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Updates in: Bowel Obstruction and Malrotation

Francesca E Stedman, Michael P Stanton

Department of Paediatric Surgery, University Hospital Southampton NHS Foundation Trust, UK

 

Correspondance:

Michael P Stanton

Department of Paediatric Surgery

University Hospital Southampton NHS Foundation

Trust Tremona Road, Southampton, Hampshire, SO16 6YD, UK

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Congenital bowel obstruction affects around 1 in 2000 live births. Presentation can be varied depending upon the level of the obstruction and whether or not there was antenatal suspicion. Causes of a proximal obstruction include pyloric atresia, duodenal atresia, jejuno-ileal atresia, and malrotation. Causes of a distal obstruction include colonic atresia, small left colon syndrome and meconium disease. This review describes in detail the presenting features, aetiology, epidemiology and clinical management of these conditions.

Keywords: bowel obstruction, neonate, bilious vomit, atresia, meconium disease

 

Introduction

The incidence of congenital bowel obstruction is estimated at 1 in 2000 live births [1]. Neonatal bowel obstruction can be sub-divided into proximal and distal obstructions. Two important clinical signs of neonatal bowel obstruction are bilious vomiting (or bilious naso-gastric aspirates) and delayed passage of meconium. Bilious vomiting is more common in proximal obstruction, though in a very high obstruction, emesis may be non-bilious. With proximal obstruction, maternal polyhydramnios may also have been a feature. Abdominal distension will develop with distal obstruction, particularly if there is a delay in diagnosis. Causes of proximal obstruction include pyloric atresia, duodenal obstruction, jejuno-ileal atresia and malrotation. Distal obstruction may be due to ileal atresia, meconium disease, colonic atresia, and small left colon syndrome. Hirschsprung disease also presents as (functional) distal bowel obstruction, and is discussed in a separate article.

Proximal Obstruction

Pyloric Atresia

Pyloric atresia is a very rare congenital anomaly, with an incidence of around 1 in 100,000 live births[2]. Maternal polyhydramnios along with an antenatal ultrasound demonstrating a single bubble are suggestive. Postnatally, non-bilious vomiting and a radiograph showing a single gas bubble, representing the distended stomach, are key features. There are three types of pyloric atresia: type I, presence of a pyloric membrane; type II, complete obstruction of the lumen of the pylorus with a solid core of tissue; and type III, absence of the pylorus resulting in loss of bowel continuity, with a fibrous band remaining. Type I and II can be dealt with by surgical excision of the obstructing tissue, along with a pyloroplasty. Type III requires gastro-jejunostomy. Based on the pattern of familial cases, Bar-Maor et al postulated in 1972 that the anomaly is transmitted as an autosomal recessive condition. Epidermolysis bullosa is a well-recognised association which has a significant impact on outcome, but for isolated cases (50% of cases) the outcome is usually good.

Congenital Duodenal Obstruction

Congenital duodenal obstruction occurs in 1 in 5000 – 10,000 live births[1]. Duodenal atresia represents over 60% of intestinal atresia. Intrinsic congenital duodenal obstruction may occur as a result of an atresia, stenosis or web. Such anomalies are believed to occur as a result of failure of re-canalisation of the duodenal lumen by the 8th week of gestation. Extrinsic duodenal obstruction may occur as a result of anatomical abnormalities such as an annular pancreas (pancreatic tissue surrounding the duodenum), pre-duodenal portal vein, or malrotation.

Duodenal atresia is diagnosed antenatally in 50% of cases. The main features are polyhydramnios, due to reduced reabsorption of swallowed amniotic fluid, and a ‘double bubble’, which may be seen in the third trimester. Other associated anomalies will be present in more than half of patients [1]. The majority of associated anomalies are cardiac (30% of patients), with atrial septal defect being the commonest problem [3]. Around one-third of patients have trisomy 21. Keckler et al reported a relative risk of 2.6 for a patient to require open heart surgery with duodenal atresia and trisomy 21, compared to a patient with duodenal atresia and normal karyotype [4]. Malrotation is seen in up to 40% of patients[5]. Rarely, a second distal atresia may be present and this should be investigated for at operation.

Postnatal presentation is with either bilious or non-bilious vomiting within the first 48 hours of life. Non-bilious vomiting occurs in 20% of patients, as a result of an atresia occurring proximal to the insertion of the common bile duct into the 2nd part of the duodenum. The classical X-ray demonstrating a double bubble appearance in the upper abdomen without distal gas is diagnostic (Fig. 1). Distal gas may be seen in patients with a stenosis or incomplete web. It may also occur due to an accessory pancreatic duct of Santorini, which inserts into the proximal duodenal segment allowing gas to pass to the distal duodenal segment. An X-ray with dilated stomach and duodenum and paucity of distal gas may also be seen in a patient with malrotation/volvulus. Any suspicion of this diagnosis should be immediately investigated and managed due to the risk of ischaemia/infarction of the bowel. An antenatal diagnosis is highly suggestive of duodenal atresia, and a smooth end (as opposed to ‘bird’s beak’) to the most distal dilated bowel on X-ray is also more suggestive of duodenal atresia than malrotation.

Figure 1. Plain abdominal radiograph demon - strating the ‘double bubble’ appearance sug - gestive of duodenal atresia. There is a rounded end to the proximal duodenal pouch and no distal gas.

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There are three main types of duodenal atresia: type I - membrane or web (approximately 80% of cases); type II - obliteration of a segment of duodenum with a fibrous cord between proximal and distal segments; and type III - complete separation of the proximal and distal segments. A web may distend distally as a ‘windsock’, giving the external appearance that the obstruction is more distal than it is. Advancement of a nasogastric tube intra-operatively will demonstrate the site of origin of the web [1] .

Provided there is no suspicion of malrotation, operative intervention for duodenal atresia is usually not an emergency. Management should begin with intravenous fluid resuscitation and nasogastric decompression. Investigations should be carried out as necessary to assess for associated anomalies, before proceeding to surgical intervention.

Surgery involves bypassing the atretic or stenotic segment of duodenum, by performing a duodeno-duodenostomy (incision proximal to the obstruction anastomosed to incision in the distal segment without excision of the atretic ends). Where this is not possible (for example due to obstruction of the fourth part of the duodenum) duodeno-jejunostomy may be performed, though this is associated with an increased risk of blind-loop syndrome. Resection of a duodenal web, with a duodenoplasty, is possible however there is a risk of injury to the bile duct, pancreatic duct or ampulla of Vater with this technique. Investigation for the presence of distal atresia should be performed, as well as assessment of the duodeno-jejunal flexure. If malrotation is present, a Ladd’s procedure should also be performed Laparoscopic repair is increasing in popularity with reported reduced time to feeding and reduced hospital stay. Peri-operative placement of a trans-anastomotic tube results in shorter time to first enteral feed and shorter time to full enteral feeds. Furthermore, it reduces the need for a central line and parenteral nutrition. Early complications of duodeno-duodenostomy include anastomotic leak or stenosis. Duodeno-gastric reflux, mega-duodenum and blind loop syndrome or adhesive bowel obstruction may occur later. Subsequent tapering duodenoplasty is sometimes necessary in the management of mega-duodenum. Long term survival approaches 90%, with mortality mainly attributed to associated anomalies and prematurity [1].

Jejuno-Ileal Atresia

Jejuno-ileal atresia affects 1 in 14000 live births in Europe, with less than 4% having an associated chromosomal or genetic syndrome [6]. In 1979, Grosfeld modified the classification system proposed by Louw and Barnard into the following types: type I, membrane obstruction; type II, atretic ends separated by a fibrous cord; type IIIa, atretic ends separated by a ‘V’ shaped mesenteric gap; type IIIb, ‘apple peel’ atresia; type IV, multiple atresia (Fig. 2 – 6). The site of the most proximal atresia determines its classification as jejunal or ileal, and those occurring in the jejunum are more common, with type IIIa being the most frequently seen variant. In type IIIb, the distal small bowel receives its blood supply from a single ileo-colic or right colic artery The incidence of premature birth is greater in this group of patients, resulting in increased morbidity and mortality [1].

Figure 2. Type I jejunal atresia; image adapted from Grosfeld JL et al. JPS 1979; 14:368-75 by Stephen Adams

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Figure 3. Type II jejunal atresia; image adapted from Grosfeld JL et al. JPS 1979; 14:368-75 by Stephen Adams

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Figure 4. Type IIIa jejunal atresia; image adapted from Grosfeld JL et al. JPS 1979; 14:368-75 by Stephen Adams

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Figure 5. Type IIIb jejunal atresia; image adapt - ed from Grosfeld JL et al. JPS 1979; 14:368-75 by Stephen Adams

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Figure 6. Type IV jejunal atresia; image adapted from Grosfeld JL et al. JPS 1979; 14:368-75 by Stephen Adams

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The most widely-held belief regarding aetiology is the vascular insult theory. In the 1950’s experiments on canine fetuses were performed by Louw and Barnard, demonstrating that ligation of a mesenteric vessel resulted in atresia, with the final outcome depending on the site and extent of the vascular interference [7]. The presence of epithelial cells, bile salts and lanugo hair in the distal atretic segments of human pathology specimens suggests the insult occurs much later in fetal development than the presumed failure of re-canalisation theory relating to duodenal atresia. This results in a much lower incidence of associated anomalies of around 15% in those patients without an abdominal wall defect. Such associated anomalies include concurrent duodenal or colonic atresia in up to 6% of patients, cardiac anomalies in 8% and renal tract anomalies in 4% [8]. A further fifteen percent of jejuno-ileal atresia were found in patients with gastroschisis, and 1.5% in patients with omphalocele. Very rarely there may be multiple atresias throughout the gastro-intestinal tract. This condition has an extremely high mortality rate [1]. Malrotation is found in 20% of patients with jejuno-ileal atresias; indeed, an antenatal volvulus may have been the causative factor for development of the atresia. Cystic fibrosis occurs in up to 17% of patients and therefore performing a sweat test or genetic testing on all patients with jejuno-ileal atresia should be considered, in particular type III or IV. Screening for common mutations of cystic fibrosis is routine in the United Kingdom at day 5 of life, and is approximately 98% sensitive.

Up to one-third of cases are suspected antenatally, with polyhydramnios and dilated bowel seen. Postnatally, bilious vomiting and abdominal distension occur, depending upon the level of the atresia. Passage of meconium is often delayed, and may consist of pale, mucousy plugs rather than pigmented meconium. Intravenous fluid resuscitation and nasogastric decompression is initiated. Abdominal x-ray will usually be diagnostic. The presence of calcification suggests an ante-natal perforation. If there are any signs suggestive of acute volvulus, which may occur as a result of a heavy, dilated proximal loop twisting, or due to malrotation, then operative intervention is urgent. A contrast enema may be performed as part of the investigative work-up where meconium ileus, Hirschsprungs disease or small left colon syndrome are amongst the differentials. At laparotomy, full exploration of the bowel is performed with assessment for associated malrotation. Catheterisation and irrigation of the distal bowel is performed to exclude atresias. Resection and end-to-end anastomosis is required for each atresia, though it is advised that no more than three anastomoses are performed. Preservation of bowel length is key. Resection of 10-15cm of the proximal atretic end is recommended to ensure that the anastomosis is performed in healthy bowel, as the dilated loop remains redundant without adequate peristalsis, resulting in a higher risk of post-operative complications [1,7]. A discrepancy in diameter of bowel of up to 7:1 is considered suitable for end-to-end anastomosis. Beyond this, tapering or ‘fish-mouthing’ may be required. Careful measuring and documentation of the remaining bowel length is essential as one of the risks is short bowel syndrome. Occasionally a temporary stoma is required, for example if there is perforation, or if the proximal end is extremely dilated and overall bowel length is an issue. Morbidity and mortality is largely related to prolonged intestinal dysmotility, short bowel syndrome and parenteral nutrition complications. Overall long term survival rate is approximately 90% [2,8].

Malrotation

Malrotation occurs in around 1 in 500 live births, with up to 80% of those presenting in the first month of life [9]. Anomalies of rotation and fixation of the midgut that result in a narrow based mesentery, predisposing to volvulus. Volvulus causes a proximal bowel obstruction as well as catastrophic bowel infarction if not treated urgently.

Malrotation occurs when the normal process of midgut rotation and fixation is arrested or anomalous. During the fifth week of gestation, rapid elongation of the midgut results in physiological herniation through the umbilical cord. Initially, a 90 degree anti-clockwise rotation around the superior mesenteric artery occurs, followed by a further 180 degree anti-clockwise rotation when the midgut returns to the peritoneal cavity at around week eight to ten of gestation. The duodenal-jejunal flexure thus is expected to lie in the left upper quadrant, and following the final stage of normal rotation, the caecum will be in a relatively fixed position in the right iliac fossa. Malrotation usually results in the duodenal-jejunal flexure lying to the right of the midline, the caecum being poorly fixed and often lying high, the presence of congenital adhesions between the caecum and the duodenum (Ladd’s bands), and a mesentery that is narrow. Ladd’s bands themselves may lead to duodenal obstruction, however the major concern is that the narrow based mesentery predisposes to volvulus of the entire midgut, resulting in ischaemia. Other abnormalities of rotation may occur in the context of abdominal wall defects and congenital diaphragmatic hernias; this is usually described as ‘non-rotation’. However, it is not the ultimate position of the duodenal-jejunal flexure and caecum that is of clinical significance, but the width of the mesentery and resultant propensity to volvulus. Patients with non-rotation do not usually have a narrow based mesentery and therefore are not considered to be at increased risk of volvulus. These patients will have surgical intervention for their underlying condition resulting in the formation of adhesions that may further reduce the risk of volvulus.

Malrotation occurs more commonly in patients with cardiac or genito-urinary anomalies as well as trisomy 21 [1,9], and may be the causative factor in jejuno-ileal atresia. Patients with heterotaxy may undergo screening for rotational abnormalities but an abnormal position of the duodenal-jejunal flexure does not necessarily imply a narrow based mesentery with increased risk of volvulus. Controversy exists regarding prophylactic elective surgery in asymptomatic patients, and thus the need for such screening. Malrotation usually presents when there is associated volvulus most commonly in the first month of life. This results in a proximal bowel obstruction with resultant bilious vomiting, Presentation beyond this age can be varied, with intermittent abdominal pain, nausea, vomiting (not necessarily bilious), or failure to thrive. In older children and adults, the diagnosis may be made at laparotomy for other indications, or as an ‘incidental’ finding on investigation for unrelated symptoms. In such cases, urgent (elective) surgical correction should be considered.

The gold standard for diagnosis of malrotation is an upper gastro-intestinal contrast study (Fig. 7). This is abnormal if the duodenal-jejunal flexure does not lie to the left of the left pedicle of L2, and is below, the level of the pylorus. Furthermore, evidence of volvulus may be seen with ‘beaking’ or corkscrew appearance of the mid-duodenum. In experienced hands, further evidence can be identified on ultrasound. The normal configuration is the superior mesenteric artery lying to the left of the superior mesenteric vein, though this relationship can vary. A ‘whirlpool’ sonographic appearance of the vessels suggests volvulus and immediate surgical exploration should occur following this finding. A plain abdominal x-ray may appear grossly normal, but may show a dilated stomach and proximal duodenum, with paucity of distal gas. A ‘gasless’ x-ray in a clinically unwell infant, or in one with peritonism, should lead to lead to immediate surgical intervention without waiting for an upper gastro-intestinal contrast study due to the risk of ischaemia and infarction of the entire midgut.

Figure 7. Radiograph from an upper GI contrast examination demonstrating classical appearances of malrotation with volvulus of the midgut. The duodeno-jejunal flexure lies to the right and there is a corkscrew appearance beyond

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Ladd’s procedure remains the operation of choice. The initial step is to de-rotate the bowel in an anti-clockwise position if volvulus is present. If any Ladd’s bands exist, these should be divided along with lateral mobilisation of the right colon and then duodenum, followed by widening of the base of the mesentery. The colon is then placed on the left with the small bowel on the right. Finally, inversion appendicectomy is usually performed to avoid future diagnostic confusion. Bowel ischaemia may be evident at the time of laparotomy, and midgut resection would commit the infant to short gut syndrome, the need for long-term parenteral nutrition and intestinal transplantation. Any question of viability should be managed with a re-look laparotomy at 24-48 hours. In cases of total midgut loss, full discussion with the family and multi-disciplinary team are essential to allow an informed decision to be made. Laparoscopy may be a useful tool for the diagnosis of malrotation with or without volvulus, however extensive mobilisation and bowel handling occurs in the open procedure which may result in the development of ‘beneficial’ adhesions that help to prevent recurrence of volvulus. As fewer abdominal adhesions may develop following a laparoscopic procedure, there is concern that this could result in a higher rate of recurrent volvulus. A recurrent volvulus rate of 20% was seen in one laparoscopic series [1,5].

Complications of Ladd’s procedure include adhesive small bowel obstruction (6%), incisional hernia, perforation and recurrent volvulus (around 1% each). Malrotation still accounts for a significant proportion of children with short-gut syndrome. Mortality depends on whether bowel ischaemia is present and is approximately 3% [6].

Distal Obstruction

Colonic Atresia

Colonic atresia affects approximately 1 in 20,000 live births [1,5] and comprises around 8% of intestinal atresias [1,8]. About one third of babies with colonic atresia are born prematurely, and there is a slight male preponderance (4:3) [3]. The vascular insult theory thought to be responsible for jejuno-ileal atresias is also believed to be implicated in colonic atresia. Grosfeld’s classification of atresias also applies to those affecting the colon. Type III atresia proximal to the splenic flexure is the most common variant, with type I and II atresias occurring less frequently and are predominantly located in the descending or sigmoid colon.

Associated anomalies are rare, although up to 20% of colonic atresias occur in patients with gastroschisis [8,10]; conversely around 2.5% of patients with gastroschisis have a colonic atresia. Fewer than 25 cases of Hirschsprungs disease associated with colonic atresia have been reported but suction rectal biopsy should be considered where primary anastomosis is being performed [1,5].

Reports of antenatal definitive diagnosis are extremely rare. Postnatal clinical signs include bilious vomiting, abdominal distension and delayed passage of meconium. The extremely dilated proximal colon may be palpable. Plain abdominal x-ray may be similar to that seen with a distal intestinal atresia. Contrast enema demonstrating a blind ending micro-colon is highly suggestive of colonic atresia. Diagnosis is frequently delayed, and due to the closed loop obstruction formed by a competent ileo-caecal valve, perforation occurs in around 10% of cases [1]. Management begins with intra-venous fluid resuscitation and nasogastric decompression, but operative intervention should occur in a timely manner due to the risk of perforation.

Where the right side of the colon is involved, most surgeons would advocate performing a primary excision and anastomosis. For left sided colonic atresias, initial formation of a stoma with delayed anastomosis, may be preferred. Overall mortality rate is around 25%, though some series report zero mortality [8]. A review of the literature highlighted a significantly higher mortality rate in those cases who were operated on after 72 hours [1]. With prompt diagnosis, the lack of associated anomalies and adequate bowel length in isolated colonic atresia, the outcome should be excellent.

Small Left Colon

Syndrome Small left colon syndrome is a rare cause of distal intestinal obstruction in the neonate, in which the obstruction is functional and transient. The condition was first reported by Davis in 1974[6,11] and the aetiology is unknown. Over 50% of patients with this condition are born to diabetic mothers, and in one study it was shown to be the most frequent cause of bowel obstruction in infants of diabetic mothers [1]. The rest of the affected patients are often either hypoglycaemic, septic or hypothyroid.

Clinical signs at presentation are those of distal intestinal obstruction - vomiting (usually bilious), abdominal distension and delayed passage of meconium. Plain abdominal x-ray shows distal bowel obstruction, and contrast studies show a pathognomonic, narrow, often shortened, left colon with a transition point at the splenic flexure [7,11]. Contrast enema is frequently therapeutic as well as diagnostic, and although plugs of meconium may be passed in such patients, they are thought to occur as a result of the functional obstruction rather than to be the cause of it [11,12].

Suction rectal biopsy may be required to exclude Hirschsprungs disease. Operative intervention should not be necessary and recovery is apparently full, without any further gastro-intestinal issues on long term follow-up. Of interest, further work by Davis revealed that 50% of asymptomatic infants born to diabetic mothers who had a contrast enema, showed the same characteristic narrowing of the left colon [8,13].

Meconium Disease

Meconium disease comprises meconium plug syndrome, meconium ileus (complicated and uncomplicated), and meconium obstruction of prematurity All present clinically as distal intestinal obstruction with vomiting (usually bilious), abdominal distension and delayed passage of meconium.

Meconium Plug Syndrome

Meconium plug syndrome was first described by Clatworthy in 1956 and affects 1 in 500 live births [1]. The aetiology is unknown. Meconium plug syndrome is characterised by distal bowel obstruction relieved by the passage of meconium plugs. Contrast enema may be required and can frequently be therapeutic as well as diagnostic, demonstrating filling defects in the left colon. Further enemas with water-soluble contrast, gastrograffin or saline can be performed until the meconium plug is passed, which usually alleviates the symptoms. Following this, the neonate should stool spontaneously. Some reports quote that Hirschsprungs disease may be present in up to 13% of patients [1], however others would argue that if Hirschsprungs disease was confirmed on histology, then that was the cause of the distal intestinal obstruction, rather than the plug itself. In one series, one third of the patients diagnosed with Hirschsprungs disease in a 6 year period presented with a distal intestinal obstruction and passage of a meconium plug relieved their obstruction [14]. Another association is cystic fibrosis, seen in up to 25% of cases. Most centres would advocate investigating for CF in all patients with meconium plug syndrome. Indeed, due to the considerable overlap some authors suggest that the terms ‘small left colon syndrome’ and ‘meconium plug syndrome’ be abandoned altogether [14].

Meconium Ileus

Meconium ileus may account for up to 30 percent of neonatal intestinal obstructions with an incidence of 1 in 3000 live births [1]. There is a very strong association with CF - at least 95% of patients with meconium ileus have CF, and 15% of patients with CF present with meconium ileus [1]. Meconium ileus is the only form of intestinal obstruction that presents with distension at birth. Simple meconium ileus (50% of cases) causes a functional obstruction as viscid, inspissated meconium adheres to the bowel wall. Babies presenting with this are usually relatively well, with classical features of distal bowel obstruction. In addition the ‘putty sign’ may be present (indentation on the abdominal wall after palpation) [15]. On gastrograffin enema, micro-colon is seen and contrast may be seen to outline inspissated pellets of meconium. Gastrograffin enema may also be therapeutic, although multiple enemas for example with 2-4% N-acetylcysteine irrigations, may also be needed. Success rates are between 16 and 50% and should be achieved within 48 hours, with contrast entering dilated (gas-filled) proximal loops of bowel. If this does not occur, laparotomy is required, with enterotomy to wash out the inspissated stool with N-acetylcysteine. Resection or stoma formation may be required for simple meconium ileus.

Complicated meconium ileus (50%) occurs in association with volvulus, atresia or perforation and necessitates laparotomy. A plain abdominal x-ray may reveal calcification, free air or very large air-fluid levels. There are four types of meconium peritonitis: meconium ascites; meconium adhesive peritonitis; meconium pseudocyst; and infected meconium pseudocyst [1]. Meconium ascites may result from perforation that occurs days before delivery, and the peritoneal cavity fills with meconium. Adhesive meconium peritonitis is thought to occur due to perforation days to weeks before delivery, and dense adhesions form making operation technically challenging. A meconium pseudocyst may be the result of a perforation occurring weeks to months before delivery. Meconium accumulates in the peritoneal cavity over time with formation of a fibrous wall around it, and surrounding bowel loops are pushed to the periphery. Laparotomy is necessary but can be extremely difficult; after decompression of the pseudocyst it is usually necessary to fashion a proximal stoma and perform further definitive surgery at a later date. Survival for complicated meconium ileus is around 80 – 90%, and up to 100% for simple meconium ileus.

Meconium obstruction of prematurity

Meconium obstruction of prematurity is a term coined by Siddiqui et al [16] to describe the entity of intestinal obstruction due to luminal contents in very low or extremely low birth weight babies. It has been termed meconium ileus of prematurity in the past. Delayed passage of meconium occurs in up to one third of premature babies, and this can make diagnosis of meconium obstruction of prematurity difficult, particularly as such babies may suffer also from distension and bilious aspirates for other reasons (dysmotility, sepsis). As with meconium plug syndrome or meconium ileus, cautious contrast enema with gastrograffin may be therapeutic as well as diagnostic.

Key Guidelines

• Bilious vomiting in an infant is abnormal and warrants immediate further investigation

• Malrotation should always be considered high on the list of differentials as missing this could have disastrous consequences

• Pyloric atresia can be associated with epidermolysis bullosa, which will have a major impact on operative management

• Duodenal atresia is strongly associated with trisomy 21, and it’s associated cardiac abnormalities

• Jejuno-ileal atresias are associated with cystic fibrosis in up to 20% of cases

• There is an association between colonic atresia and Hirschsprungs disease and therefore some clinicians would advocate rectal biopsy in all cases



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