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Key Concepts • Cloacal anomalies: These are among the most complex of pediatric colorectal conditions. Careful anatomic assessment must be done prior to surgical correction. Long-term follow-up is essential as future gynecological and genitourinary problems may arise that also need corrective action. • Anorectal atresias: Imperforate anal anomalies are commonly associated with both genitourinary and spinal anomalies; both of which may complicate the long-term management and care of such patients. Life-long incontinence may be seen in up to 50 % of such patients. • Hirschsprung disease: While surgical correction will result in a good stooling pattern. Many patients may suffer from recurrent enterocolitis after a pull-through, which requires immediate attention by a surgeon experienced in dealing with this secondary disorder. Children may also occasionally suffer from anorectal incontinence. In general, they need to be followed long-term for similar potential problems. • Necrotizing enterocolitis (NEC): NEC may lead to fullthickness intestinal necrosis, resulting in the loss of a considerable amount of small and/or large intestine. Even without intestinal loss, an ischemic-associated stricture may occur, which is typically in the splenic flexure region, and may require dilation or resection of this portion of the colon. • Inflammatory bowel disease: Diagnosis and the treatment are quite similar to adults. A key consideration is the need to ensure adequate growth and maturation through adolescence. Often this plays directly into the timing and decision for surgery. • Constipation: Constipation can be challenging and typically can be initially approached with medical management. It is important to rule out Hirschsprung disease and anatomic obstruction. For the most intransigent of cases, some will benefit from an appendicostomy.

Introduction Pediatric colorectal disease processes range from complex congenital developmental disorders to a wide range of acquired disorders. This chapter, while not trying to be a comprehensive review, will discuss some of the key colorectal disorders that are commonly cared for in childhood, and often carry with them life-long issues that colorectal surgeons may often encounter. Thus, familiarization with both of these types of disorders, and the management of potential complications, is paramount.

Congenital Anomalies Cloacal Anomalies Cloacal anomalies are the most severe and complex form of anorectal malformation (ARM) and occur in ~1:20,000 livebirths [1]. Embryologically, the cloaca is a transient organ that becomes divided to separate the gastrointestinal tract from the genitourinary tract [1]. Clinically, a cloaca describes the condition in which the urethra, vagina, and rectum empty into a single channel with a single perineal orifice, located on the anterior perineum at the expected site of the urethra [2] (Figure 64-1). Typically, this is associated with a hypoplastic formation of the labial structures, and the perineal opening may be so small that adequate removal of secretions m