Paediatric surgery
From Primary Surgery
Surgery in children
There are two periods in paediatric surgery: (1) The surgery of the neonatal period, which is mostly concerned with those congenital malformations which need urgent treatment at birth. The few you can treat are described here, but you may feel that most of them are too difficult, and unrewarding, to come high in your list of priorities. (2) The surgery of the rest of childhood, most of which is more conveniently described in other chapters, particularly in those in Volume Two on trauma, which describes children's fractures (69.6) and burns (Chapter 58). Other important aspects of paediatric surgery are most kinds of sepsis, particularly abscesses (5.2), pyomyositis (7.1), and osteomyelitis (7.2); intestinal obstruction by Ascariasis (10.6) and intussusception (10.8); inguinal hernias (14.5), the urological problems of childhood (Chapter 23), poliomyelitis (Chapter 27), talipes (27.15), Burkitt's lymphoma (32.3) and other childhood tumours. Such surgical paediatric problems as do not more conveniently fit anywhere else are here. Many of them will probably have a low priority. If possible refer them.
The fluid requirements of children are described in Chapter 15 of Primary Anaesthesia and their anaesthetic requirements in Chapter 18. Very young children tolerate fluid and electrolyte loss badly: they don't have the normal postoperative sodium and water retention of adults, they cannot pass a concentrated urine, and they easily become dehydrated and hypoglycaemic.
PAEDIATRIC SURGERY
ANAESTHESIA. See A 18.1. If you want to use local anaesthesia on a neonate, sedate him with 1 mg of diazepam: (1) You can dilute 1 ml of 2°1o lignocaine to 20 ml, and use this as the maximum dose. (2) You can infiltrate the line of the incision with 0.5% procaine, or 0.25% lignocaine. The maximum dose is about 5 ml-see Fig. A 5- 1. Avoid adrenalin in the neonate.
General anaesthesia is better if your anaesthetist is good. Use Ayer's T-piece, awake intubation (A 13.6), followed by general anaesthesia and intramuscular suxamethonium, as in A 18.2.
If a neonate requires an urgent operation, operate at 24 hours, or as soon after wards as possible. Lung function is poor if you operate before 24 hours, when his lungs are not yet fully expanded. Weigh him and put up a drip. Give him vitamin K(,1) 1 mg, and start him on ampicillin 100 mg/kg/24 hours in 3 divided doses.
Make sure the theatre is warm. Place him on a well-padded cross made of two splints, and bandage his arms and legs to it. Cover the rest of his body except for his abdomen with cotton wool. Monitor and replace his blood loss with the greatest care (3-1). If HIV is common in your area, try to use blood from his father or mother. Weigh all blood-soaked swabs accurately, on a scale borrowed from the pharmacy if necessary. Replace blood ml for ml if more than 5 ml are lost. During the operation he will probably need 5 to 10 ml/kg/hour of 5% dextrose in Ringer's lactate.
INTRAVENOUS LINES. An infant requiring general anaesthesia should have a scalp vein drip, a cut-down (A 15.2), or a central venous line (A 19.2).
FLUIDS AND ELECTROLYTES. Replace all a child's initial fluid deficit (A 15.3) during the 3 to 6 hours that you are starving him, before you start to operate Prescribe his postoperative fluids yourself, as in Figure A 15-4. Don't leave this to the nurses, and don't exceed 100 ml/kg in 24 hours.
For major surgery, where possible, pass an indwelling catheter. This is not appropriate under the age of 3 years; so, for a boy, use about 50 cm of plastic Paul's tube of suitable size; strap a plastic bag around the perineum of a girl. A child should pass 1 ml/kg/hour, or: o to 1 year, 10 to 20 ml an hour. 1 to 4 years, 20 to 24 ml an hour. 4 to 7 years, 24 to 28 ml an hour. 7 to 10 years, 28 to 30 ml an hour. 10 to 12 years, 30 to 35 ml an hour. More than 12 years, 50 ml an hour.
POTASSIUM. If a child is not taking oral fluids by 24 hours, he needs a potassium supplement. Add 10 mmol to 500 ml of intravenous fluid (20 mmol/litre). Don't give him more than 10 mmol/hour or 3 mmol/kg/day. Or, use half-strength Darrow's solution, which contains 18 mmol/litre. Potassium replacement can be very dangerous in children, if it is handled incorrectly.
If he becomes drowsy post operatively, and his gut becomes silent, suspect ileus, and give him potassium.
NUTRITION. Interrupt feeding as little as you can. Don't starve a child for more than 4 hours before the operation, and get him feeding again as soon after wards as you can. Listen every 6 hours for the return of bowel sounds, and note whether he has passed faeces or flatus; these signs show that feeding can start. Bowel sounds alone are not so reliable in children, so some surgeons adjust the time of feeding to the nature of the operation, and decide if it is safe to feed a child by aspirating his stomach hourly, before each intake of feed.
Where possible, give him the fluid he needs as half-strength Darrow's solution in 5% dextrose (A 15-6). If he was not starved for more than 4 hours, and feeds are restarted soon, he is not going to be short of energy.
If you are able to give him 50% glucose through a central venous line (unlikely), use it to replace his energy deficit resulting from starvation. Reckon that, if he cannot feed orally for more than 3 days, he needs 1 to 2 g/kg/day. Test his urine and watch for glycosuria and an osmotic diuresis.
Alternatively, dilute 25 ml of 50% dextrose in 500 ml of half strength Darrow's solution and increase the concentration gradually.
In working out the energy content of various fluids, use Figure 15-6 in Primary Anaesthesia, or remember that a litre of 10% dextrose contains 1700 kJ (400 cal). A child's daily postoperative energy needs are-
A newborn child needs 190 to 210 kJ/kg (45 to 50 cal/kg). From 3 to 10 kg he needs 250 to 335 kJ/kg (60 to 80 cal/kg). From 10 to 15 kg he needs 190 to 270 kJ/kg (45 to 65 cal/kg). From 25 to 35 kg he needs 145 to 190 kJ/kg (35 to 45 cal/kg). From 35 to 60 kg he needs 125 to 145 kJ/kg (30 to 35 cal/kg).
CAUTION! If he becomes drowsy, or unconscious or behaves strangely, suspect hypoglycaemia, or less commonly, water intoxication from lack of electrolytes relative to water.
If you are operating proximal to the upper jejunum, a jejunostomy at the time of the operation is a good way to feed him. See also 28.3.
Intestinal obstruction in the first few days of life
When a newborn baby vomits repeatedly he may have: (1) Some severe infection, such as septicaemia, originating in an infection of his umbilicus. (2) Intestinal obstruction, due to the conditions described in this chapter. (3) Meningitis. (4) Intracranial haemorrhage. The first three of these are often readily treatable if you diagnose them early.
Most babies vomit a few times during the first few days of life, but bile-stained vomiting soon after birth almost always indicates gut obstruction. A child's gut can be obstructed at the level of his oesophagus, his duodenum, his small gut, or, rarely,in his colon; but it is most often often obstructed at his anus.Anorectal malformations form a separate group, and present as the failure to pass meconium, combined with abdominal distension, rather than vomiting; so they are described separately in Section 28.4. Here we are concerned with obstruction higher up a child's gut.
Congenital obstruction of a child's small gut presents as bilious vomiting shortly after birth, and often (but not always) the failure to pass meconium. If the obstruction is proximal to the middle of his small gut, his abdomen does not distend, but if the obstruction is below this point, it does. The distension may be localized or generalized.
An obstructed gut is an emergency. Electrolyte loss and energy lack affect a baby sooner than an adult, so he needs urgent treatment,usually a laparotomy, within a few hours. If you are a careful, nimble operator, you may be able to save a few of these children with some of the most technically demanding methods described in this manual.
Hypertrophic pyloric stenosis presents later, as a previously healthy baby 2 to 3 weeks old who starts to vomit, and does not have diarrhoea. This form of obstruction is readily treatable and is described in Section 28.4. In Africa it is about as common as duodenal atresia.
NEONATAL GUT OBSTRUCTION
EXAMINATION. Assess the distension of the child's abdomen, look for visible peristalsis. Feel for the mass of a hypertrophic pylorus. Examine for visible and palpable coils of terminal ileum, that feel as if they might be filled with inspissated meconium (meconium ileus, not described here).
CAUTION! Neither the passage of meconium during the first 3 days, nor the absence of distension, excludes obstruction.
X-RAYS. After 12 hours he will have swallowed enough air to assist diagnosis. If possible, take erect straight films before you start aspirating his stomach.
Don't use contrast media introduced from above, except as described in oesophageal atresia. It is particularly dangerous in obstructions lower than the mid gut, and may make them worse.
CAUTION! (1) The neonatal jejunum, ileum, and colon all have the same smooth outline, and normally contain a few fluid levels. (2) Don't use contrast media.
THE DIFFERENTIAL DIAGNOSIS OF NEONATAL GUT OBSTRUCTION Suggesting septicaemia-a site of origin for the infection, such as an infected umbilicus; he is more ill than you would expect from obstruction alone; a positive blood culture.
Suggesting raised intracranial pressure -signs of cerebral irritation, a swollen fontanelle (enlargement of the head and papilloedema are late signs).
Suggesting some other cause of abdominal distension a part of the abdomen which is dull to percussion. Causes include distension of the bladder in urethral Obstruction, tumors, ascites, and congenital cystic kidneys, etc.
GENERAL MANAGEMENT. As soon as you suspect the diagnosis, pass a nasogastric tube, strap it to his face, see that it is aspirated at least every 30 minutes, and let it decompress into a bag-aspiration is a common cause of death. Put up a drip. Keep him warm. If possible refer him-urgently. If not, consider operating as soon as possible (28.3).
SPECIFIC CONDITIONS OBSTRUCTING THE NEONATAL GUT
OESOPHAGEAL ATRESIA is often associated with a tracheooesophageal fistula. The proximal oesophageal pouch fills with saliva, so that he dribbles excessively. If he is given milk or water, he is likely to aspirate it into his trachea. He froths, coughs, and becomes cyanotic. If oesophageal atresia is common in your area, pass a nasogastric tube on all neonates. It has a higher incidence" in underweight babies.
Confirm the diagnosis by passing a small (8 Ch in a full term baby and 5 Ch preterm one) firm (you don't want it to curl up) nasogastric tube with a radio-opague line in it as far as it will go, and then taking AP and lateral chest X-rays. This will show his oesophagus ending in a blind pouch. Suck out the contrast medium immediately after wards.
Refer him immediately with the tube reaching the blind end of his proximal oesophagus. Frequent aspiration is particularly important to prevent him aspirating saliva. Also, give him a small dose of atropine (A 2-4) to minimize secretion. Make sure that the staff who accompany him understand that they must continue to aspirate the tube, and suck out his pharynx during transport, to prevent fluid entering his lungs. If you cannot refer him, there is little you can do.
DUODENAL ATRESIA AND STENOSIS present as vomiting on the first day of life. His vomit is usually bile-stained, because the obstruction is usually below the ampulla of Vater. His upper abdomen is distended. If the obstruction is above the ampulla of Vater, there will be no bile in his vomit. The absence of dribbling saliva and cyanosis after feeding distinguishes duodenal from oesophageal atresia.
Erect AP and lateral films show a characteristic 'double bubble', with no air (or very little) in his small gut beyond. The bubble on the right is in his distended duodenal cap, and that on the left is in his stomach. He may also have Down's syndrome. If you cannot refer him, you may possibly be able to do a duodeno-jejunostomy or a duodeno-duodenostomy (28.3).
JEJUNO-ILEAL ATRESIA OR STENOSIS may occur at any point in the small gut. Typically, it presents as bilious vomiting within 24 hours of birth slightly later than with duodenal atresia, perhaps an hour after the first breast-feed; but it may be delayed for 2 or 3 days. If he has jejunal stenosis rather than atresia, it may be delayed for as long as 2 weeks. Obstruction in the upper jejunum is more common. If the obstruction is low,it presents more slowly, with distension more evident than vomiting. About half these children pass some meconium. Hydramnios is common in the mother.
Erect AP and lateral films show considerable gaseous distension, ending at the site of obstruction, usually with several fluid levels-one or two are normal. Unfortunately, by the time that several fluid levels are present obstruction is advanced.
If you cannot refer him you may be able to resect and anastomose his gut (28.3).
VOLVULUS OF THE SMALL GUT may present in older children, as in Section 10.9, with sudden abdominal pain, distension and shock, or it may present in the first week of life, usually very soon after birth, as an acute abdomen with bilestained vomiting and abdominal distension. Volvulus usually involves the distal small gut and proximal colon, and is due to a congenital malrotation. If Ladd's bands (see below) are responsible, surgery is simple, because there no need to anastomose gut. Strangulation obstruction develops rapidly.
Operating for a neonatal acute abdomen
Anastomosing neonatal gut is no easy task, but if you are surgically nimble, are well-experienced with adult gut, and have the right sutures and devoted nurses, you may succeed. You have one advantage-the contents of a neonate's gut are sterile, so that contamination of his abdominal cavity is less of a hazard than it is later on. Anaesthesia is a major problem, but you can use local anaesthesia, although the absence of relaxation does make the operation more difficult. Whatever the difficulties, you may be sure that if you cannot refer him, and don't operate, he will surely die.
An abdominal wound easily breaks down in an infant. It is least likely to do so if you make a transverse muscle-cutting incision. A muscle splitting paramedian incision is next best for healing, and gives good exposure. A child grows, so suture his gut and his wound with interrupted sutures to allow him to do so. Suture his fascia meticulously, with good bites on each side.
You will find that that distal end of an obstructed neonatal gut is so collapsed, and small, that an end-to-end anastomosis is difficult. So make an end-to-side or a side-to-side one. The disadvantage of doing this is that, later, the cul-de-sac that you make may become a refuge for bacteria, and cause the malabsorption syndrome, especially in the small gut. These anastomoses can however be revised later, if necessary.
NEONATAL ACUTE ABDOMENS
ANAESTHESIA AND PREPARATION. See Section 28.1. If you cannot refer a child, operate at 24 hours, or as soon after wards as possible.
INCISION. Make a right muscle-splitting paramedian, or supraumbilical transverse incision. Examine his whole gut, because there may be more than one obstruction.
DUODENAL ATRESIA takes various forms, including one in which the pancreas is all round the duodenum (annular pancreas, very rare). Anastomose the first part of his duodenum to the first loop of his jejunum behind his colon. If this is difficult, make the anastomosis in front of his colon. Do a side to side anastomosis in one 'all coats' layer, or in two layers with interrupted sutures. One of the difficulties is that you cannot see his pancreatic or bile ducts, and if you injure them he will certainly die. Bring up a loop of his proximal jejunum through his gastrocolic omentum, and anastomose it to his distended duodenum. Anastomosing collapsed jejunum is difficult.
JEJUNAL ATRESIA. You may find a variety of lesions, perhaps combined with malrotation, volvulus, meconium ileus, and meconium peritonitis (uncommon). Most often, there is a short segment, or segments, of atresia, or marked stenosis.
Anastomose his dilated jejunum above the stenosed segment to his collapsed jejunum below it. They will be very different in size, so do a side-to-side anastomosis. Unfortunately, a cul-de-sac will form, which will tend to grow.
Alternatively, if there is enough gut, resect the proximal loop to a point where it is about 1 to 1.5 cm in diameter. Cut it off at at 90º, and the distal one at 45º. If there is still a difference in size, make a short incision in the antemesenteric border of the distal segment, as in F, Fig. 28-2.
VOLVULUS OF THE MIDGUT. When you open his abdomen, you see distended coils of small gut, which may be cyanotic and congested, and obscure his right colon. Deliver his small gut to the surface, and protect it with warm moist packs. Examine the base of his mesentery to see which way it has twisted (usually anticlockwise). Untwist it, and hope that its normal colour will return. If it is viable, lay it back in place. If it is not viable, resect the gangrenous part, and anastomose viable gut. If you have to resect much gut, his outlook is poor. Before you close his abdomen make sure that: (1) there is no band of tissue obstructing his duodenum (band of Ladd, see below), and (2) his duodenum is patent. Do this by manipulating his stomach contents through into his small gut.
OBSTRUCTION BY BANDS (arrested rotation), If you find that his duodenum is obstructed by a band of tissue (band of Ladd) running from his caecum or ascending colon towards the right of his abdomen, this is the result of arrested rotation of his gut. These bands are often present but cause no problem.
If a band does appear to be causing obstruction, divide it. Divide the attachment of the band to his parietal peritoneum on the right side of the second part of his duodenum. Displace the right half of his colon to the left. If you see a second band extending from the mid line to the start of his jejunum, divide that also. There may be a third band running from his ileum to his ascending colon. His caecum will now be free; leave it on the left side of his abdomen, and don't try to put it back it in its normal place.
OBSTRUCTION BY A REMNANT OF MECKELS DIVERTICULUM,Sometimes a cord of tissue runs from his ileum to his umbilicus, causing his gut to strangulate round it (28-4). If so, check its viability, resect it if it is strangulated, and remove the 'cord'.
ANASTOMOSING AN INFANT'S GUT see also Section 9.3. Use the finest haemostats, and handle his gut with the greatest care. Hold it with stay sutures, and don't use clamps-its contents are sterile, so contamination is unimportant. Tailor its ends so that their sizes are a little more equal (see above). If you cannot do this, make a side to side anastomosis. Use a catheter to suck the proximal end clear of any inspissated meconium that would otherwise block the anastomosis. Make a single layer of instrument-tied interrupted sutures, using atraumatic 3/0 chromic catgut, or, better, 4/0 polyglycolic acid ('Dexon'), or 5/0 silk. If you have none of these sutures, don't operate. In most situations there is no room for a second layer.
Gently invert the posterior wall of his gut, as you insert the first sutures. If you can do this satisfactorily, inverting the anterior wall should not be difficult. As you go round the corner from the posterior wall of the anastomosis, to the anterior one, continue to invert the sutures.
POSTOPERATIVELY, see also Section 28.1. Give him 80 to 100 ml/kg/day of fluid. Either give him 0.18% saline in 5% dextrose to which you have added 2 mmol/kg/day of potassium (A 15.1). Or, give him half-strength Darrow's solution in 2.5% dextrose, which will contain the necessary potassium. Replace clear gastric losses with 0.45% saline in 5% dextrose. Replace greenish gastric aspirate with 5% dextrose in Ringer's lactate.
Start oral feeding as soon as possible-when signs of bowel function return. Start with 5 ml of expressed breast milk 2-hourly, and increase this gradually as he tolerates it. This should be possible on the 2nd or 3rd day. Either give it by nasogastric tube, or better, through a tube passed down his gut beyond the anastomosis (making an enterostomy beyond the anastomosis is difficult, because his gut is so small).
DIFFICULTIES WITH NEONATAL GUT OBSTRUCTION If you find part of his TERMINAL ILEUM IS FILLED WITH THICK FLUID, and that distal to this is some putty-like material, and distal to that again, some meconium pellets just above his caecum, he has meconium ileus (rare). Emptying his gut operatively is difficult, and is not sufficiently rewarding to be described here.
If you find that his SMALL GUT IS DUPLICATED, anastomose the parallel parts to one another at either end.
If you find extensive GANGRENOUS GUT, he has: (1) a volvulus, or (2) twisting round a band or cord (see above), or (3) neonatal necrotizing enterocolitis (rare). Exteriorize the gangrenous part, and close his abdomen. His chances are poor.
Hypertrophic pyloric stenosis
Hypertrophic pyloric stenosis presents as forceful bile-free vomiting, with constipation rather than diarrhoea, in a baby about three weeks old the range is 5 days to 5 months. It is more common in boys than in girls, and in the eldest child. To begin with he vomits one or two of his feeds each day, but as the obstruction gets worse, his vomiting becomes more constant and more projectile. Occasionally, he vomits brownish 'coffee grounds'. If he is not treated, he becomes dehydrated, alkalotic, hypochloraemic, hypokalaemic, and constipated; he loses weight, and becomes malnourished. Pyloric stenosis is not Diagnosed as it should be, and is too often thought to be yet another case of 'gastroenteritis'. But he does not have diarrhoea! Misdiagnosis is a tragedy, because surgery is not too difficult and is very effective.
You should be able to feel a child's hypertrofhied pylorus as a smooth olive-shaped swelling to the right 0 the mid line in his upper abdomen. If he cries you certainly won't be able to feel it, so sit him on his mother's lap, and feel for it while she feeds him from the breast. If you have difficulty, return a few minutes later, while she is still feeding him. Sit opposite her, look for waves of gastric peristalsis passing from his left upper quadrant towards the right. As they do so, his pyloric swelling will harden under your finger. Feel for the lump again. If you are persistent, you should be able to feel it in all cases-it establishes the diagnosis.
RAMSTEDFS OPERATION
RESUSCITATION. You can correct minor degrees of dehydration with oral fluids, but if a child becomes severely dehydrated and needs intravenous fluids, give him 20 ml/hour of half strength Darrow's solution, and reduce this to 10 ml/hour when he is passing urine. Don't give him >150-180 ml/kg/24 hours. Provided he is not severely dehydrated, he should be ready for operation in 4 to 6 hours. Most children need intravenous fluids for about 4 hours pre-operatively. He will usually stop vomiting as soon as his stomach is empty. If not, aspirate it through a nasogastric tube.
Alternatively, depending on what fluids you have, you may find this regime useful:
If he is only minimally dehydrated, with no significant electrolyte disturbance, give him 60 ml of Ringer's lactate orally. When his renal function is satisfactory, add 3 mmol of potassium chloride to each feed.
If he is moderately dehydrated, give him the first half of the calculated replacement intravenously as 5% dextrose in 0.45% saline, followed by 5% dextrose in 0.3% saline. As soon as he has passed urine, add 20 to 40 mmol/l of potassium to his intravenous fluid, depending on how ill he is.
If he is severely dehydrated, give him part of his initial replacement intravenously as 5% dextrose in 0.45% saline, partly as 0.9% saline, and partly as 5% dextrose in 0.3% saline. Add potassium as above.
ANAESTHESIA. (1) Ketamine (A 8.5). (2) Local anaesthesia. (3) Awake intubation and general anaesthesia. Sedate him pre-operatively with chloral hydrate 300 mg, and place him on a Dennis Browne crucifix, with his limbs bandaged to prevent excessive movement. He will not be able to clear his throat, so suck out his pharynx frequently. Aspirate his stomach just before you anaesthetize him, and leave the tube down.
The maximum dose of 2% lignocaine you can use is 1 ml, so dilute this in 20 ml of saline and use it to infiltrate his skin and rectus sheath.
INCISION. Open his abdomen through a transverse incision, centered over the swelling to the right of the mid line; it is usually half way between his xiphisternum and his umbilicus. Divide all his tissues in the line of the incision. Open his peritoneum.
Make the incision long enough (about 2.5 cm) to deliver the swelling into the wound. It may be quite difficult to find at first, because it may lie deep, partly covered by his transverse colon. Feel it with your finger. A small retractor may help to deliver it into the wound-it is always mobile. Hold the swelling between the thumb and index finger of your left hand. Keep your left middle finger against the distal extremity of his swollen pyloric muscle. Turn this so as to expose its antero-superior border.
Cut through the circular muscle along the length of his pylorus. Start just proximal to the white line (the junction of his pylorus and duodenum); at this point (the distal end of the swelling) the wall of his gut becomes thin suddenly. Extend the incision along the whole length of his thickened pylorus (the proximal end is less clear, because his stomach wall is also thickened). Spread its circular muscle, without harming the submucosa, which should bulge out of the incision. You are less likely to harm this, if you use the tip of a haemostat to separate the deeper muscle fibres.
Still using the tip of the haemostat, separate the fibres distallyon the duodenal side, under the white line, so as to divide all circular fibres without perforating his duodenal mucosa. As you do this, continue to mark and protect his duodenum with the middle finger of your left hand.
CAUTION! (1) Don't cut the white line at the site of the pyloric vein, or you may open his duodenum. (2) Don't sew up the muscle incision.
If, by mistake, you open his duodenal mucosa, close it with a few 4/0 atraumatic sutures, and suture omentum over the hole to fill the defect made by incising the muscle layer.
If a vessel bleeds, press with gauze for a few minutes; if this fails transfix it with 4/0 multifilament.
Return his stomach to his abdomen, and place omentum over the operation site. Close his peritoneum and posterior rectus sheath with continuous 3/0 chromic catgut. Close his anterior rectus sheath with interrupted 4/0 monofilament. Bring the skin edges together with fine monofilament.
POSTOPERATIVELY, if you succeeded in not perforating his duodenal mucosa, remove the nasogastric tube as he recovers from the anaesthetic. If you perforated it, leave the tube down for 24 to 48 hours, before you remove it and start feeding him. If he is alert and sucks well, start breast-feeding at 6 hours.
If he vomits frequently during the first 24 hours, wash out his stomach to remove the excess mucus.
If he is taking Insufficient fluid by mouth to maintain an intake of 100 ml/kg/day, give him half-strength Darrow's solution.
If he continues to vomit after 24 hours, you may not have divided his pylorus adequately. If necessary, operate again. Wait until 14 days, if you can; but it is better to operate earlier than to let him get worse.
Disorders of the omphalo-mesenteric duct (Meckel's diverticulum)
In fetal life the intestinal tract and the yolk sac are joined by the omphalo-mesenteric or vitelline duct. Remnants of this may persist and present as: (1) A persistent discharge from the umbilicus which is occasionally faecal. (2) Gastro-intestinal bleeding from ectopic gastric mucosa in Meckel's diverticulum. (3) Gut obstruction caused by gut twisting around a persistent vitelline duct, as described in Section 28.3. You may occasionally have to resect and anastomose gut in connection with any of these three, or, rarely, with the other abnormalities in Fig. 28-4.
Anorectal malformations
Anorectal malformations are not uncommon. A child who has the misfortune to be born with one presents soon after birth with abdominal distension and the failure to pass meconium. Some lesions are incomplete, and present later with difficulty passing faeces, small faeces, or distension.
There are many kinds of lesion, but what matters most is whether a child's rectum ends above or below his puborectalis sling. If his rectum extends below it (infralevator lesions), you can make him an anus, or dilate a stenosed one without difficulty. But if his rectum ends above this sling (supralevator lesions), you will have to make a temporary colostomy, and refer him later for his sigmoid colon to be pulled through to his perineum. This is difficult, but continence can be achieved in most cases.
A child's anus or rectum may fail to develop entirely (agenesis), they may partly fail to develop (atresia), or his rectum or anus may be narrowed (stenosis). Agenesis (but not the other lesions) may be combined with fistulae between the rectum, and the urinary, or genital tracts, of either sex. These variables combine to produce a complex series of lesions. Some fistulae are useful, because you may be able to dilate them to make an anus. About 75% of cases are low lesions with fistulae.
LOW ANORECTAL LESIONS
Anal stenosis in boys or girls (rare). Imperforate anal membrane (3%) in boys and girls. Low rectal (anal) agenesis without a fistula. If there is a fistula (75%), it is rectoperineal or rectourethral in a boy. In a girl it is rectoperineal, rectovaginal, or rectovulvar.
HIGH ANORECTAL LESIONS
High rectal agenesis in boys or girls without a fistula (7%). If there is a fistula (15%), it is rectourethral or rectovesical in a boy. In a girl it is rectovaginal or rectocloacal.
Rectal atresia in boys and girls (rare and not discussed further). There is a normal looking anus in the normal place, low gut obstruction (no meconium, abdominal distension and vomiting late). A contrast enema is safter than probing.
You should be able to diagnose which kind of lesion a child has from the clinical signs and a single X-ray (28-6). The risk in trying to repair an anorectal lesion is that, if the lesion is higher than you expect, you may so damage his rectal anatomy that even an expert cannot repair it later. If you find you are operating below skin level, stop, do a colostomy, and refer the child for repair later. Don't divide any muscle. If you are in any doubt, a colostomy would be wiser, even though you may occasionally do one unnecessarily. The penalty for failure is incontinence. Make a transverse colostomy rather than a sigmoid one, because it will make the secondary procedure easier, although it will cause more fluid loss.
ANORECTAL MALFORMATIONS
If possible, refer these children, unless they need dilatation only.
EXAMINATION. Ask your midwives to examine the anuses of all children. Ask them to refer: (1) any child with an abnormal anus, (2) any child who passes no faeces for 12 hours, and whose abdomen distends. Examine him, and if necessary, pass a rectal thermometer or a stiff catheter. If he has no anus, an opening must be provided before he distends. Look for other congenital abnormalities.
If there is a mass of irregular epithelium where the anus should be, the diagnosis is almost certainly anal stenosis. Probe it and look for even a trace of meconium to confirm the presence of an anus.
If the anal skin is smooth, there must be some other kind of lesion, other than stenosis.
If there is a thin veil of epithelium overlying the anal orifice, surrounded by normal puckering and rugae, there is an imperforate anal membrane (only 3%).
If the anus looks normal, until you put a probe into it, when you find that the rectum is almost or completely blocked, the diagnosis is rectal atresia. You may find a very small hole which you can dilate.
LOOK FOR FISTULAE IN ANORECTAL MALFORMATIONS
If a boy has low rectal (anal) agenesis, look for: (1) meconium in his urine. If you don't find it, he has probably not got a fistula. (2) Examine his perineum, and his perineal raphe along his scrotum. If you see any sign of a black head of meconium, he probably has a fistula leading to a low lesion.
If a girl has low rectal (anal) agenesis, examine her perineum and vulva for fistulae. If you find a suspicious opening, probe it gently it may extend downwards and backwards under the skin of her perineal body, and confirm that she has a low lesion.
If a boy has high rectal agenesis, examine his urine for meconium.
If a girl has high rectal agenesis, look for a fistula on the posterior wall of her vagina, below her introitus. It may connect with a cloaca where her urinary tract, vagina, and rectum come together into a common channel.
X-RAYS FOR ANORECTAL MALFORMATIONS
Wait until 12 to 16 hours after birth, when the gas the child has swallowed has reached the blind lower end of his gut.
Turn him upside down so that his anus is uppermost. Flex his knees. Strap a small piece of metal such as the metal adapter of a needle flat on the skin where his anus should be.
Take a lateral film. The bubble of gas in his gut will be uppermost. The distance between it and the metal will show you how much tissue there is between the bottom of his gut and his skin.
Interpret the film in two ways: (1) If the gas bubble is less than 2 cm from his anus, he has a low lesion. If it is more than 2 cm from his skin, he probably has a high one. (2) Draw a line between the posterior part of his pubis, and the lowest segment of his sacrum. If any blind dilated bowel has gone beyond this line, he has a low lesion. If it remains above this line, his lesion is high.
CAUTION! (1) X-rays are useful but not completely reliable. There may not have been enough gas in his gut, or he may have a fistula higher up. (2) He may have a vesicorectal fistula and a fluid level in his bladder (uncommon). Exclude this by taking a supine lateral film.
OPENING A LOW LESION
If a child has a low lesion, operate early, especially in a boy. His obstructed gut is going to distend, so, as soon as he has swallowed enough air to help make the X-ray diagnosis, pass a nasogastric tube. Keep him hydrated and give him vitamin K.
ANAESTHESIA. (1) Ketamine (A 8.5). (2) Chloral hydrate and local infiltation anaesthesia. (3) Awake tracheal intubation and general anaesthesia.
ASPIRATION. Immediately before you operate, aspirate his perineum with a syringe and large-bore needle. This will tell you the exact level of his anal pouch, while he is relaxed and anaesthetized.
CAUTION! If you are in doubt, do a colostomy.
ANAL STENOSIS (boys and girls). If the opening is very small, use a filiform urethral catheter with a metal follower or the smallest Hegar dilator. Continue to dilate the child about every 2 weeks with your little finger. Later, this can be done by a nurse or the child's mother.
IMPERFORATE ANAL MEMBRANE (boys and girls). Incise the epithelial covering of the anus, and dilate it with a Hegar dilator. Continue to dilate it as above.
LOW RECTAL (anal) AGENESIS WITHOUT A FISTULA. Make a cross-shaped incision so as to mobilize four separate flaps, as in A, Fig. 28-7. Contributors then differ. One says: don't divide any muscle; if you don't immediately find the rectum under the skin, stop, make a colostomy and refer.
Another says: as soon as you have found the external sphincter, divide it at 12 o'clock, and dissect towards the blind end of the gut, up to 2 cm from the skin. Mobilize the blind gut for a few more centimetres, so that you can relax it, and bring it down to the skin within the cut external sphincter. Cut four triangular flaps, so that they interdigitate with the skin flaps. Join them with interrupted stitches of fine catgut (B, and C). Don't make the anastomosis under tension, if it is tight, you have not mobilized gut enough.
LOW RECTAL (anal) AGENESIS WITH A FISTULA IN A GIRL.
This is the commonest anorectal lesion in a girl. Her sphincter muscles pass round the fistula. You can do two things: (1) is much the best; only do (2) if you cannot do (1).
(1) You can dilate her fistula until it makes an adequate anus. Start by using a rectal thermometer, and later a uterine sound. Then at 6 months, refer her to have her anus brought back to its proper place in her perineum. If this is impractical, it is not a disaster, but it will mean that she will always defecate from her fourchette. This is much less of a disability than it might seem, because it is compatible with a normal sex life, and her husband may be quite unaware of it!
(2) You can do a cutback operation, as in B, Fig. 28-8, but only if, before you cut, your scissors point directly backwards and are superficial. If they point in any other direction, and especially if they point deeply, she probably has a high lesion, not a low one, and she may subsequently become incontinent. After you have cut, mobilize the posterior 1800 of the cut, so as to bring the apex of the incision comfortably down to her skin. Join her gut and her skin with interrupted sutures.
LOW RECTAL (anal) AGENESIS WITH A FISTULA IN A BOY. His fistula is usually perineal, and his lesion is low. Unroof the fistulous track, as in C, Fig. 28-8. This will lead back to a normal anorectum, which you can open and dilate (D). Stitch his rectal mucosa to his skin. Finally, remove the mucous membrane lining the fistula.
POSTOPERATIVE CARE (all low lesions). Follow the child up carefully. The anus will probably need dilating at least once a week for a month or more. Warn the child's mother that toilet training will be delayed.
TRANSVERSE COLOSTOMY FOR HIGH ANORECTAL MALFORMATIONS
PREPARATION. Pass a nasogastric tube and suck out the child's stomach frequently. Check his temperature, and put him in a warm bath if it is low. Put him on an electric blanket on the operating table, and bandage his arms and legs in cotton wool to conserve heat.
ANAESTHESIA. (1) Ketamine (A 8.5). (2) Give him a general anaesthetic, as in section 28.3. (3) Local anaesthesia as for the other operations described here.
INCISION. Make a short transverse incision in his right hypochondrium (see Section 9.5). Identify his transverse colon, from its connection by a short omentum to the greater curvature of his stomach.
CAUTION! Don't deliver his sigmoid colon.
Deliver his transverse colon through the wound, and pass a plastic rod under it through a hole in its mesentery, to make sure it remains outside his abdomen. Connect its ends with a rubber tube. Place some catgut sutures between the muscle of his gut and his abdominal wall.
Open the exteriorized loop immediately with a longitudinal incision. Suck out the contents of the proximal loop to decompress his abdomen. Refer him early if fluid loss from his colon is serious; otherwise refer him for definitive repair at 6 months.
DIFFICULTIES WITH ANORECTAL MALFORMATIONS
If you are NOT SURE WHAT TO DO, remember that a fistula is a favourable lesion because there is already an opening between the gut and the outside. If it is narrow, dilate it. If it is wide, there is no need for an urgent colostomy unless: (1) he has signs of intestinal obstruction, or (2) his urinary tract is involved. If so, a colostomy is urgent.
If his ANAL MUCOSA EVERTS after you have operated on his imperforate anus, there is little you can do about it. This is not uncommon during the first three months.
If his ANUS BECOMES STENOSED, it will probably do so because it is fibrosed. Unfortunately, regular dilatation perpetuates the cause of the fibrosis. There may come a time when you will have to balance the softening that will follow dilating less often, with the widening that will follow dilating more often.
If he LOSES FLUID EXCESSIVELY FROM HIS COLOSTOMY, wait for 14 days, while giving him extra fluid to maintain a satisfactory fluid balance. His stools will usually become formed; if they don't, refer him.
If his PROXIMAL TRANSVERSE COLON PROLAPSES, this is a nuisance, but is not serious. You may be able to reduce it by pushing it back with vaseline gauze, or KY jelly, on a glove. If necessary, teach his mother to do this.
Hirschsprung's disease
In this not uncommon disease, the parasympathetic plexi of a child's rectum and distal colon do not develop normally, so that his faeces are not propelled onwards as they should be. The length of the aganglionic segment varies, and may stretch from his upper rectum, to half-way along his transverse colon. He becomes constipated, and his abdomen distends; and in acute cases his gut may obstruct. He may present at any time during childhood in any of the following ways:
HIRSCHSPRUNG'S DISEASE
If a child presents soon after birth with subacute obstruction, its onset is more gradual than with complete mechanical obstruction, but it is still fairly acute. Rectal examination is normal. X-rays show fluid levels and an upside-down X-ray shows air as far as his anus. His distallarge gut may look narrow. Do a transverse colostomy, as for a high rectal malformation (28.6), and refer him.
If he presents later, during the first three years, with abdominal distension, and his condition is not acute (usual), refer him for resection of the diseased segment. If it is acute (very unusual), and if it is essential for his health, do a transverse colostomy before referring him.
If he presents up to the age of 10 years as 'a child whose abdomen is large and who does not open his bowels easily', refer him. The surgeon will have to distinguish Hirschsprung's disease from 'chronic constipation', by doing a biopsy which includes the submucosa, and with it the faulty plexi. At operation, he will have to decide where the faulty plexi end, excise the diseased segment, and anastomose the colon to the distal rectum.
Congenital abnormalities of the female genital tract
The female genital tract occasionally fails to develop normally. The more common abnormalities are fortunately quite easy to treat. If the vagina fails to communicate with the vulva, a patient may present at birth with hydrocolpos (rare, described below), or at puberty with haematocolpos (uncommon), as described in Section 20.14.
FEMALE GENITAL ABNORMALITIES
If you are shown a baby girl with an ABSENT VAGINA, examine her carefully and consider the possibility of labial fusion. This is common, whereas an absent vagina is rare. If her labia minora are not obviously present and normal, and you can see even the faintest line of adhesions, this is what she has. Gently separate them with a probe. Ask her mother to clean her vulva with tepid boiled water daily for a week, and to bring her to a nurse at one week and two weeks.
If you feel a LOW ABDOMINAL MASS IN A GIRL BABY(rare), examine her vulva and vagina. If you find a bulging membrane just above her vagina, her uterus and vagina are full of watery fluid-hydrocolpos.
If the membrane feels thin, incise it with a cross-shaped incision. Do nothing else, or you will increase the risk of infection. She will settle down.
If there is no thin membrane, her vagina is absent through all or part of its length. You have a difficult problem. Refer her.
Omphalocele (exomphalos)
Not uncommonly, a child is born with a defect in his abdominal wall which involves his umbilicus, and leaves his viscera covered only by a translucent layer of peritoneum and amnion. In omphalocele minor there are only a few loops of gut in the sac, but in omphalocele major, it may contain most of his abdominal organs,including even his liver. Often he has no other defects, so he is well worth saving.
The sac of an omphalocele is weak, and easily ruptures or becomes infected, causing peritonitis. If it ruptures during delivery, surgery is urgent. If it survives delivery intact, you have the choice between operative and non-operative treatment. Surgery is the usual method; non-operative treatment needs particularly good nursing care.
If you can reduce the contents of the sac, he has omphalocele minor, which you can treat without difficulty. If you cannot reduce them, he has omphalocele major, which has to be managed surgically in two stages: (1) Undermine skin flaps to make an artificial hernia, and bring them together over his herniated viscera, which will then lie subcutaneously. (2) At nine months to a year, his abdominal cavity should have grown enough to allow you, or an expert, to reduce the hernia into it.
OMPHALOCELE (exomphalos)
As soon as the child is born, apply warm saline soaks to the omphalocele, to keep it moist and to prevent him getting cold. Wrap him up well.
If his omphalocele has ruptured, or is threatening to do so, operate betwen 6 and 24 hours. If its covering appears stable, you can wait a few days. If possible refer him, especially if his omphalocele is large.
NON-OPERATIVE TREATMENT FOR OMPHALOCELES
If the sac is intact and its coverings are fairly strong (common with small omphaloceles, but not with large ones), you can, if necessary, treat omphaloceles of any degree this way. It is however second best, so repair them if you can.
Clean the sac with an antiseptic, and dress it with spirit. Don't use mercurochrome. Apply spirit to the sac hourly for the first 48 hours, and then less often as an eschar forms. This will separate from the periphery, as epithelialisation takes place, but it may take several weeks. He will then be left with a large skin-covered ventral hernia (H, Fig. 28-9) which will need repair later.
THE SURGICAL REPAIR OF OMPHALOCELES
ANTIBIOTICS. Always give these if the sac ruptures before or during the operation. Give him ampicilllin 10 mg/kg 6-hourly, and metronidazole 7.5 mg/kg 8-hourly.
ANAESTHESIA. Set up a scalp vein drip or do a cut-down. Give him a general anaesthetic (A 18.1) or ketamine, tracheal intubation, and suxamethonium (18.2). You can use local anaesthesia for a small omphalocele (not easy), but not for a large one because you need muscular relaxation. If he has an omphalocele major, have blood available, and replace any blood he loses meticulously (3-1).
REPAIRING AN OMPHALOCELE MINOR
INDICATIONS. His omphalocele is small enough for you to be able to: (1) reduce it while he is anaesthetized and breathing spontaneously, and (2) bring the edges of the sac together, without embarrassing his respiration. If it is embarrassed, do a two-stage repair.
REDUCTION. Prepare his skin, including the hernial sac and drape him. Tie and divide his cord, as it emerges from the sac, with '0' chromic catgut (monofilament cuts through too easily).
Try to reduce the contents of the sac into his abdominal cavity. lf ou fail, manage him as an omphalocele major. If you succeed, incise the skin 0.5 cm, or less, from the edge of the defect (8, in Fig. 28-9). Find and tie his umbilical arteries in the 5 and 7 o'clock positions; tie his umbilical vein superiorly in the 12 o'clock position. Expose the edge of his fascia and peritoneum, and remove a ring of tissue. Excise any tissue of doubtful viability from the sac wall.
Reduce his hernia, and close the defect in three layers. If necessary, hold up the edges of the defect with haemostats(E). Close his peritoneum with 3/0 catgut. Close his fascia with 2/0 monofilament mattress sutures. Close his skin with simple sutures of 2/0 monofilament.
Postoperatively, feed him with expressed breast milk by nasogastric tube, until he is sucking well, usually in 2 or 3 days.
REPAIRING AN OMPHALOCELE MAJOR
This is an omphalocele which you cannot reduce in a single stage (A, in Fig 28-9).
FIRST STAGE. Incise his skin and subcutaneous tissue 0.5 cm or less from the edge of the sac (8). Find and tie the vessels as above. Excise any tissue of doubtful viability from the wall of the sac, including the stumps of the vessels. Extend the incision in the midline superiorly and inferiorly (D). Use scissors to extensively undermine a superficial layer of skin and subcutaneous tissue, and separate this from the muscles of his abdominal wall and the aponeurotic layer. Reduce dissection over his thorax to the absolute minimum that will provide enough skin cover. The flaps must cover the hernia without undue tension.
Hold the edges of the wound up in haemostats applied to the subcutaneous tissue, and reduce the contents of the sac so that they lie subcutaneously. Close the wound in two layers. Use 2/0 chromic catgut for the subcutaneous tissue and 2/0 monofilament for the skin.
Postoperatively, care for him as for omphalocele minor, but continue tube feeding for longer.
SECOND STAGE. Do this at nine months or a year, and refer him for it if you can.
If you cannot refer him, give him a general anaesthetic, and try to reduce the contents of the sac into his abdominal cavity. If you cannot reduce most of them, delay the operation and try again six months later.
If you can reduce all the contents, excise the sac, as for the first operation. Disssect out skin flaps down to the edge of the defect in his abdominal wall. Divide any adhesions between the subcutaneous tissue of the flaps and the sac, or his abdominal organs. Define the peritoneum and fascia at the edge of the defect. Then close the peritoneum and fascia longitudinally if you can (unusual).
If you cannot reduce all the contents into his abdominal cavity, reduce what you can. This will help his abdominal cavity to enlarge. Make his hernia smaller by suturing his subcutaneous tissue to the muscles of his abdominal wall with catgut. Then close his subcutaneous tissue and his skin as before. Operate again about a year later. The greater volume of his abdominal contents will help his abdominal cavity to enlarge.
The surgery of neonatal jaundice
Most neonatal jaundice is 'medical', and only occasionally is it 'surgical'. Surgically, a child's biliary tract can be blocked by epithelial debris or biliary sand (less common), in which case it may be temporary and clear spontaneously, or by atresia of his biliary tree (more common), for which his only hope is surgery. If much of his bile-duct is not canalized (25%), nothing can be done. If however his bile duct is sufficiently canalized for his gallbladder to be distended, and in connection with his proximal duct system (75%), it can be anastomosed to his proximal jejunum. If he has an extra-hepatic stump, this too can be anastomosed. Without surgery, he will die. Even in experienced hands his outlook is only fair. If a neonate passes clay coloured stools, try to refer him before 8 weeks.
NEONATAL JAUNDICE
The time of onset is of great diagnostic value.
If a child starts to become jaundiced in the first 6 to 24 hours, his jaundice is likely to be haemolytic as the result of: (1) Septicaemia from an umbilical infection. Look for signs of infection of his cord and the surrounding tissues (septicaemia from cord infection can also occur later). (2) Haemolytic disease of the newborn (uncommon in Africa). Usually, he is D+ve, his mother is D-ve and has anti-D antibodies as the result of having had a previous O+ve child or abortion, or of having been given D+ve blood. Other blood group incompatibilities may have the same effect (for example ABO). (3) Congenital syphilis (severe infection).
If he starts to become mildly jaundiced at 24-72 hours, he is likely to have 'physiological jaundice' due to a deficiency of glycuronidase (common, especially in 'small for dates' babies). This usually clears spontaneously, but can be helped by sunlight and phenobarbitone 1-2 mg/kg 12-hourly.
If he starts to become jaundiced at 24-72 hours, and is severely jaundiced by the 3rd to the 5th day, he may have G6PD (glucose-6-phosphate dehydrogenase) deficiency. This is common, especially in SE Asia and the Mediterranean littoral; a less severe form occurs in parts of sub-Saharan Africa.
If he has little or no jaundice for 3 to 6 weeks, by which time his liver is enlarged, and his stools are clay-coloured, he probably has some 'surgical' reason for his jaundice. Sometimes he is slightly jaundiced at birth, or becomes so a few days later; his urine is dark brown early.
CHOLECYSTOJEJUNOSTOMY
Surgery and anaesthesia are difficult, so refer him if you can; he is very small, and tends to bleed. If you cannot refer him and decide to operate, give him vitamin Kt (water-soluble) 2 mg intramuscularly, and operate next day.
ANAESTHESIA. (1) General anaesthesia. (2) Intramuscular ketamine, followed by small intravenous doses.
INCISION. Make a subcostal transverse or right upper paramedian incision. If he has a distended gall-bladder, proceed. If he has some inoperable anomaly, close his abdomen. Anastomose the fundus of his gall-bladder to his jejunum using interrupted sutures of 3/0 catgut or 4/0 multifilament (e.g. silk). See Fig. 13-5.
Postoperatively, manage him as in Section 28.3.
Spina bifida, meningocele, and myelomeningocele
Congenital abnormalities of the spinal cord and vertebral column are not uncommon. You may see:
Spina bifida alone, in which the arches of a child's vertebrae remain open, usually in his lumbar region. He often has a Mongolian spot over the defect (this is less easily seen in a dark skin, but it is there if you look for it), and/or some extra hair and fatty tissue. Uncomplicated spina bifida is usually symptomless, but watch him carefully. If his legs become weak, refer him: he may have a bifid spinal cord separated by a bony spur (diastematomyelia, rare).
A meningocele is a eSP-filled extension of his spinal canal, without any spinal cord or spinal nerves in it; commonly in his lumbar region, and usually associated with spina bifida. His cord is normal and he has no neurological abnormality. It is a relatively simple procedure to obliterate the sac, close his spinal membranes at the level of his laminae, and then to close his skin. This is a closed lesion, so there is no hurry.
A myelomeningocele is more common than a simple meningocele, and takes two forms: (1) He may have a closed swelling containing spinal cord and/or spinal nerves. (2) More often, his spinal canal is open and leaks esp, with his flattened cord forming a plaque on its surface. Both varieties may occur in the cervical (rare), thoracolumbar, lumbar, or lumbosacral regions, and other abnormalities are frequent, particularly hydrocephalus. Most children have irreversible paralysis of their legs, and loss of sphincter control, so that surgery merely prolongs their misery, and that of their parents. You may however be justified in operating on sacral myelomeningoceles, as described below.
MENINGOCELE
DIAGNOSIS. If the swelling on the child's back is covered by skin, his legs are not weak, his anus is not lax, and he micturates normally, he probably has a simple meningocele. Otherwise, you cannot tell a meningocele from a myelomeningocele from its site, or its covering. Transilluminate it; you may occasionally see nerves outlined inside it.
If he has any neurological signs, he certainly has a myelomeningocele. These signs are: (1) No movement in his legs. (2) An absent or meagre response to tickling or pin-prick. (3) A lax anal sphincter which does not contract on digital examination. (4) Observe the way he passes urine; he may pass it as a jet at specific times (normal), or it may leak out from time to time (abnormal).
Look for other anomalies, particularly talipes (27.15). Measure the circumference of his head, now and later; he may develop hydrocephalus.
MANAGEMENT OF MENINGOCELES
If his meningocele is covered with normal skin (usual), surgery is not urgent. Operate when convenient, normally at about 6 months.
If his meningocele is not covered by skin (unusual), surgery is urgent, because it is easily damaged, and may become infected. If he is fortunate and well cared for, it may epithelialize.
If the mass is in his cervical or thoracic regions, it is more likely to be a simple meningocele, so try to refer him. Don't try to operate on lesions in these sites yourself.
If: (1) the central canal of his spinal canal is open to the surface, or (2) he has a myelomeningocele with neurological signs (commonly In the thoracolumbar or lumbar regions), there is nothing to be done, except compassionate palliation.
If he has a sacral myelomeningocele, with normal power in his legs (normal sphincter control is unusual), urgent operation may be justified-see below.
CLOSING A MENINGOCELE
If you cannot refer him, proceed as follows. Take the strictest aseptic precautions and be careful not to damage his cord.
INDICATIONS. A fairly small sacral or lumbar meningocele, with no neuroJogical signs, no hydrocephalus, and no other congenital abnormalities.
CONTRADICTIONS. (1) Complete or virtually complete denervation below the level of the lesion. (2) Progressive hydrocephalus. (3) A very large lesion, in which you will have difficulty closing the skin and subcutaneous tissues without tension. (4) Lesions in sites other than the lumbar or sacral region.
ANAESTHESIA. (1) General anaesthesia, with precautions, particularly tracheal intubation, for the prone position (A 16.12). (2) Ketamine (A 8.5). If you use ketamine without intubating him, lie him semiprone (which will make surgery more difficult), when his breathing should be satisfactory.
PREPARATION. Lay him prone with the head of the table depressed at least 30°, to minimize the loss of CSF when you incise the sac. Prepare and drape a wide operative field.
Clean the sac meticulously and aspirate it slowly with a 0.8 mm needle until it collapses.
If the swelling is covered by skin, make a longitudinal elliptical incision, through normal skin at the base of the swelling. Cut through the subcutaneous tissue to the deep fascia, and define the neck of the sac by blunt dissection. The defect in the fascia will usually be quite small, and easy to expose. Free all surfaces of the sac, and open it over its dome.
If there are no nerve filaments, amputate the sac at its base, and close it with continuous 4/0 or 5/0 catgut.
If you find nerve filaments, he is not a case of simple meningocele, and his prognosis is poor. Preserve the nerve filaments with the greatest care, try to free them from the sac, close it, and try to cover every filament.
Complete the repair by overlapping his deep fascia and closing his skin.
If you have difficulty closing his skin: (1) Raise flaps by undermining extensively, and making lateral relaxing incisions if necessary. Or, (2) use double pedicle advancement flaps (57-19), with split skin grafts to cover the raw areas. Seal the wound meticulously, and nurse him in the prone position.
SACRAL MYELOMENINGOCELE
This is the child with a sacral myelomeningocele and normal power in his legs, as described above.
Dissect the sac from his cauda equina, being careful not to damage it. Close his dura with continuous 4/0 catgut. Raise flaps of skin and subcutaneous tissue by undercutting with scissors, making lateral relaxing incisions if necessary. Close his skin with 4/0 monofilament, apply a dressing, and nurse him as above.
Congenital vascular lesions
Congenital vascular lesions are not uncommon, and may worry a mother, so you should know how to advise her. There is little you can do for most of them except, to watch a child carefully, and refer him when necessary.
A capillary haemangioma, or 'strawberry naevus' (D, in Fig. 28-11) is a skin lesion, and is commonest on the face. It is bright red, and a little raised, sometimes quite markedly. It may occur on the mucous membranes and may bleed severely. A baby is usually normal at birth; the lesions appear one to four weeks later, and enlarge for a few months. Rapid growth is unusual after six months, but slow growth is common up to a year. If you leave them alone, 95% will resolve spontaneously. The first sign that this is happening is the appearance of a lighter, flatter area. Resolution may be precipitated by trauma, but this usually causes only minor scarring. Advise his mother that his lesion will probably disappear slowly. Warn her not to allow traditional healers to scarify the lesion, which may cause bleeding, infection, and worse scarring. If resolution is slow, or she presses you, refer him.
A port-wine stain (B, in Fig. 28-11) consists of cavernous channels, and usually occurs on the face or neck, but is not uncommon on the trunk. It is usually present at birth and does not progress, but it may be quite extensive. The texture of his skin is normal, and is not usually thickened; occasionally there is some hypertrophy and irregularity.
If a lesion is particularly ugly, as in a light skin, cosmetic creams may help. Otherwise, there is no treatment. Reassure his mother that his lesion will not enlarge.
Lesions on the face, in the area of distribution of the ophthalmic and maxillary branches of the fifth nerve, may be associated with vascular abnormalities of the cerebral cortex (Sturge-Weber syndrome), and present with seizures. Glaucoma of the ipsilateral eye sometimes occurs, and is treatable (24.6). Refer him to a neurosurgeon.
A capillary cavernous haemangioma consists of abnormal capillaries, arteries, and veins, and is partly compressible. It is usually present at birth, and commonly occurs on a child's face, axillae, or neck, where it may extend into his mediastinum. It may occasionally resolve spontaneously over several years (unusual), or it may enlarge rapidly.
Watch him carefully, and at each visit use a measuring tape to record the exact size of his lesion in two dimensions at right angles. If it does enlarge, refer him-surgery is likely to be difficult, and bleeding severe.
A lymphangioma is a congenital capillary cavernous lesion, which is usually present at birth, in the same sites, commonly the neck, as a haemangioma. Some are well circumscribed, but others merge with the surrounding tissues. Some become slowly smaller, others enlarge, sometimes quite quickly, and cause pressure effects in the chest. Most eventually need surgery, which can be difficult and may not be complete.
Watch him and measure his lesion regularly. Refer him if it enlarges, especially if it enlarges rapidly.
A cystic hygroma (E, and F, in Fig. 28-11) is a fluctuant, lobulated, translucent mass of abnormal lymphatic vessels, which presents as a soft, diffuse swelling in the posterior triangle of the neck, or occasionally the axilla. It appears early, and may be present at birth. The mass is not attached to the skin, and does not move on the deep tissues. A cystic hygroma usually causes no serious problems, and usually enlarges slowly. Occasionally, it advances rapidly and causes pressure symptoms at the root of the neck, or, rarely, it may regress spontaneously. The main differential diagnoses are: (1) Alymphangioma. (2) Abranchial cyst; this is unilocular and low in the neck, along the anterior border of the sternomastoid; aspiration yields a thicker opalescent fluid, instead of the thin, clear, watery fluid from a hygroma. (3) A capillary cavernous haemangioma.
Surgery can be difficult, because a hygroma may penetrate deeply, or have lymphangiomatous extensions. Watch him carefully, and unless it is clearly regressing, refer him. If it is very large, it should be operated on earlier. If it is not so large, removal will be easier when he is more than a year old.
28.11 Other paediatric problems
Here are some of the other problems you may meet, and which do not require complete sections to themselves. Intussusception (10.8), and Ascaris obstruction (10.6) are described elsewhere.
OTHER PROBLEMS
PROBLEMS IN NEONATES
If a neonate or an infant has RETENTION OF URINE with overflow, the neck of his bladder is probably obstructed, by the contraction of his internal meatus, or more commonly by URETHRAL VALVES. These usually present in the first six months of life, but they can occur at any age, and even into adult life. They can present as retention, urinary tract infection, dribbling incontinence or renal failure. You may feel that an infant's bladder is distended, and you may be able to feel his kidneys. His distended bladder may simulate a rhabdomyosarcoma of the bladder, but will disappear on catheterization. Pass a urethral catheter (a 6-8 Ch feeding tube is suitable) under ketamine or general anaesthesia. Alternatively insert a suprapubic catheter. Treat any infection and refer him. A urethral catheter is unsatisfactory, because its bore is so narrow that it soon blocks. A suprapubic tube is better, but even this does not drain his ureters or kidneys well.
If a neonate is born with a LARGE SOLID MASS BELOW HIS SACRUM, it is probably a coccygeal teratoma or hamartoma (benign or malignant). Refer him for surgery soon.
PROBLEMS IN OLDER CHILDREN
If a child DISCHARGES URINE FROM HIS UMBILICUS and his urethra, he has a persistent urachus (rare). Sometimes urine discharges in a small spurt during micturition. He may not present until he is a year old or later. Excising the track is not difficult. See Section 28.5.
If he has a dirty UMBILICUS WHICH DISCHARGES, smells, and occasionally bleeds, he has a GRANULOMA OF THE UMBILICUS (very common), caused by an infected remnant of his cord (28.5). Clean it with spirit daily, apply alum powder (Paediatric Alum and Zinc Dusting-powder BPC), and keep it dry. Alum powder is also preventive, because it quickly dries the stump of the cord.
If a boy's URETHRA OPENS ON THE VENTRAL SURFACE OF HIS PENIS, he has hypospadias. Refer him at 3 years, so that the necessary plastic surgery can be completed by the age of 6.
If a child has RECURRENT ATTACKS OF CHOLANGITIS which may lead to JAUNDICE, suspect cystic dilatation, usually of his common bile-duct (CHOLEDOCAL CYST). An expert should be able to anastomose the fundus of his gall-bladder to his Jejunum, and excise the cyst. If this is impossible, anastomosis alone is of some value.
