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The child with rectal bleeding
Alimentary
Mini-symposium
The child with rectal bleeding
R. R. Turnock Systemic features
Although the causes of rectal bleeding in the paediatric age group are legion, a few relatively common diagnoses account for the majority, and in contrast to the adult situation, rectal bleeding presaging malignancy is rare. ’ The first step in the management of rectal bleeding is to recognize that most causes are broadly age related, hence the child should be considered in one of the following age groups;2 Newborn, up to 1 month, (i) (ii) Infant, from 1 month to 1 year, (iii) Child, from 1 to 12 years, (iv) Adolescent. A sound history and examination may often be diagnostic, although special attention to the following factors is especially useful.
Rectal bleeding may be but one manifestation of more widespread systemic disease. When rectal bleeding is sufficiently brisk to warrant transfusion, the presence or absence of an upper GIT source must be determined by passing a nasogastric tube and aspirating the stomach contents for evidence of blood ~ recording the vital signs in such cases is obviously essential. Pyrexia and other signs of infection should be noted, including vomiting and diarrohea. The entire child must be examined, with special attention to the abdomen and perineum. A rectal examination is usually unnecessary.
Pain The presence or absence of pain associated with the bleeding must be noted, especially in relation to defaecation. Localisation is vital; for instance, if there is ano-rectal pain, is it occurring as an isolated phenomenon, or is there associated abdominal pain, and if so, what is its nature. More widespread pain, for instance in the joints, may be a clue to systemic illness. At this juncture, many clues will have been furnished, and the diagnosis may be readily apparent. Tables l-4 list the diagnoses within each age group broadly based on the site of origin of the bleeding. It is clear that several disorders occur in more than one age group, and it would be sensible to discuss these commoner conditions first, followed by the rarer and more age specific causes of rectal bleeding.
Severity and nature of the bleeding Rectal bleeding is rarely life-threatening, but on occasions may require transfusion. Although bleeding is usually light, an estimation of the amount, the number of bleeding episodes and their timing, should all be made. The nature of the bleeding must be determined, with particular attention being paid to its appearance, colour and timing in relation to defaecation, and whether the blood merely streaks the outside of the stool, or is mixed in with it, and also the presence or absence of mucous. Bleeding from the upper gastro-intestinal tract (GIT) may first present per rectum, and in this context the dividing point between the upper and lower GIT is the ligament of Treitz; melaena may result from bleeding on either side of the ligament, whilst blood that is bright red or dark maroon has in almost all cases arisen from the lower GIT.2
Fissure in ano Fissure in ano occurs at any age and is characterised by the streaking of small amounts of bright red blood on the surface of the stool. Even in the newborn infant, pain on defaecation can be frequently
R. ‘lbnwk MRChR FRCS (Paed), Consultant Paediatric Surgeon, Department of Paediatric Surgery, Royal Liverpool Children’s Hospital, Alder Hey, Liverpool L12 2AP. Correspondence and requests for offprints to RRT. Richard
Current Puedlnrrrc.~ (1994) 4, 97-101 Q 1994 Longman Group Ltd
97
98
CURRENT
PAEDIATRICS
Table
1
of rectal
Causes
bleeding
in newborns
1. Local Anal fissure 2. Intestinal Necrotising enterocolitis Infectious gastro-enteritis Milk intolerance volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vascuIitides 4. Upper GIT Gastritis
Table
2
Causes
of rectal
bleeding
in infants
1. Local Anal fissure 2. Intestinal lntussusception Infectious gastro-enteritis Meckel’s diverticulum Milk intolerance/Food allergy volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vasculitides 4. Upper GIT Gastritis/peptic ulceration
Table
3
Causes
of rectal
bleeding
in children
1. Local Anal fissure 2. Intestinal Polyps lntussusception (~3-4 years) Infectious gastro-enteritis Meckel’ diverticulum Inflammatory bowel disease volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vasculitides 4. Upper GIT Gastritis/peptic ulceration Oesophageal varices
Table
4
Causes
of rectal
bleeding
in adolescents
1. Local Anal fissure 2. Intestinal Polyps Inflammatory bowel disease Infectious gastro-enteritis Meckel’ diverticulum volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vasculitides 4. Upper GIT Gastritis/peptic ulceration Oesophageal varices
ascertained, and is often the overriding feature in older children. Bleeding only occurs with defaecation and although the infant or child is generally well, the episode may be preceded by a pyrexial illness with mild dehydration and passage of a harder than normal stool resulting in the fissure. In many cases a
vicious circle sets up with further faecal retention occuring in anticipation of pain and the added complication of chronic constipation. The diagnosis is clinched on close inspection of the anal verge when the characteristic crack in the mucosa is seen, usually posteriorly. When the fissure is of a more chronic nature, a heaped up ‘sentinel’ skin tag may be seen, which if present in the older child may raise the suspicion of Crohn’s disease (see Inflammatory Bowel Disease below). Treatment is initially medical, with attention to dietary fibre intake and the use of stool softeners such as lactulose, which can be used judiciously even in small infants. In the older infant, a regular bowel habit should be re-established by a dose of senna syrup at night, and the application of small amounts of a local anaesthetic ointment to the area of the fissure will alleviate pain especially if applied half an hour prior to the anticipated time of defaecation (although it is accepted that this is easier said than done!). Fissures which are refractory to this regimen often benefit from an anal stretch under general anaesthesia. Both modes of treatment appear to have a success rate of around 50%, and a judicious combination of medical and surgical treatment probably yields the best results. ‘s2
Infectious gastroenteritis Infectious diarrohea is a common condition in all age groups. The child usually has colicky abdominal pain, premonitory vomiting and a contact history. There is often a fever, an ileus with abdominal distension but no clinical or radiological evidence of obstruction. Profuse bloody diarrohea with mucous is the key feature, the diagnosis being confirmed microbiologically. Stool should be sent for microscopy, culture and sensitivity, for ova and parasites, and electron microscopy. The common infective agents are Salmonella spp., Shigella spp., Giardia, Campylobacter, toxigenic Clostridia spp. and E. coli, and round viruses.1*2 Polyps By far the commonest type of polyps are juvenile polyps which, apart from fissure in ano, are most frequent cause of rectal bleeding in the older infant and school age child. They are benign, usually single, and up to a third occur in the last few centimetres of the rectum, where they may be detected on rectal examination by the experienced examiner. The history is of bright red bleeding, in most cases only occurring with the passage of stool, within which the blood is frequently mixed. Defaecation is painless. The initial investigation is a sigmoidoscopy under general anaesthesia, with endoscopic removal of any polyps seen using a wire snare. If undetected by this examination, and bleeding continues, colonoscopy should be the next investigation of choice, but must be performed
THE CHILD WITH RECTAL BLEEDING
by an experienced operator. A double contrast enema may be useful in the older child. Histology is essential, especially if the polyps are multiple or recurrent. Whilst juvenile polyps may be multiple, as long as they remain hamartomatous, no further action is needed and persistence beyond adolescence is very rare. Conversely, adenomatous polyps even if single, must be regarded as possessing premalignant potential. When such polyps are multiple (familial polyposis coli and Gardener’s syndrome) adenocarcinomatous change is inevitable and total colectomy is essential before the third decade. In all adenomatous lesions life-long surveillance is essential. One further condition seen in childhood which resembles polpyosis is lymphoid hyperplasia, as the mucosa becomes nodular in appearance, due to overexuberant areas of gut-associated lymph tissue (the colonic equivalent of Peyer’s patches). Histology is diagnostic, and no action is required.‘*2
Intussusception Intussusception virtually always occurs between the ages of 3 months and 3 years, with most in the first 12 months. The typical history is one of an acute onset of severe colicky abdominal pain with updrawing of the legs, frequently associated with vomiting which may become bilious; in between the attacks, the infant becomes quiet and floppy, frequently being described by the mother as ‘lifeless’. Passage of blood and mucous per rectum, the aptly named ‘red currant jelly stool’, is pathognomnic, but only occurs in one third of cases. Abdominal tenderness with guarding in the upper right quadrant commonly overlies a palpable mass, the presence of which may be confirmed on a straight abdominal X-ray together with absence of the caecal gas shadow. Contrast enema using barium or air is diagnostic and combined with controlled pressure may often achieve reduction. Surgical reduction is usually straightforward, although occasionally a limited right hemicolectomy is required. Intussusception can recur, and many paediatric surgeons consider that in this situation a pathological lead point must be excluded, as it should if the intussusception is not of the common ileocaecal variety.
Milk intolerance/food allergy Milk intolerance is usually to cow’s milk based formulae, but can also occur in infants who have been breast fed, and to other food stuffs. Presentation is before 2 years of age, with a chronic history of diarrohea with blood and mucous, and often a history of atopy in the child or family. On sigmoidoscopy, the mucosa has a patchy granular appearance with an infiltrate of eosinophils, but few other chronic inflammatory cells, being demonstrated on biopsy.
Staged food substitution supervision is curative.3
99
under specialist paediatric
Inflammatory Bowel Disease Both ulcerative colitis (UC) and Crohn’s disease (CD) are rare before school age, the average age at diagnosis being around g-10 years. Common clinical features include abdominal pain, diarrohea with blood and mucous in 40% (CD) to 90% (UC) of patients, weight loss and anaemia. Perianal and oral manifestations are commoner in CD, whilst joint pain and hepato-splenomegaly feature in UC. The essential first investigation is colonoscopy and biopsy. Often the experienced endoscopist can differentiate between the two diseases by the appearance of the mucosa, whilst in most cases histological examination is diagnostic, although there is some overlapping of features. A nodular mucosa without ulceration or contact bleeding suggests lymphoid hyperplasia (see Polyps above). Contrast studies are essential in delineating the extent of the disease, especially in CD. Treatment is dictated by the severity of the disease. Short courses of steroids may be needed to control acute excacerbations, but long term use does not affect relapse rate unless used in unacceptably high doses. The mainstay of treatment is sulphasalazine, or derivatives such as mesalazine. The route of administration of both steroids and aminosalicylates is dictated largely by the anatomical distribution of affected bowel, but may be oral (including slowrelease preparations), or by suppository/enema. Both elemental and total parenteral nutrition are of benefit in resistant CD, but are probably of little use in UC. A significant proportion of patients will come to surgery, which can be life-saving when there is a fuhninant toxic megacolon. Quoted figures for surgery in the 2 years after first diagnosis are 57% in CD and 26% in UC. Mortality in childhood is 4%.46
Meckel’s diverticulum Bleeding from a Meckel’s diverticulum is the commonest cause of substantial rectal bleeding in infants and younger children, and not infrequently requires transfusion. The colour of the blood may range from bright red to melaena, although in the latter case there is usually a plum coloured tinge (see Upper GIT Bleeding). A 99”Tc scan is diagnostic in up to 90% of cases, but unfortunately a negative scan does not exclude a Meckel’s; in these cases when the bleeding is recurrent, or in whom the initial episode is torrential, a ‘blind’ laparotomy may be required. The favoured operation is resection of the Meckel’s diverticulum together with a few centimetres of ileum on either side, followed by a primary anastomosis. Wedge resection of the diverticulum is not recommended since the bleeding point is often on the mesenteric border of the bowel opposite the mouth
100
CURRENT
PAEDIATRICS
of the gastric mucosa lined diverticuhun, and may be left in situ unless a full resection is performed.
Necrotising Enterocolitis (NEC) NEC is a disease of neonates and premature infants, usually following a period of hypoxic stress which causes shut down of the splanchnic circulation in response to the ‘diving reflex’, with resultant bowel ischaemia. Gut flora invade the damaged mucosa producing further damage and infarction, and if gasforming will result in gas being produced within the bowel wall (pneumatosis intestinalis) which is often visible on X-ray. The baby presents with sepsis, ileus and rectal bleeding. Treatment is initially medical with broad spectrum antibiotics and complete rest of the bowel, nutrition being supported parenterally. Urgent paediatric surgical assessment is required for those infants in whom there is no improvement on medical treatment or there is evidence of intestinal perforation. In these circumstances, the smallest, illest infants pose a management dilemma. Whilst surgery may be life-saving, sometimes the sickest infants cannot even be guaranteed to survive transport to the operating theatre. As long as it is regarded only as a temporising measure, a peritoneal drain (the little finger of a surgeon’s glove is ideal!) can be placed in the right iliac fossa under local anaesthetic as a ward procedure on the SCBU. Often this will lead to an improvement in the infant’s condition, giving a window of opportunity for surgery. In the longer term, up to a fifth of surviving infants with NEC will develop a colonic stricture, re-presenting with a wide variety of symptoms including intestinal obstruction, episodes of sepsis, failure to thrive and rectal bleeding; any unexplained symptoms in a baby at risk should initially be investigated by a contrast enema.7
and steroids; and raised intracranial pressure. Endoscopy is confirmatory, with supportive measures including antacids, sucralfate, Hz antagonists and proton pump inhibitors such as omeprazole being the mainstay of treatment. Surgery is reserved for complications such as perforation or life threatening haemorrhage. Such stress related peptic ulcer disease can obviously affect older children and adolescents in exactly the same way, but more commonly this age group are affected by ‘adult-like’ peptic ulcer disease, indeed many have a strong family history. There is increasing awareness of the role of Heliobacter pylorii in the pathogenesis of peptic ulcer disease, but it can only be diagnosed histologically; thus gastroscopy with multiple mucosal biopsies from antrum and duodenum is essential for the assesment of ulcer like symptoms in this age group. Heliobacter is treated with triple therapy of bismuth sulphate (‘De-Nol’) together with amoxycillin and metronidazole for at least 6 weeks (although compliance can be a problem), with repeat endoscopy and biopsy prior to cessation of treatment. Otherwise an Hz antagonist is the treatment of choice in nonHeliobacter disease. Serum gastrin levels should be measured to exclude Zollinger-Ellison syndrome. Complicated ulcer disease is rare in this age group; bleeding should be managed conservatively, with surgery reserved for life threatening haemorrhage. Surgery is also necessary for perforation, but in both situations should be limited to the absolute minimum, for example oversewing of a bleeding vessel, or omental patching of a perforation; gastric resection and/or vagotomy should be avoided if at all possible, especially with the emergence of the new proton pump inhibitor drugs. The only remaining cause of significant upper GIT haemorrhage is portal hypertension, which should only be managed by paediatric gastroenterologists and surgeons with specialist experience and expertise.2
Upper GIT bleeding Upper GIT bleeding may be sufficiently brisk to present as dark red rectal bleeding, thus mimicking lower GIT bleeding such as from a Meckel’s diverticulum. A naso-gastric tube should be passed in all cases of profuse rectal bleeding especially when not bright red; blood in the gastric aspirate confirms that its source is cephalad to the duodenal-jejunal junction. Upper GIT bleeding in newborns is usually due to a non-specific gastritis related to the stress of birth trauma and rarely causes rectal bleeding but swallowed maternal blood at birth or from a cracked nipple at breast feeding should be excluded. It is treated medically with saline gastric washouts and Hz antagonists; it rarely requires intervention. Gastritis and peptic ulcer disease are seen in infants and younger children but usually as a consequence of other severe systemic problems such as sepsis, trauma, burns and cardiac surgery; chemotherapy
v01vulus Unrecognised mid gut volvulus remains a potentially fatal condition until such time as small bowel transplantation becomes a feasible procedure. The predisposing condition to volvulus is intestinal malrotation, in which the intestine fails to attain its normal fully rotated position when returning to the abdominal cavity from the amniotic sac near to the end of the first trimester. The base of the mid gut mesentery is foreshortened, leaving it unstable and at risk of twisting (volvulus), with occlusion of the superior mesenteric vessels and potential infarction of the entire mid gut. Volvulus can occur at any time from birth onwards, although it appears to be commonest in the first 10 days or so. The infants are profoundly ill, with sepsis, bilious gastric aspirates, a variable degree of abdominal distension, and often profuse bloody mucous stools. Straight abdominal X-ray showing a paucity of gas is suggestive, and a
THE CHILD WITH RECTAL BLEEDING
101
contrast meal using iso-osmolar water soluble contrast demonstrates the duodenal-jejunal flexure malpositioned to the right of the midline, with complete obstruction distally. After basic resuscitation, immediate laparotomy by a paediatric surgeon is indicated.8
bleeding, but also as a palpable mass, volvulus or obstruction. Diagnosis is confirmed on ultrasound, CT scan or at laparotomy or laparoscopy.g Vascular malformations are rare in childhood, but in common with colonic angiodysplasia of the elderly, can be very frustrating to diagnose. ‘J
Coagulation disorders,thrombocytopeniaand vasculitides
References
This mixed group includes primary diseases such as haemophilia, von Willebrand’s disease and idiopathic thrombocytopaenic purpura. Commoner are such secondary conditions as haemorrhagic disease of the newborn (nowadays rare due to routine administration of vitamin K at birth); disseminated intravascular coagulation (DIC) seen in severe sepsis and trauma; adverse drug reactions and the direct effect of salicylates and other non-steroidal antiinflammatories; myelodysplasia, leukaemia and aplastic anaemia, and the marrow depression following chemotherapy; and conditions where the clotting problems are part of a systemic disease, for example, haemolytic uraemic syndrome, Henoch-Schonlein purpura, and the connective tissue disorders.‘,’
Structural and vascular anomalies Intestinal duplication cysts are most important structural anomaly that can present not only with rectal
1. Raine PAM. Investigation of rectal bleeding. Arch Dis Child 1991; 66: 279-280. 2. Oldham KT, Lobe TE. Gastrointestinal haemorrhage in childhood. Pediatr Clin North Am 1985; 32: 1247-1263. 3. Hill SM, Milla PJ. Colitis caused by food allergy in infants. Arch Dis Child 1990; 65: 132-140. 4. Chong SKF, Walker-Smith JA. Ulcerative colitis in childhood. J Roy Sot Med 1984; 77 suppl3: 21-25. 5. Hanauer SB. Medical therapy of ulcerative colitis. Lancet 1993; 342 (ii): 412-417. 6. Raine PAM. BAPS collective review. Chronic inflammatory bowel disease. J Pediatr Surg 1984; 19: 18-23. 7. Lister J, Tam PKH. Neonatal necrotising enterocolitis. In: Lister J, Irving IM, eds. Neonatal Surgery 3rd edn. London; Butterworths, 1990; 485-498. 8. Lister J. Malrotation and volvulus of the intestine. In: Lister J, Irving IM, eds. Neonatal Surgery 3rd edn. London; Butterworths, 1990; 442452. 9. Lister J. Duplications of the alimentary tract. In: Lister J, Irving IM, eds. Neonatal Surgery 3rd edn. London; Butterworths, 1990; 474-484.
Mini-symposium
The child with rectal bleeding
R. R. Turnock Systemic features
Although the causes of rectal bleeding in the paediatric age group are legion, a few relatively common diagnoses account for the majority, and in contrast to the adult situation, rectal bleeding presaging malignancy is rare. ’ The first step in the management of rectal bleeding is to recognize that most causes are broadly age related, hence the child should be considered in one of the following age groups;2 Newborn, up to 1 month, (i) (ii) Infant, from 1 month to 1 year, (iii) Child, from 1 to 12 years, (iv) Adolescent. A sound history and examination may often be diagnostic, although special attention to the following factors is especially useful.
Rectal bleeding may be but one manifestation of more widespread systemic disease. When rectal bleeding is sufficiently brisk to warrant transfusion, the presence or absence of an upper GIT source must be determined by passing a nasogastric tube and aspirating the stomach contents for evidence of blood ~ recording the vital signs in such cases is obviously essential. Pyrexia and other signs of infection should be noted, including vomiting and diarrohea. The entire child must be examined, with special attention to the abdomen and perineum. A rectal examination is usually unnecessary.
Pain The presence or absence of pain associated with the bleeding must be noted, especially in relation to defaecation. Localisation is vital; for instance, if there is ano-rectal pain, is it occurring as an isolated phenomenon, or is there associated abdominal pain, and if so, what is its nature. More widespread pain, for instance in the joints, may be a clue to systemic illness. At this juncture, many clues will have been furnished, and the diagnosis may be readily apparent. Tables l-4 list the diagnoses within each age group broadly based on the site of origin of the bleeding. It is clear that several disorders occur in more than one age group, and it would be sensible to discuss these commoner conditions first, followed by the rarer and more age specific causes of rectal bleeding.
Severity and nature of the bleeding Rectal bleeding is rarely life-threatening, but on occasions may require transfusion. Although bleeding is usually light, an estimation of the amount, the number of bleeding episodes and their timing, should all be made. The nature of the bleeding must be determined, with particular attention being paid to its appearance, colour and timing in relation to defaecation, and whether the blood merely streaks the outside of the stool, or is mixed in with it, and also the presence or absence of mucous. Bleeding from the upper gastro-intestinal tract (GIT) may first present per rectum, and in this context the dividing point between the upper and lower GIT is the ligament of Treitz; melaena may result from bleeding on either side of the ligament, whilst blood that is bright red or dark maroon has in almost all cases arisen from the lower GIT.2
Fissure in ano Fissure in ano occurs at any age and is characterised by the streaking of small amounts of bright red blood on the surface of the stool. Even in the newborn infant, pain on defaecation can be frequently
R. ‘lbnwk MRChR FRCS (Paed), Consultant Paediatric Surgeon, Department of Paediatric Surgery, Royal Liverpool Children’s Hospital, Alder Hey, Liverpool L12 2AP. Correspondence and requests for offprints to RRT. Richard
Current Puedlnrrrc.~ (1994) 4, 97-101 Q 1994 Longman Group Ltd
97
98
CURRENT
PAEDIATRICS
Table
1
of rectal
Causes
bleeding
in newborns
1. Local Anal fissure 2. Intestinal Necrotising enterocolitis Infectious gastro-enteritis Milk intolerance volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vascuIitides 4. Upper GIT Gastritis
Table
2
Causes
of rectal
bleeding
in infants
1. Local Anal fissure 2. Intestinal lntussusception Infectious gastro-enteritis Meckel’s diverticulum Milk intolerance/Food allergy volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vasculitides 4. Upper GIT Gastritis/peptic ulceration
Table
3
Causes
of rectal
bleeding
in children
1. Local Anal fissure 2. Intestinal Polyps lntussusception (~3-4 years) Infectious gastro-enteritis Meckel’ diverticulum Inflammatory bowel disease volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vasculitides 4. Upper GIT Gastritis/peptic ulceration Oesophageal varices
Table
4
Causes
of rectal
bleeding
in adolescents
1. Local Anal fissure 2. Intestinal Polyps Inflammatory bowel disease Infectious gastro-enteritis Meckel’ diverticulum volvulus Structural and vascular anomalies 3. Systemic disorders 1” and 2” coagulopathy/thrombocytopenia/vasculitides 4. Upper GIT Gastritis/peptic ulceration Oesophageal varices
ascertained, and is often the overriding feature in older children. Bleeding only occurs with defaecation and although the infant or child is generally well, the episode may be preceded by a pyrexial illness with mild dehydration and passage of a harder than normal stool resulting in the fissure. In many cases a
vicious circle sets up with further faecal retention occuring in anticipation of pain and the added complication of chronic constipation. The diagnosis is clinched on close inspection of the anal verge when the characteristic crack in the mucosa is seen, usually posteriorly. When the fissure is of a more chronic nature, a heaped up ‘sentinel’ skin tag may be seen, which if present in the older child may raise the suspicion of Crohn’s disease (see Inflammatory Bowel Disease below). Treatment is initially medical, with attention to dietary fibre intake and the use of stool softeners such as lactulose, which can be used judiciously even in small infants. In the older infant, a regular bowel habit should be re-established by a dose of senna syrup at night, and the application of small amounts of a local anaesthetic ointment to the area of the fissure will alleviate pain especially if applied half an hour prior to the anticipated time of defaecation (although it is accepted that this is easier said than done!). Fissures which are refractory to this regimen often benefit from an anal stretch under general anaesthesia. Both modes of treatment appear to have a success rate of around 50%, and a judicious combination of medical and surgical treatment probably yields the best results. ‘s2
Infectious gastroenteritis Infectious diarrohea is a common condition in all age groups. The child usually has colicky abdominal pain, premonitory vomiting and a contact history. There is often a fever, an ileus with abdominal distension but no clinical or radiological evidence of obstruction. Profuse bloody diarrohea with mucous is the key feature, the diagnosis being confirmed microbiologically. Stool should be sent for microscopy, culture and sensitivity, for ova and parasites, and electron microscopy. The common infective agents are Salmonella spp., Shigella spp., Giardia, Campylobacter, toxigenic Clostridia spp. and E. coli, and round viruses.1*2 Polyps By far the commonest type of polyps are juvenile polyps which, apart from fissure in ano, are most frequent cause of rectal bleeding in the older infant and school age child. They are benign, usually single, and up to a third occur in the last few centimetres of the rectum, where they may be detected on rectal examination by the experienced examiner. The history is of bright red bleeding, in most cases only occurring with the passage of stool, within which the blood is frequently mixed. Defaecation is painless. The initial investigation is a sigmoidoscopy under general anaesthesia, with endoscopic removal of any polyps seen using a wire snare. If undetected by this examination, and bleeding continues, colonoscopy should be the next investigation of choice, but must be performed
THE CHILD WITH RECTAL BLEEDING
by an experienced operator. A double contrast enema may be useful in the older child. Histology is essential, especially if the polyps are multiple or recurrent. Whilst juvenile polyps may be multiple, as long as they remain hamartomatous, no further action is needed and persistence beyond adolescence is very rare. Conversely, adenomatous polyps even if single, must be regarded as possessing premalignant potential. When such polyps are multiple (familial polyposis coli and Gardener’s syndrome) adenocarcinomatous change is inevitable and total colectomy is essential before the third decade. In all adenomatous lesions life-long surveillance is essential. One further condition seen in childhood which resembles polpyosis is lymphoid hyperplasia, as the mucosa becomes nodular in appearance, due to overexuberant areas of gut-associated lymph tissue (the colonic equivalent of Peyer’s patches). Histology is diagnostic, and no action is required.‘*2
Intussusception Intussusception virtually always occurs between the ages of 3 months and 3 years, with most in the first 12 months. The typical history is one of an acute onset of severe colicky abdominal pain with updrawing of the legs, frequently associated with vomiting which may become bilious; in between the attacks, the infant becomes quiet and floppy, frequently being described by the mother as ‘lifeless’. Passage of blood and mucous per rectum, the aptly named ‘red currant jelly stool’, is pathognomnic, but only occurs in one third of cases. Abdominal tenderness with guarding in the upper right quadrant commonly overlies a palpable mass, the presence of which may be confirmed on a straight abdominal X-ray together with absence of the caecal gas shadow. Contrast enema using barium or air is diagnostic and combined with controlled pressure may often achieve reduction. Surgical reduction is usually straightforward, although occasionally a limited right hemicolectomy is required. Intussusception can recur, and many paediatric surgeons consider that in this situation a pathological lead point must be excluded, as it should if the intussusception is not of the common ileocaecal variety.
Milk intolerance/food allergy Milk intolerance is usually to cow’s milk based formulae, but can also occur in infants who have been breast fed, and to other food stuffs. Presentation is before 2 years of age, with a chronic history of diarrohea with blood and mucous, and often a history of atopy in the child or family. On sigmoidoscopy, the mucosa has a patchy granular appearance with an infiltrate of eosinophils, but few other chronic inflammatory cells, being demonstrated on biopsy.
Staged food substitution supervision is curative.3
99
under specialist paediatric
Inflammatory Bowel Disease Both ulcerative colitis (UC) and Crohn’s disease (CD) are rare before school age, the average age at diagnosis being around g-10 years. Common clinical features include abdominal pain, diarrohea with blood and mucous in 40% (CD) to 90% (UC) of patients, weight loss and anaemia. Perianal and oral manifestations are commoner in CD, whilst joint pain and hepato-splenomegaly feature in UC. The essential first investigation is colonoscopy and biopsy. Often the experienced endoscopist can differentiate between the two diseases by the appearance of the mucosa, whilst in most cases histological examination is diagnostic, although there is some overlapping of features. A nodular mucosa without ulceration or contact bleeding suggests lymphoid hyperplasia (see Polyps above). Contrast studies are essential in delineating the extent of the disease, especially in CD. Treatment is dictated by the severity of the disease. Short courses of steroids may be needed to control acute excacerbations, but long term use does not affect relapse rate unless used in unacceptably high doses. The mainstay of treatment is sulphasalazine, or derivatives such as mesalazine. The route of administration of both steroids and aminosalicylates is dictated largely by the anatomical distribution of affected bowel, but may be oral (including slowrelease preparations), or by suppository/enema. Both elemental and total parenteral nutrition are of benefit in resistant CD, but are probably of little use in UC. A significant proportion of patients will come to surgery, which can be life-saving when there is a fuhninant toxic megacolon. Quoted figures for surgery in the 2 years after first diagnosis are 57% in CD and 26% in UC. Mortality in childhood is 4%.46
Meckel’s diverticulum Bleeding from a Meckel’s diverticulum is the commonest cause of substantial rectal bleeding in infants and younger children, and not infrequently requires transfusion. The colour of the blood may range from bright red to melaena, although in the latter case there is usually a plum coloured tinge (see Upper GIT Bleeding). A 99”Tc scan is diagnostic in up to 90% of cases, but unfortunately a negative scan does not exclude a Meckel’s; in these cases when the bleeding is recurrent, or in whom the initial episode is torrential, a ‘blind’ laparotomy may be required. The favoured operation is resection of the Meckel’s diverticulum together with a few centimetres of ileum on either side, followed by a primary anastomosis. Wedge resection of the diverticulum is not recommended since the bleeding point is often on the mesenteric border of the bowel opposite the mouth
100
CURRENT
PAEDIATRICS
of the gastric mucosa lined diverticuhun, and may be left in situ unless a full resection is performed.
Necrotising Enterocolitis (NEC) NEC is a disease of neonates and premature infants, usually following a period of hypoxic stress which causes shut down of the splanchnic circulation in response to the ‘diving reflex’, with resultant bowel ischaemia. Gut flora invade the damaged mucosa producing further damage and infarction, and if gasforming will result in gas being produced within the bowel wall (pneumatosis intestinalis) which is often visible on X-ray. The baby presents with sepsis, ileus and rectal bleeding. Treatment is initially medical with broad spectrum antibiotics and complete rest of the bowel, nutrition being supported parenterally. Urgent paediatric surgical assessment is required for those infants in whom there is no improvement on medical treatment or there is evidence of intestinal perforation. In these circumstances, the smallest, illest infants pose a management dilemma. Whilst surgery may be life-saving, sometimes the sickest infants cannot even be guaranteed to survive transport to the operating theatre. As long as it is regarded only as a temporising measure, a peritoneal drain (the little finger of a surgeon’s glove is ideal!) can be placed in the right iliac fossa under local anaesthetic as a ward procedure on the SCBU. Often this will lead to an improvement in the infant’s condition, giving a window of opportunity for surgery. In the longer term, up to a fifth of surviving infants with NEC will develop a colonic stricture, re-presenting with a wide variety of symptoms including intestinal obstruction, episodes of sepsis, failure to thrive and rectal bleeding; any unexplained symptoms in a baby at risk should initially be investigated by a contrast enema.7
and steroids; and raised intracranial pressure. Endoscopy is confirmatory, with supportive measures including antacids, sucralfate, Hz antagonists and proton pump inhibitors such as omeprazole being the mainstay of treatment. Surgery is reserved for complications such as perforation or life threatening haemorrhage. Such stress related peptic ulcer disease can obviously affect older children and adolescents in exactly the same way, but more commonly this age group are affected by ‘adult-like’ peptic ulcer disease, indeed many have a strong family history. There is increasing awareness of the role of Heliobacter pylorii in the pathogenesis of peptic ulcer disease, but it can only be diagnosed histologically; thus gastroscopy with multiple mucosal biopsies from antrum and duodenum is essential for the assesment of ulcer like symptoms in this age group. Heliobacter is treated with triple therapy of bismuth sulphate (‘De-Nol’) together with amoxycillin and metronidazole for at least 6 weeks (although compliance can be a problem), with repeat endoscopy and biopsy prior to cessation of treatment. Otherwise an Hz antagonist is the treatment of choice in nonHeliobacter disease. Serum gastrin levels should be measured to exclude Zollinger-Ellison syndrome. Complicated ulcer disease is rare in this age group; bleeding should be managed conservatively, with surgery reserved for life threatening haemorrhage. Surgery is also necessary for perforation, but in both situations should be limited to the absolute minimum, for example oversewing of a bleeding vessel, or omental patching of a perforation; gastric resection and/or vagotomy should be avoided if at all possible, especially with the emergence of the new proton pump inhibitor drugs. The only remaining cause of significant upper GIT haemorrhage is portal hypertension, which should only be managed by paediatric gastroenterologists and surgeons with specialist experience and expertise.2
Upper GIT bleeding Upper GIT bleeding may be sufficiently brisk to present as dark red rectal bleeding, thus mimicking lower GIT bleeding such as from a Meckel’s diverticulum. A naso-gastric tube should be passed in all cases of profuse rectal bleeding especially when not bright red; blood in the gastric aspirate confirms that its source is cephalad to the duodenal-jejunal junction. Upper GIT bleeding in newborns is usually due to a non-specific gastritis related to the stress of birth trauma and rarely causes rectal bleeding but swallowed maternal blood at birth or from a cracked nipple at breast feeding should be excluded. It is treated medically with saline gastric washouts and Hz antagonists; it rarely requires intervention. Gastritis and peptic ulcer disease are seen in infants and younger children but usually as a consequence of other severe systemic problems such as sepsis, trauma, burns and cardiac surgery; chemotherapy
v01vulus Unrecognised mid gut volvulus remains a potentially fatal condition until such time as small bowel transplantation becomes a feasible procedure. The predisposing condition to volvulus is intestinal malrotation, in which the intestine fails to attain its normal fully rotated position when returning to the abdominal cavity from the amniotic sac near to the end of the first trimester. The base of the mid gut mesentery is foreshortened, leaving it unstable and at risk of twisting (volvulus), with occlusion of the superior mesenteric vessels and potential infarction of the entire mid gut. Volvulus can occur at any time from birth onwards, although it appears to be commonest in the first 10 days or so. The infants are profoundly ill, with sepsis, bilious gastric aspirates, a variable degree of abdominal distension, and often profuse bloody mucous stools. Straight abdominal X-ray showing a paucity of gas is suggestive, and a
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contrast meal using iso-osmolar water soluble contrast demonstrates the duodenal-jejunal flexure malpositioned to the right of the midline, with complete obstruction distally. After basic resuscitation, immediate laparotomy by a paediatric surgeon is indicated.8
bleeding, but also as a palpable mass, volvulus or obstruction. Diagnosis is confirmed on ultrasound, CT scan or at laparotomy or laparoscopy.g Vascular malformations are rare in childhood, but in common with colonic angiodysplasia of the elderly, can be very frustrating to diagnose. ‘J
Coagulation disorders,thrombocytopeniaand vasculitides
References
This mixed group includes primary diseases such as haemophilia, von Willebrand’s disease and idiopathic thrombocytopaenic purpura. Commoner are such secondary conditions as haemorrhagic disease of the newborn (nowadays rare due to routine administration of vitamin K at birth); disseminated intravascular coagulation (DIC) seen in severe sepsis and trauma; adverse drug reactions and the direct effect of salicylates and other non-steroidal antiinflammatories; myelodysplasia, leukaemia and aplastic anaemia, and the marrow depression following chemotherapy; and conditions where the clotting problems are part of a systemic disease, for example, haemolytic uraemic syndrome, Henoch-Schonlein purpura, and the connective tissue disorders.‘,’
Structural and vascular anomalies Intestinal duplication cysts are most important structural anomaly that can present not only with rectal
1. Raine PAM. Investigation of rectal bleeding. Arch Dis Child 1991; 66: 279-280. 2. Oldham KT, Lobe TE. Gastrointestinal haemorrhage in childhood. Pediatr Clin North Am 1985; 32: 1247-1263. 3. Hill SM, Milla PJ. Colitis caused by food allergy in infants. Arch Dis Child 1990; 65: 132-140. 4. Chong SKF, Walker-Smith JA. Ulcerative colitis in childhood. J Roy Sot Med 1984; 77 suppl3: 21-25. 5. Hanauer SB. Medical therapy of ulcerative colitis. Lancet 1993; 342 (ii): 412-417. 6. Raine PAM. BAPS collective review. Chronic inflammatory bowel disease. J Pediatr Surg 1984; 19: 18-23. 7. Lister J, Tam PKH. Neonatal necrotising enterocolitis. In: Lister J, Irving IM, eds. Neonatal Surgery 3rd edn. London; Butterworths, 1990; 485-498. 8. Lister J. Malrotation and volvulus of the intestine. In: Lister J, Irving IM, eds. Neonatal Surgery 3rd edn. London; Butterworths, 1990; 442452. 9. Lister J. Duplications of the alimentary tract. In: Lister J, Irving IM, eds. Neonatal Surgery 3rd edn. London; Butterworths, 1990; 474-484.