History case

A child born in term from the Ist pregnancy with nephropathy, polyhydramnios. The Apgar score 7-8. Birth weight 3000 gr. For the first time was fed with breast milk on the 2d day, he sucked actively but had profuse regurgitations immediately after feedings. On the first day he had poor stool (green color mucus), the next days stools were absent. Along the feeding volume increasing the milky vomiting became more prominent, and the body weight loss occurred. At the age of 4 days the child is in a poor condition, he is flaccid, adynamic, the skin turgescence is decreased. The teguments are pale with cutis marmorata. The respiration is hard without rales. Heart sounds are softened, the pulse rate is 160 b/min. The abdomen is soft and hollow. At the gastric probing 60 ml of milk without impurities were obtained. The rectal ampulla is empty. The body weight at the 4th day is 2460 gr., blood tests pH=7,51, pCO2 -34,6 mm Hg, BE - +4,5, Hb 180 g/l, Ht 75%, plasma Na+ - 132 mEq/l, plasma K+ - 3,8 mEq/l.

It is not a lupus !!!

History case

Esophageal atresia Pediatric hypertrophic pyloric stenosis Duodenal atresia (above papilla Fateri). Jejunoileal atresia.

N. Testemitanu State University of Medicine and Pharmacy Department of Pediatric Surgery, Orthopedics and Anesthesiology

Intestinal Atresia, Stenosis, and Webs

Jalba Alexandru, MD, PhD, associate professor

Chisinau 2012

GI development
The GI tract that looks like this, starts up as a straight tube consisting of the foregut, hindgut and the midgut. The midgut will further become the duodenum, the jejuno-ileum and the proximal colon. In the course of development the midgut forms a loop that twists counterclockwise on itself and fixes like this. In the course of this rotation the blue loop becomes the proximal colon, the yellow part forms the duodenum and the green one the jejuno-ileum. At the end of embryogenesis the GI tract looks like in

an adult.

Duodenal Stenosis and Atresia

Embryology
The hepatobiliary system and pancreas form during the third week of gestation, as the second portion of the duodenum gives rise to biliary and pancreatic buds at the junction of the foregut to the midgut.

Embryology
The duodenum also undergoes a solid phase during this time; between 8-10 weeks' gestation, the duodenal lumen is reestablished by the gathering of vacuoles, and recanalization occurs. Insults during this crucial period of development are believed to result in failure of recanalization and consequent atresias, stenoses, and webs. In addition, duodenal atresias have been associated with a closely surrounding piece of pancreatic tissue. Whether this tissue is an annular pancreas or merely a failure of duodenal development is debatable.[1, 2]

Classification
Anatomically, duodenal obstructions
are classified as either atresias or stenoses.

An incomplete obstruction due to a

fenestrated web or diaphragm is considered a stenosis.

Most stenoses involve the third and/or fourth part of the duodenum.

Classification
Atresias, or complete obstruction, are further classified into three morphologic types

Classification
Type I atresias account for more than 90% of all duodenal obstructions and contain a luminal diaphragm that includes mucosal and submucosal layers. A diaphragm that has ballooned distally (windsock) is a type I atresia. The windsock deformity is of particular concern because a portion of the dilated duodenum may actually be distal to the actual obstruction. Type II atresias are characterized by a dilated proximal and collapsed distal segment connected by a fibrous cord.
Type III atresias have an obvious gap separating the proximal and distal duodenal segments.
I II III

Duodenal web windsock anomaly

a prolapsing membrane in the duodenum

Classification
Unlike other intestinal atresias, duodenal atresias are associated with other congenital anomalies.
Approximately 50% of patients with duodenal atresias have some form of anomaly (eg, cardiac, anorectal, genitourinary), and as many as 40% have trisomy 21.[3, 4, 5] Esophageal atresia and the VATER (ie, vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, and renal and radial anomalies) syndrome have also been associated with duodenal atresia.[6] Hence, all neonates with duodenal atresia should be assessed for concomitant malformations. Growth retardation and polyhydramnios are often present prenatally.

Pathology
Anatomically, most congenital duodenal obstructions are considered periampullary, with the biliary outlet occurring proximal or distal to the site of obstruction. The degree of obstruction dictates the amount of resulting pathology. The obstruction causes dilation of the proximal duodenum and stomach as well as hypertrophy and distension of the pylorus. A common variation is the windsock anomaly, in which the duodenum is dilated distal to the point of obstruction because of a prolapsing membrane or web (see the image below). This may be confused with a more distal duodenal obstruction.

Clinical manifestations

Clinically, the presentation of the neonate with duodenal obstruction varies depending on whether the obstruction is complete or incomplete, and on the location of the ampulla of Vater in relation to the obstruction In the newborn, clear or bilious emesis is evident within hours of birth, with or without abdominal distension. An output of more than 20 mL of gastric contents is indicative of possible obstruction as normal aspirate is less than 5 mL Because of incomplete obstruction, patients with a stenosis or web may present later with dehydration or failure to thrive.

Diagnosis

Prenatal

ultrasonography

may indicate structural and associated abnormalities, and a double bubble sign such as

dilated

stomach

and

proximal duodenum.

Diagnosis

Polyhydramnios is highly suggestive of a proximal GI tract obstruction because the fetus is unable to swallow the amniotic fluid. Common associated anomalies and chromosomal defects may be assessed by screening maternal serum and amniotic fluid.

Diagnosis

Plain radiography is helpful and may reveal the classic double bubble sign, representing air in the stomach and proximal duodenum, which is associated with complete or near complete duodenal obstruction. Upright and contrast radiography using air or contrast may confirm the diagnosis (see the images below). Malrotation with volvulus may also result in duodenal obstruction with a double-bubble sign and can coexist with duodenal atresia in as many as 30% of cases.[8]

Imaging

Plain abdominal X-ray reveal a duodenal atresia

Contrast illustrating atrezia

GI study a duodenal

Imaging

A contrast study illustrating duodenal stenosis.

Plain radiograph of duodenal stenosis.

Treatment

Gastric decompression is essential to prevent aspiration, and thermoregulation should be monitored at all times.

Unless malrotation with volvulus remains a concern, preoperative assessment of other associated anomalies should be performed.

When fluid resuscitation and a full assessment have been

accomplished, the neonate may proceed to surgery.

Surgical treatment

Two basic options for repair of duodenal obstruction secondary to a web or atresia are noted: duodenoduodenostomy and duodenotomy with excision of the web.

Duodenoduodenostomy

Duodenotomy and web excision

Surgical treatment

The most common repair of a duodenal atresia is a duodenoduodenostomy open or laparascopic. For the open approach, a right upper quadrant supraumbilical transverse incision is made. Currently, a proximal transverse to distal longitudinal, or diamond-shaped anastomosis (see figure below) is preferred. Catheter is inserted through the stomach to help define the site of obstruction. In addition, the entire small bowel is carefully explored for other sites of obstruction. Care is taken to examine the gallbladder to ensure that a preduodenal portal vein is not evident and to locate the ampulla of Vater to avoid inadvertent injury.

Surgical treatment

The technique of duodenoduodenostomy. A diamond-shaped anastomosis is created via the proximal transversely oriented and distal vertically oriented duodenotomies

Outcome

An anastomotic leak, injury to the bile duct, and sepsis are early complications. Late complications include peptic ulceration secondary to alkaline reflux, blind-loop syndrome due to duodenal stasis, and recurrent obstruction. In one series, as many as 12% of patients had late complications requiring reoperation for a number of reasons, including reflux and peptic ulcer disease, associated biliary abnormalities, and bowel obstruction. The prognosis is good for patients with a repaired duodenal stenosis or atresia; however, coexisting diagnoses, such as Down syndrome and cardiac anomalies, affect the outcome. Recent long-term data demonstrated that a 9% combined early and late mortality rate over an average 6-year follow-up period was due almost exclusively to associated congenital anomalies. In addition, birth weight has been shown to be a predictor of mortality. Infants with duodenal atresia who weigh less than 2 kg at birth have poorer survival, regardless of the presence of other congenital abnormalities.

Jejunoileal Stenosis and Atresia

Etiology
Jejunoileal atresia occurs in approximately 1 in 5000 live births.
It occurs equally in males and females, and about one in three infants is premature. Although the majority of cases are thought to occur sporadically, familial cases of intestinal atresias have been reported.

Etiology
It is generally accepted that jejunoileal atresia occurs as a result of an intrauterine ischemic insult to the midgut affecting single or multiple segments of the already developed intestine.

End arteries Precarious blood supply Relative ischemia Impaired function

Vessels trombosed Precarious blood supply

No blood supply Critical ischemia Irreversible changes Disintegration Atretic segment

Defective propulsion Early necrosis

Intrauterine vascular disruption can lead to ischemic necrosis of the bowel with subsequent resorption of the affected segment or segments.

Etiology (hereditary implications)

Although jejunoileal atresias are usually not hereditary, there is a welldocumented autosomal recessive pattern of inheritance of multiple atresias first documented in Montreal and later identified elsewhere.
In these cases of inherited jejunoileal atresia, rotation was normal, mesenteric defects were never observed, and lanugo hairs and squamous cells were not identified distal to the most proximal atresia, suggesting an early intrauterine event. This disorder is uniformly lethal even with successful bowel resection. No correlations have been found between jejunoileal atresia and parental or maternal disease. However, the use of maternal vasoconstrictive medications as well as maternal cigarette smoking in the first trimester of pregnancy has been shown to increase the risk of small bowel atresia. Chromosomal abnormalities are seen in less than 1% of the patients.

Pathology
Following the Grosfeld classification, the defects of jejunoileal atresia are separated into four groups based on the type of atresia, with an additional consideration for

type III(b) (apple peel or Christmas tree appearance)

(See figure below).

Classification

Classification
This classification has significant prognostic and

therapeutic value because it emphasizes the importance of associated loss of intestinal length, abnormal collateral intestinal blood supply, and concomitant atresia or stenosis. Regarding classification, the most proximal atresia determines whether the atresia is classified as jejunal or ileal atresia. Multiple atresias are found in up to 30% of patients.

Stenosis
Stenosis is defined as a localized narrowing of the intestinal lumen without disruption in the intestinal wall or a defect in the mesentery. At the stenotic site, a short, narrow, somewhat rigid segment of intestine with a small lumen is found. Often the muscularis is irregular and the submucosa is thickened. Stenosis may also take the form of a type I atresia with a fenestrated web. Patients with jejunoileal stenosis usually have a normal length of small intestine.

Type I jejunal atresia

In type I atresia, the intestinal obstruction occurs secondary to a membrane or web formed by both mucosa and submucosa while the muscularis and serosa remain intact. On gross inspection, the bowel and its mesentery appear to be in continuity. However, the proximal bowel is dilated while the distal bowel is collapsed. With the increased intraluminal pressure in the proximal bowel, bulging of the web into the distal intestine can create a windsock effect. As with stenosis, there is no foreshortening of the bowel in type I atresias.

Type II atresia
The clinical findings of a type II atresia are a largely dilated, blindending proximal bowel loop connected by a fibrous cord to the collapsed distal bowel with an intact mesentery. Increased intraluminal pressure in the dilated and hypertrophied proximal bowel may lead to focal proximal small bowel ischemia. The distal collapsed bowel commences as a blind end, which sometimes assumes a bulbous appearance owing to the remains of an intussusception. Again, the total small bowel length is usually normal.

Type III(a) atresia

In type III(a) atresia, the atresia ends blindly with no fibrous connecting cord to the distal intestine. A V-shaped mesenteric defect of varying size is present between the two ends of intestine. The dilated, blind-ending proximal bowel is often aperistaltic and frequently undergoes torsion or becomes overdistended, with subsequent necrosis and perforation occurring as a secondary event. In this scenario, the total length of the small bowel is variable (but usually less than normal), owing to intrauterine resorption of the affected bowel.

Type III(b) atresia

Type III(b) atresia (apple peel, Christmas tree, or maypole deformity) consists of a proximal jejunal atresia, absence of the superior mesenteric artery beyond the origin of the middle colic branch, agenesis of the dorsal mesentery, a significant loss of intestinal length, and a large mesenteric defect. The decompressed distal small bowel lies free in the abdomen and assumes a helical configuration around a single perfusing vessel arising from the ileocolic or right colic arcades.

Type IV atresia
Multiple-segment atresias or a combination of types I to III are classified as type IV. Twenty to 35 percent of infants affected with jejunoileal atresia present with multiple atresias.

A familial form of multiple intestinal atresia involving the stomach, duodenum, and both the small and large bowel has been described. It is associated with prematurity and shortened bowel length. To date, it has been uniformly fatal. It is associated with type I and II atresias, with type II predominating.

Pathophysiology
The vascular and subsequent ischemic insult not only causes morphologic abnormalities but also adversely influences the structure and subsequent function of the remaining proximal and distal bowel. The blind-ended proximal bowel is dilated and hypertrophied with histologically normal villi, but without effective peristaltic activity. A deficiency of mucosal enzymes and muscular adenosine triphosphatase has also been found. At the level of the atresia, the ganglia of the enteric nervous system are atrophic with minimal acetylcholinesterase activity.

These changes are most likely secondary to local ischemia.

Obstruction alone can elicit similar, but less severe, morphologic and functional abnormalities.

Clinical manifestations
In neonates with atresia or stenosis, the presenting symptoms are consistent with bowel obstruction, including bilious emesis and abdominal distention.
Although the meconium may appear normal, it is more common to see gray plugs of mucus passed via the rectum. Occasionally, if the distal bowel in type III(b) atresia is ischemic, blood may be passed through the rectum. Intestinal stenosis is more likely to create diagnostic difficulty when compared with intestinal atresia. Intermittent partial obstruction or malabsorption may improve without treatment. Clinical investigations may initially be normal. However, these infants usually develop failure to thrive and ultimately progress to complete intestinal obstruction and require exploration.

Diagnosis
The diagnosis of jejunoileal atresia can usually be made by radiographic examination of the abdomen with only swallowed air as contrast.
Swallowed air reaches the proximal bowel by 1 hour and the distal small bowel by 3 hours in a normal vigorous infant in whom its passage is blocked, but this pattern may be delayed in premature or sick infants with poor sucking. Jejunal atresia patients have a few gas-filled and fluidfilled loops of small bowel, but the remainder of the abdomen is gasless (See figure below). When the atresia is associated with cystic fibrosis, fewer air-fluid levels are evident, and the typical ground-glass appearance of inspissated meconium is present. A limited-contrast meal may be useful if intestinal stenosis is suspected.

Diagnosis

Figure. A, The abdominal radiograph in this neonate shows several proximally dilated intestinal loops consistent with jejunal atresia. B, A type III(a) distal atresia was found at operation.

Differential diagnosis

Diseases that mimic jejunoileal atresia include:

colonic atresia, midgut volvulus, meconium ileus, duplication cysts,

internal hernias,
ileus due to sepsis, birth trauma, maternal medications, prematurity, hypothyroidism.

Special investigations, including an upper gastrointestinal contrast study, contrast enema, rectal biopsy, and a F508 gene deletion assay or sweat test to exclude associated cystic fibrosis, may be needed.

Management

Delay in diagnosis may lead to impairment of intestinal viability (50%), frank necrosis and perforation (10% to 20%), fluid and electrolyte abnormalities, and sepsis. Preoperative management involves insertion of a nasogastric or orogastric tube to decompress the stomach and fluid resuscitation to correct electrolyte abnormalities and hypovolemia. Antibiotics should be initiated if there is any concern for perforation or infection.

Surgical considerations

The surgical management of intestinal atresias is based on the location of the lesion, anatomic findings, associated conditions noted at operation, and the length of the remaining intestine. Resection of the dilated and hypertrophied proximal bowel, with primary end-to-end anastomosis with or without tapering of the proximal bowel, is the most common surgical technique.

Surgical considerations

Figure. The operative approach through the umbilical ring allows for complete evaluation of the intestine and repair of an atresia. Shown here is the technique using the Lonestar retractor to maintain exposure during repair of a type IIIa jejunal atresia.

Surgical considerations

Figure. The intestinal anastomosis in the infant seen in previous figures is shown. At the time of repair, there can be a significant size discrepancy between the proximal and distal bowel. The proximal bowel has been resected to a point that will allow for a more appropriately sized intestinal anastomosis

Outcome

Functional outcome ultimately depends on the following factors:

(1) the location of the atresia (the ileum adapts to a greater degree than the jejunum), (2) the maturity of the intestine (the small intestine in a premature infant still has time for maturation and growth), and (3) the length of the small intestine, which can be difficult to determine accurately after birth. The ileocecal valve is critically important because it allows for more rapid intestinal adaptation when the residual small bowel length is short.

Colonic atresia

Epidemiology
Colonic atresia is a rare cause of intestinal obstruction and comprises 1.8% to 15% of all gastrointestinal atresias. The reported incidence of colonic atresia varies greatly from 1:5000 to 1:60,000 live births. The accepted incidence is approximately 1 in 20,000 live births. Although it is most commonly reported as an isolated anomaly, approximately one third of infants have associated congenital lesions.

Classification

Type I consists of mucosal atresia with an intact bowel wall and mesentery. In type II the atretic ends are separated by a fibrous cord. In type III the atretic ends are separated by a V-shaped mesenteric gap (See figure). In the ascending and transverse colon, type III colonic atresias predominate. Types I and II are seen more commonly distal to the splenic flexure. Type III lesions are the most commonly occurring lesions overall

Associated anomalies

The rate of associated anomalies with colonic atresias is much smaller when compared with other atresias. Colonic atresias have been found in approximately 2.5% of neonates with gastroschisis. There are fewer than 25 published cases of colonic atresia and Hirschsprungs disease. Complex urologic abnormalities, multiple small intestinal atresias, an unfixed mesentery, and skeletal anomalies have also been reported to occur with colonic atresia. Similar to small bowel atresias, a vascular insult to the fetal intestine continues to be the accepted cause for all types of colonic atresia.

Prenatal diagnosis

Prenatal diagnosis is of great importance in the management of colonic atresia. On prenatal ultrasonography, the colon has a relatively characteristic appearance. The presence of an obstruction can usually be determined when the diameter of the colon is larger than expected for gestational age.

Clinical features
The characteristic clinical features of colonic atresia are abdominal distention, bilious emesis, and failure to pass meconium. On plain radiographs, air-fluid levels are usually appreciated as well as dilated intestinal loops of large bowel often associated with a groundglass appearance of meconium mixed with air. Occasionally, the dilation can be so massive that it mimics pneumoperitoneum. The diagnosis is made with a contrast enema showing a small diameter distal colon that comes to an abrupt halt at the level of the obstruction (See figures below).

X-ray

A, Abdominal radiograph of colonic atresia showing huge air-filled proximal colon mimicking a pneumoperitoneum. B, Colonic atresia with rectal stenosis (arrow) is seen on this retrograde contrast study.

X-ray

The contrast enema in a patient with a distal intestinal obstruction shows a small colon and failure of the contrast agent to move proximally past the mid-transverse colon.

Surgical considerations

A staged surgical approach consisting of colostomy with mucous fistula is generally preferred.

Because the proximal and distal ends adjacent to the atresia are abnormal in both innervation and vascularity, resection of the bulbous proximal colon as well as a portion of the distal microcolon is suggested.
Primary resection with anastomosis has a higher incidence of complications, usually due to undiagnosed distal pathology.

Prognosis

In the absence of other serious co-morbidities, the prognosis in colonic atresia is excellent.

If diagnosed early, the overall mortality is less than 10%.

A delay in diagnosis beyond 72 hours, however, may result in a mortality of greater than 60%.

This high mortality is due, in part, to the formation of a closed loop obstruction between an intact ileocecal valve and the atresia, leading to massive colonic distention and perforation.