above intestinal wall increases contractions to push stool through the constricted intestinal segment below, so the intestinal wall muscle layer becomes hypertrophied, and the mucosa and submucosa are infiltrated with inflammatory cells

1.1.2 Diagnosis of HD

Clinical characteristics:

Male children are more common with a male/female ratio of 3/1 to 4/1, mainly with short-segment adenopathy Newborns often present with delayed meconium defecation (> 48 hours), food intolerance, bloating, bile vomiting, and intestinal obstruction Children with long aganglionic segments often present in the neonatal period with symptoms of intestinal obstruction Children with HD may also have difficulty feeding, sometimes vomiting bile, severe constipation, failure to grow and bloating, or blood in the stool, fever, and diarrhea in the setting of enterocolitis Preoperative enterocolitis accounts for 6-60% In older children, symptoms of frequent constipation requiring enemas may occur, and there may be episodes of enterocolitis or episodes of intestinal obstruction The whole body shows signs of malnutrition and anemia Diagnosis is made in 65% of cases before one month of age and in 95% of cases before one year of age

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Radiology:

Children suspected of HD often have a colonogram with contrast or anorectal manometry Characteristics of HD on colon X-rays include images of the transition zone, dilated bowel, narrow bowel, irregular colonic contractions, mucosal abnormalities, and prolonged contrast retention (> 24 hours) and a rectal/sigmoid ratio less than 1 According to de Lorijn, X-rays have a lower average sensitivity and specificity of 70% and 83%, respectively, compared with an anorectal manometry and biopsy The gold standard for diagnosis is based on the histopathology of a rectal biopsy specimen at least 2 cm above the dentate line For the experienced pathologist, Hematoxylin and Eosin (H&E) stained specimens are usually sufficient to diagnose HD However, additional diagnostic tests such as acetylcholinesterase histochemistry or calretinin immunohistochemistry are beneficial in some cases where the diagnosis is difficult to establish

Classification:

The American Pediatric Surgical Association recommends the following standard nomenclature for determining the location of aganglionic segments:

• Ultrashort-segment: No clear definition and usage of this term should

Surgical techniques:

Many surgical approaches have been described for the treatment of HD All methods are based on Swenson's surgical principles, including removing the aganglionic colon and reconstructing the digestive tract by bringing the normal ganglionic intestine down and anastomose to the anal canal In

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addition, surgeries must preserve the ability to control bowel movements and urogenital function The long-term results of any technique are good if done correctly The three most commonly applied techniques include the Swenson technique (1948), the Duhamel technique (1956), and the Soave technique (1963)

Surgical incisions:

In the past, surgeons often performed classic incisions such as the midline, the left paramedian, the modified Pfannenstiel, the transanal, and the posterior sagittal approach Currently, the two most commonly applied incisions are laparoscopic surgery according to the principles of Georgeson (1995) and the transanal presented

by De la Torre (1998) Karlsen compared transanal and laparoscopic surgery with the aganglionic segment in the rectosigmoid and showed no difference in bowel function, operation time, hospital stay, and rates of early and late complication between the two methods

1.2 RESEARCH ON THE APPLICATION OF SILEP FOR HD

1.2.1 Surgical technique in SILEP for HD:

SILEP is a new step forward in laparoscopic surgery in general, aiming

to reduce trauma and achieve better cosmetic results for patients Although the general principle is to use only one incision to place the trocar for the camera and instruments, many different techniques have been successfully applied in practice Differences in technique may be in the incision, trocar placement, and use of special laparoscopic or conventional instruments

Incision and trocar placement:

The navel, with the advantage of being a natural hole during the fetal period, can hide scars and is used by most authors as the place to make incisions The incision can be a circumference around the upper or lower half

of the umbilical circumference, a midline through the umbilicus, or a Z-shaped umbilical incision Most operations on newborns can be performed without expensive specialized multiport systems; separate laparoscopic trocar placement through a single incision provides good efficacy and superior cosmetic results without additional cost

Difficulties in technique:

Until now, SILEP is still considered more challenging to perform, and therefore, the indications are more limited than classic laparoscopy Existing difficulties in performing SILEP are changing the working posture in a more unfavorable direction for the surgeon, collision between surgical instruments or collision between instruments and the camera, surgical operations are more complicated, the tools often cross each other, and many surgeons have to work with a new spatial geometric environment, losing the standard triangle principle, access to organs is also more limited There have been reports of successful SILEP using only camera and standard laparoscopic instruments without specially designed instruments

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1.2.2 Research on SILEP for HD in children

In 2010, Muensterer applied SILEP to treat HD for 6 patients, showing

no complications during surgery After surgery, all 6 patients recovered well, with an average of 7 postoperative days The study showed that although SILEP has technical difficulties, it can still be performed safely through an incision at the navel with good postoperative and outstanding cosmetic results

In 2012, Zhu performed subtotal colectomy using SILEP for 15 patients with long-segment HD As a result, there were no cases requiring conversion to laparoscopic or open surgery and no intraoperative complications

In 2013, Tang compared SILEP and conventional laparoscopic surgery and showed that SILEP was safe, feasible, and equal results to conventional laparoscopic surgery However, surgery is more difficult when the aganglionic segment is above the sigmoid

In 2015, Xia compared the mid-term results of SILEP and conventional laparoscopic surgery and found that SILEP had shorter operation time even though the proportion of patients with a high level of the aganglionic segment

in the SILEP group, while the mid-term results between the two groups were not different The study also confirms the cosmetic superiority of SILEP compared to conventional laparoscopic surgery

In 2016, Tran Ngoc Son reported the results of research on the application of SILEP to treat 144 pediatric patients of 5 groups, including (1) multicystic dysplastic kidneys and ectopic dysplastic kidneys; (2) choledochal cyst; (3) palmar hyperhidrosis; (4) HD; (5) high-grade imperforate anus The author concluded that SILEP is a highly feasible and safe method; no patient was converted to laparoscopic surgery or required open surgery, and there were

no intraoperative complications and deaths related to surgical technique SILEP with conventional laparoscopic instruments does not require expensive specialized instruments, is cost-effective, and is suitable for Vietnam's economic conditions SILEP gives good cosmetic results and brings practical benefits to patients

In 2021, Nguyen Thanh Liem reported applying SILEP to treat 40 children with HD in the period 2013-2015 Evaluating long-term results in 38 patients (95%) with an average follow-up time of 65 ± 9.9 months, including 2 patients with enterocolitis (5.3%), all patients had good results according to the Rintala scoring system for bowel continence

- Patients underwent SILEP surgery to remove the aganglionic colon segment, resect the rectum close to the anal canal while preserving the sphincter, pull the remnant colon/rectum through the anus, and anastomose it with the anal canal

- Patients were operated at the Department of Surgery, National Children's Hospital, following a standardized procedure and by a consistent surgical team

- Successfully performed SILEP cases and cases converted to open surgery or other techniques are included in the study However, only successful SILEP cases were evaluated in the final research results

- Postoperative follow-up lasted at least 12 months

- Parents or legal guardians of the patients consented to their participation in the study

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2.3 Technical process

2.3.1 Patient position and surgical team position

The patient lies supine horizontally on the operating table, the patient's buttocks are placed close to the edge of the table, the buttocks are raised about

30 degrees higher than the head, and the legs are apart and fixed For older children, lie along the operating table, the patient's buttocks close to the edge of the table, legs apart and fixed The patient had a gastric tube and a urinary catheter inserted

The surgeon stands at the patient's head, assistant 1 (camera holder) stands to the right of the surgeon, assistant 2 stands to the right of the patient's feet and the instrument nurse stands to the left of the patient's feet; screen to face surgeon

2.3.2 Anesthesia

Endotracheal anesthesia combined with sacral analgesia

2.3.3 Surgical technique

• Phase 1: Trocar placement:

- Make a skin incision along the upper border of the umbilicus which is less than 2 cm in length

- Insert a 5mm trocar into the abdomen

- After insufflation of CO2, insert a 3mm trocar to the left of the first trocar and a third trocar (5mm) to the right of the first trocar

• Phase 2: Laparoscopic dissection:

- Perform frozen section biopsies at two locations: one at the suspected aganglionic narrowed rectum and one at the suspected ganglionic dilated colon

- Create a window through the sigmoid mesocolon From this window, cauterize and cut the mesocolon of the sigmoid colon and then dissect the rectum close to the rectal wall

- Ligate the sigmoid and superior rectal arteries Mobilize the mesocolon to the level of the inferior mesenteric artery

- Bring the bowel segment for anastomosis down into the pelvis to assess mobility and mesenteric length

• Phase 3: Perineal surgery:

- Place a Lone Star retractor

- The mucosa is separated from the muscular wall through an incision 0.5-1cm above the dentate line

- Clamp the rectal muscular sleeve at the 12 o'clock position, make a longitudinal incision of about 1 cm, then proceed circumferentially dissection until the colon is separated from the anal canal

- Make a V-shaped myotomy at the posterior aspect of the muscular sleeve so that the length of the remaining musuclar sleeve from the dentate line

is 1.5 cm (for neonates) and 2 cm (for non-neonates)

- Resect the colon 5 cm above the biopsy site where ganglion cells were found

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- Create a coloanal anastomosis with a single layer of interrupted sutures The anastomosis is 0.5 cm above the dentate line Insert a rectal catheter through the anastomosis

• Phase 4: Irrigation of the Abdomen and Closure of Trocar Sites:

- Perform a laparoscopic inspection and irrigation of the abdomen Suture the trocar sites

2.4 Research indications

2.4.1 General research indicators for the study population

• Patient characteristics: Age, gender, weight, physical abnormalities, medical history

• Clinical symptoms: Delayed meconium passage, constipation, colitis, nutritional assessment

• Imaging: Barium enema, plain abdominal X-ray

2.4.2 Research indicators for objective 1: Application of the SILEP Protocol in treatment of HD in children

Recording parameters, advantages, difficulties, and complications in each surgical phase of the SILEP protocol

• Laparoscopic abdominal surgery phase:

- Length of skin incision (cm)

- Trocar insertion time (minutes)

- CO2 insufflation pressure (mmHg)

- Extent of aganglionic segment: Rectum, sigmoid colon, splenic flexure, ascending colon, small bowel

- Intraoperative frozen section biopsy

- Colon mobilization: Time (minutes), management of rectosigmoid vessels, extent of colon mobilization (sigmoid colon, descending colon, splenic flexure)

- Laparoscopic surgery time (minutes)

- Correlation between surgical time and length of aganglionic segment, length of resected bowel

- Intraoperative complications

- Surgical difficulties

• Perineal Surgery Phase:

- Time of rectal mucosal dissection (minutes)

- Time to prepare the muscular sleeve (minutes)

- Time of colon preparation for anastomosis (minutes)

- Length of agangionic segment and length of resected bowel (cm)

- Coloanal anastomosis: Time (minutes), anastomotic technique

- Perineal surgery time (minutes)

- Correlation between surgery in neonates and non-neonates in terms of colon diameter, length of resected bowel, and surgical time

- Difficulties in the perineal surgery phase

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• Overall Surgery:

- Surgical time (minutes), correlation between surgery in neonates and non-neonates

anesthesia) and management

- Assessment of fully successful surgery or need for additional trocar placement with another skin incision Conversion rate to conventional laparoscopic surgery or laparotomy

2.4.3 Research Indicators for Objective 2: Evaluation of the outcomes

of SILEP in treatment of HD in children

Early Outcomes: Recorded within 30 days after surgery

• Assessment of bowel function (hours)

• Postoperative treatment time (days)

• Number of bowel movements per day at discharge

• Record postoperative complications and management of complications

• Correlation between surgery in neonates and non-neonates

Post-discharged outcomes:

• Follow-up time: 1 month, 3 months, 6 months, 12 months, 24 months,

36 months, and at the end of the study

• Assessment of bowel inflammation: According to the Delphi criteria

• Assessment of surgical complications:

- Perianal dermatitis

- Anastomotic condition

- Postoperative complications and their management, outcomes

- Classification of postoperative complications according to Dindo

Clavien-• Assessment of Bowel Function after Surgery:

- Record the number of bowel movements per day

- Assess fecal incontinence, constipation, and encopresis using the Krickenbeck classification

- Overall outcome of bowel movement, based on the Wingspread criteria and Moore's classification for patients ≥ 36 months old, can be classified into 4 types as follows:

▪ Good: Continent of stool; no incontinence or constipation; occasional incontinence (grade 1) or constipation can be controlled by diet (grade 1)

▪ Fair: Continent of stool; occasional incontinence (grade 1); Constipation may be controlled by diet or requires laxatives (grade 2)

▪ Average: Continent of stool; daily incontinence, no social problems (grade 2); constipation requiring laxatives (grade 2)

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▪ Poor: Incontinent of stool; frequent incontinence, social problems (grade 3); constipation requiring enemas, unresponsive to diet and laxatives (grade 3)

• Assessment of long-term outcomes after surgery using a 15-question interview-based scoring system for children over 3 years old based on El-Sawaf's assessment Answers are scored accordingly, and the total score is divided into groups:

2.5 Data collection and processing

Data will be collected using a standardized case report form and analyzed using medical statistical methods in SPSS 26.0 and Prism 8.0 software Qualitative variables will be presented as percentages (%) and compared using the Chi-square test and Fisher's Exact test Quantitative variables will be presented as mean values (± SD), minimum, maximum, and median (and quartiles); compared using the t-test, one-way ANOVA, Mann-Whitney test, Kruskal-Wallis test, and Friedman test A p-value of less than 0.05 will be considered statistically significant

2.6 Research ethics:

The study will be conducted in accordance with the ethical principles of biomedical research The study has been approved by the Ethics Committee of Hanoi Medical University

Chapter 3 RESULTS

3.1 APPLICATION OF THE SILEP PROTOCOL IN TREATMENT OF

HD IN CHILDREN

3.1.1 Population characteristics

* Age and gender: Male/Female was 10.6:1 The mean age was 3.27 ± 2,95

months (range, 17 days - 15 months)

* Meconium defecation: an average of 36.6 hours after birth (range 6-72

hours) Meconium defecation after 48 hours accounts for 36.6%

* Associated defects: 4 patients (4.3%) had associated defects, all related to

Down’s syndrome, with or without other defects: congenital hearing loss, triscuspid regurgitation

* Chief complaints: 2 common chief complaints was abdominal distension and