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Anorectal malformations (ARM) affect 1 in 5000 live births. The majority of ARM can be repaired through a posterior sagittal incision as the rectum is reachable from below. However there are some types of anorectal malformations which need an abdominal approach. This abdominal approach is ideally performed with minimal invasive surgery. In this chapter we highlight the indications and the surgical technique to perform laparoscopic assisted anorectal pull through.

Congenital abnormalities of the pelvic floor in children are strictly connected to anorectal malformations (ARM). The incidence of ARM is 1:5000, so these are relatively common anomalies. The assessment and management of that group of patients is a routine practice of pediatric surgeon, radiologist, and neonatologist. A significant group of patients with ARM will grow up and as adults need care of such specialists as gynecologist, surgeon, urologist, gastroenterologist, proctologist, and radiologist. Basic information included in this chapter is addressed for them. The spectrum of the ARM is very wide. The anatomical and clinical aspects of the rectal anomalies are quite different in male and female patients and thus have to be presented separately. The surgery of ARM has achieved major progress in the last 30 years. The mortality and morbidity diminished markedly, and long-term results assessed as social continence improved significantly.

Anorectal malformations, or imperforate anus, represent a wide range of congenital defects and are associated with other congenital anomalies, including cardiovascular, urologic, gastrointestinal, and musculoskeletal disorders. Appropriate work-up for associated anomalies should be performed prior to definitive management. After the diagnosis of an imperforate anus is made on newborn examination, evaluation for a fistula should be performed, and oftentimes the definitive anatomy is not known until after a fistulogram. In females with a single perineal opening or cloaca, the common length channel should be assessed using cystoscopy, vaginoscopy, and possibly magnetic resonance imaging (MRI). Hydrocolpos should be drained if found. In a patient without a fistula, an abdominal X-ray should be performed to evaluate the distance between the distal gas and the skin. In patients with more complicated anomalies, such as cloaca, recto-urethral fistulas and patients with rectal gas above the coccyx on abdominal X-ray, colostomy should be performed first and definitive repair delayed until further work-up can be performed. Otherwise, superficial fistulas may be managed with anoplasty and deeper fistulas managed with posterior sagittal anorectoplasty in males or posterior sagittal anorectovaginourethroplasty in females.

This chapter describes the posterior sagittal/laparoscopic/surgical procedure for posterior sagittal anorectoplasty (including laparoscopic ligation of __fistula) if anatomically appropriate, as performed by the following approaches: open and laparoscopic. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: identification of the fistula, marking the limits of the sphincter mechanism, dissection of the fistula/rectum from urethra/prostatic urethra or bladder neck, mobilization of rectum, and performing the anoplasty.

Managing colorectal surgery conditions in infants and children presents a highly detailed and technical challenge that, in many cases, requires formal training, experience, and expertise. In addition to the wide array of congenital anomalies that surgeons may encounter, this chapter will cover conditions that are also experienced in adults; however, the evaluation and management of these disease states needs to be tailored to the pediatric population.

Intestinal obstruction is one of the most common pathologies in pediatric patients and has historically been a major cause of neonatal and infant mortality due to either an inaccurate or tardy diagnosis leading to a delay in surgical intervention and supportive care (Glover and Barry, Ann Surg 130:480–509, 1949). Earlier recognition and appropriate care has led to increased patient survival with surgical correction that is usually the definitive treatment in almost all cases. Anesthetic management for a pediatric patient with intestinal obstruction is not always straightforward and can present several challenges to the pediatric anesthesiologist. This chapter will discuss the most common causes of pediatric intestinal obstruction and the perioperative and anesthetic considerations involved in the care of these patients. Table 43.1 provides a guide to the care of newborns, infants and children with intestinal obstruction.

Pull-through procedures involve complete or distal proctectomy with restoration of gastrointestinal continuity at or below the level of the anorectal junction. Examples include coloanal anastomosis, ileal pouch-anal anastomosis, perineal proctectomy, and transanal or transabdominal endorectal pull-through procedures in children for Hirschsprung’s disease or high imperforate anus. Common indications include distal rectal cancer, rectovaginal or rectourethral fistulae, inflammatory bowel disease, and rectal prolapse. Complications of these procedures are diverse, including bleeding, anastomotic leak, chronic anastomotic sinus, anastomotic stricture, and prolapse. Children who had previously undergone a pull-through procedure to treat Hirschsprung’s disease or imperforate anus may suffer from fecal incontinence or constipation with poor emptying of the neorectum. Although pull-through operations may result in a myriad of complications, there are several nonoperative and operative management strategies to not only treat the acute complication but also achieve a functional anastomosis without a permanent stoma. Nonoperative strategies include endoscopic and percutaneous procedures. An expeditious diagnosis and treatment of anastomotic complications may allow for local, less invasive methods to adequately treat the problem, ultimately preventing long-term anastomotic failure. Resection and reanastomosis should be considered the treatment of last resort following a pull-through procedure.

Congenital abnormal fistulas between urethra and anorectum or vagina are usually associated with imperforate anus. Congenital recto or anourethral fistulas without anorectal malformations are extremely rare in males and have been called N-type or H-type fistulas1. In many series, this fistulas are not considered under the types of congenital urethra fistula, because these abnormalities have been included as a subtype of anorectal malformation (ARM), even in cases of normally located anus. There is no general agreement about urethral fistula as an identified anomaly, as it may be considered as a sort of urethral duplication or an associated anomaly with other congenital malformations.

Anorectal malformation is a well-documented spectrum of disorders that has been reported in 1/4000–1/5000 births. The exact etiology is not well understood, and it has been linked to some genetic and familial dissociations. This wide spectrum of congenital disorders ranges from imperforate anus with or without fistulas to the urethra, bladder, or perineum to complex anogenital malformations involving the entire ano-genito-urinary tract. The open posterior sagittal anorectoplasty (PSARP) is the current standard for surgical management of anorectal malformations. Introduction of laparoscopy allowed better visualization of the depth of the infant’s pelvis and safe dissection of high types of anorectal malformations and led to the development of a minimally invasive laparoscopic approach for high imperforate anus, introduced in 2000 by Georgeson et al.

In any age group, creation of an intestinal stoma is a significant event with major physiologic, body image, lifestyle, and psychosocial implications. Management and indications for ostomies in infants and young children are often different than in adolescents and adults. This is a consequence of the differing diagnoses, physiology, size, growth and development issues, complications, and unique patient and parental adjustment concerns.

The authors describe this very special, unique defect that only occurs in approximately 5 % of all cases of anorectal malformations. An important feature of this defect is the fact that most of these patients also suffer from Down syndrome. The authors emphasize the fact that even when Down syndrome babies are developmentally delayed, most of them achieve bowel control when they are subjected to a meticulous, detailed operation. Emphasis is placed on the fact that even when these patients do not have a connection with the urinary tract, the separation of the rectum from the urinary tract requires a very meticulous and detailed maneuver.

This is a comprehensive review of historical documents presented in chronological order, in an attempt to divide the history of the treatment of anorectal malformations into different stages: (a) the early stage, (b) the blind surgical treatment era, and (c) the modern conception, based on the knowledge of the true intrinsic anatomy of anorectal malformations.

Hydrocephalic patients can exhibit varied medical problems, from the neonates that are underweight with acute meningitis/ventriculitis or intraventricular hemorrhages to the chronic infections such as tuberculosis that can occur in any age group, rendering some of them difficult to manage by the ventriculoperitoneal (VP) shunt, ventriculoatrial (VA) shunt and endoscopic third ventriculostomy (ETV) procedures.

Imperforate anus is a condition affecting males and females, in which the anus fails to form in the correct location. There is wide variation in abnormality requiring individualization of treatment. Often imperforate anus is accompanied by other anomalies.