Treatment Protocols

Treatment Protocols

  • Abdominal wall defects

    • Anterior abdominal wall defects are congenital defects centred around the umbilicus.
    • The abdominal wall is embryologically provided for by 4 folds (cephalic, 2 lateral and a caudal fold) which provide equal portions and meet to form a central umbilicus.
    • Umbilical anomalies may be in the form of protrusions (eg. exomphalos and hernias), or leakage of either bowel contents (eg. with patent omphalomesenteric remnants) or urine (eg. with patent urachus).
    • Anomalies may also occur juxta-umbilical (eg. gastroschisis and paraumbilical hernias).
    • A shift of the umbilicus, craniad or caudad, has serious implications (eg. bladder exstrophy, Pentalogy of Cantrel).

    PRESENTATION

    • Anterior abdominal wall defects may present shortly after delivery as obvious abnormalities with the above umbilical anomalies (umbilical, juxta-umbilical or shift in umbilical) or as a Prune Belly Syndrome.
    • Less obvious lesions may present at any time as abdominal herniations or umbilical fistulae.
    • Abdominal wall protrusions (eg. exomphalos and gastroschisis) are life-threatening.

    MANAGEMENT

    • An antenatal diagnosis warrants transferring the mother for delivery at the centre where the newborn will be definitively treated.
    • Telephonic referrals of newborns with exposed viscera should be handled urgently. Umbilical fistulae may be seen at the earliest clinic.
    • Transfer should follow standard transport protocol. Cover the exposed viscera with plastic (to retain fluid and heat). Check the blood glucose and keep the child warm.
    • Associated anomalies may warrant investigations prior to surgery (eg. cardiac echo with Pentalogy of Cantrel) or (voiding-cysto-urethrogram with patent urachus).
    • Prepare for surgery as soon as the condition allows.
    • Arrange for post-operative ventilatory care.

    SURGERY

    • The aim is to enclose exposed viscera and to correct associated defects as soon as possible.
    • Returning viscera to the abdomen may lead to raised intra-abdominal pressure (eg. splinting of diaphragm, obstruction of IVC and renal vein flow).
  • Anorectal malformations

    DIAGNOSIS

    • If the patient does not have a normal anus in the normal position an anorectal malformation is present.
    • A decision must be made as to whether the abnormality is supralevator or infralevator.
    • Remember: Any patient who has one abnormality is likely to have others.
    • Discuss the implications of your diagnosis with the patient’s mother.
    • Massive abdominal distension which is interfering with breathing may necessitate urgent surgery. This is uncommon unless the diagnosis has been seriously delayed.
    CLINICAL EVALUATION

    • Is the patient male or female?
    • In males: Is there evidence of a fistula to the perineum or along the median raphe?
    • If yes, lesion is infralevator: plan for anoplasty.
    • If no, lesion may be supralevator.
    • Is there meconium in the urine? If yes: lesion is supralevator.
    • If still cannot decide: Wait 16-24 hours and do lateral shoot-through in the genupectoral position.
    • Further management will be decided by Consultant: usually a colostomy.
    • In females: Is there evidence of a fistula to the perineum?
    • How many orifices can be seen?

    One: Cloaca – will need colostomy
    Two: High vaginal fistula – will need colostomy
    Three: Low lesion – can be managed with dilatation until mature

    RADIOLOGICAL EVALUATION

    • “Invertograms” are rarely necessary in females, and are only useful in some males.
    • Ultrasound of the urinary tract should be done pre-operatively.

    OPERATION

    • Operation is only urgent if the child has intestinal obstruction.
    • There is time to assess associated abnormalities and optimise the patient.
    • Females with low lesions can usually be managed initially non-operatively
    • When a colostomy is indicated, fashion it in the proximal limb of the sigmoid colon
    • Wash out the bowel below the stoma.

    POSTOPERATIVE CARE

    • Check the viability of the stoma personally.
    • Prescribe a feeding programme.

    SUGGESTED READING

    • An Atlas of Anorectal Malformation: Pena, A.
    • Anorectal Malformations: Freeman NV. In: “Surgery of the Newborn”.

  • Blood transfusions

    • Blood is an expensive therapy.
    • A paediatric unit costs exactly the same as an adult unit, and is half the volume. It is therefore irrational to request two paediatric units, rather order one adult unit.
    • Everything useful has been removed from the donated unit and is sold as separate items. All that is left is red cells in suspension.
    • These red cells deteriorate with age. Blood over one week of age should probably not be used in chilren.
    • As cells in suspension die potassium is releaed in large quantities and levels of 2,3 DPG fall. Seven day old blood has a K+ of 15-20 m mol/l.
    • Check the date of bleeding and do not accept outdated blood.
    • Stored blood is also low in calcium and supplements may be needed during large or rapid transfusions.
    • Stored blood contains no platelets. In large transfusions it is wise to consider giving platelets in addition.
    • Despite screening, blood transfusion may still transmit hepatitis, HIV and malaria.
    • Blood transfusion should be avoided whenever possible.
    • A low haemoglobin is not per se an indication for transfusion.
    • Normal haemoglobin levels vary with age –

    at birth ± >16.5 g/dl
    1 month ± >14.0 g /dl
    3-6 months ± >11.0 g/dl
    6-24 months ± >12.0 g/dl

    • Do not transfuse a patient the night before theatre. The transfused blood will not efficiently deliver oxygen to the tissues but haematocrit will be increased and flow impaired.
    • When a transfusion is necessary, 10 ml packed cells/kg will increase Hb 2.5-3 g/dl if bleeding is not continuing.
    • In the chronically anaemic child, repeated small transfusions are better than one large transfusion.
    • Blood should only be administered with a filter in-line.
    • Blood should always be rewarmed before transfusion.

    Do not book blood for theatre without consulting the surgeon or senior anaesthetist.

  • CONGENITAL DIAPHRAGMATIC HERNIA

    • This is a congenital abnormality of lung development, giving rise not only to abnormal lungs, but also to anomalies of the heart and a diaphragmatic herniation.
    • Most cases are sporadic but familial, drug and Vitamin A induced causes are reported.
    • 90% of diaphragmatic hernias are Bochdalek types, of these 80% are on the left side.
    • There is a universal 30-50% mortality, newer forms of treatment have not made a significant difference in outcome.
    • Associated anomalies are frequent and should be ruled out prior to surgery.

    PRESENTATION

    • Antenatal diagnosis should be made on an antenatal ultrasound scan and warrants transferring the mother for delivery at the centre where the newborn will be definitively treated.
    • All newborns who develop respiratory distress at any time following delivery should be suspected of having a congenital diaphragmatic hernia.
    • The features of respiratory distress are an increasing ventilation rate, recession and cyanosis.

    MANAGEMENT

    • Such children may require early endotracheal intubation and ventilation.
    • The diagnosis of congenital diaphragmatic hernia is made on chest x-ray (differential diagnosis = cystic adenomatous malformation, Staphylococcal cavitation, eventration, pulmonary sequestration).
    • Nasogastric aspirations and sedation of patients decrease the ventilatory difficulty and stress, which leads to pulmonary hypertension and a reversal to fetal circulation.
    • Once the child is stable and ventilatory pressures are decreasing, the child is ready for surgery.

    TRANSPORT

    • Antenatally is best.
    • Once born in a peripheral hospital, such patients must only be transported with the above management in place and continued during the transfer.
    • Transfers must be by road transport or if flying, in a pressurised cabin only.

    PROGNOSIS

    A poor prognosis is attributed to those patients with :

    • Antenatal diagnosis before 20 weeks.
    • Antenatal diagnosis of hernias containing stomach or liver.
    • Respiratory difficulty before 8 hours.
    • Patients who fit into Bohn criteria C (PaCO2 >40 mmHg, ventilatory index >1000).
  • Inguinal hernias

    • Intermittent swelling of the inguinal region and possibly the scrotum.
    • Swelling increased by increased intra-abdominal pressure (cough, crying and constipation etc).
    • Constant swelling of the inguinal region means herniation is stuck = incarcerated inguinal hernia.
    • Constant swelling with tenderness, inflammation and/or oedema of the scrotum means there is vascular compromise of the herniation (bowel) = strangulated inguinal hernia.

    IMPLICATIONS

    • Bowel in an extra-abdominal position will become obstructed.
    • Incarceration and strangulation imply vascular compromise of the bowel and the testis.

    MANAGEMENT

    Uncomplicated hernias must be given surgical bookings for closure at the soonest opportunity. The younger the patient, the earlier the booking.

    If telephonically referred, advise on reduction of incarcerated hernia (vida infra) and transfer strangulated hernias as soon as possible if resuscitation and transfer protocol has been followed.

    Incarcerated hernias. Treat for :

    • Intestinal obstruction (NG tube + aspiration, IV R/L, nil per os).
    • Herniated bowel oedema (sedate patient, elevate foot of bed).
    • Prepare for theatre, if hernia not reduced after 1 hour of sedation and elevation (ie. unsuccessful conservative treatment).

    Strangulated hernias. Treat for :

    • Intestinal obstruction (as above).
    • Prepare for theatre.
    • Operated as soon as the patient has been resuscitated.

    SURGERY

    • Standard herniotomy incision for all with a wide enough incision to assess the inguinal canal and bowel in cases of complicated herniation.
    • If bowel returns following commencement of anaesthetic, proceed with a standard herniotomy.
    • If bowel remains in sac following exposure, open inguinal canal. If bowel is viable, return it to the abdomen and close peritoneum. If not viable resect this loop of bowel, either via laparotomy or through herniotomy incision.
    • Compromised testes should be given fasciotomy.
  • Jaundice

    • In paediatric surgery jaundice is frequently associated with prematurity, sepsis and starvation.
    • Conjugated hyperbilirubinaemia is a sign of cholestatic or obstructive jaundice and warrants early investigation. An operative cholangiogram is the only early investigation able to distinguish between neonatal hepatitis and biliary atresia.
    • Obstructive (surgical) jaundice in the neonate is a rapidly progressive condition, which becomes inoperable after 3 months. Operative cholangiogram should be done within a week of first seeing the child. 

    PRESENTATION

    • The jaundice varies from lightly to brightly yellow coloured sclera and skin.
    • Stool colour is pale, never green or brown, but may have a yellow tinge from the jaundice-stained mucus of the bowel.
    • The abdomen may vary from a non-distended abdomen with mild hepatomegaly to a tensely distended abdomen with signs of portal hypertension.

    MANAGEMENT

    • Telephonic referrals should be viewed at the next PSOPD clinic, do not delay because “tests” have been booked.
    • On examination of the child, check for sepsis, jaundice and stool colour.
    • Do an urgent liver function test to assess whether there is conjugated hyperbilirubinaemia and check INR.
    • Do an urgent ultrasound examination of the abdomen to exclude a choledochal cyst.
    • If obstructive jaundice is proven, prepare the child for operation.

    SURGERY

    • This requires the child to be well hydrated and biochemically stable with a normal clotting profile.
    • Surgery commences with an operative cholangiogram. Demonstration of an extrabiliary obstruction warrants a hepato-porto-enterostomy.
    • Limiting factors are age, a bleeding diathesis and sepsis.

    SUMMARY

    • If the patient is jaundiced do a PR examination.
    • If stool is pale do an ultrasound.
    • If the ultrasound shows no cysts do an urgent laparotomy.
    • No other diagnostic investigations are necessary.
  • Neonatal transport

    • The principles of neonatal transport are independent of the distance involved and are just as important when transporting a baby within the hospital as between hospitals. It is important to provide a continuum of care which cannot be interrupted during movement of the patient. There is very little judtification for moving babies by air, despite the enthusiasm of the Amblance Service for this mode of transport. Under many circumstances air transport is dangerous. The essentials of transportation are summarised by –

    TWO SIDES –

    Tube: No baby should be transferred without a nasogastric tube in place, functioning and supervised.

    Warmth: Babies can be kept warm by wrapping them in aluminium foil. Incubators are not essential. Remember to wrap the head. Insulation will keep a baby warm but will not warm a cold baby.

    Oxygen: All babies should be transported with supplemental oxygen by head-box or nasal prongs.

    Stabilisation: This is a sine qua non. A stable patient has been rewarmed, has a normal blood sugar, unobstructed breathing, and is appropriately rehydrated. This implies a normal urine outpue (1 ml/kg/hr).

    Intravenous fluids: Drips must be kept running during transport. Drip sites should be accessible and visible. Cannulae should be used, not needles.

    Documentation: All, even the newly-born, have a history. Details of delivery, pregnancy history, perinatal problems are necessary for a management plan to be drawn up.

    Escort: Every baby must be escorted by an appropriate professional. The mother is simply not trained to continue observations, supervise nasogastric tubes and intravenous care.

    Specimens: Including x-rays, must be sent with the patient. If the mother cannot accompany the patient, it is useful to have some maternal clotted blood for x-match.

    • It should be remembered that the sending physician, which may be you, is responsible for the care provided during transportation. The care taken is reflected in the success of the operation and the clinical state of the patient on arrival. It is a public demonstration of your care and commitment.
  • Osteitis

    Cellulitis of the leg does not occur in children

    • Inflammation over the leg is a common presenting symptom, with very frequently a preceding history of minor trauma.
    • All such patients must be regarded as having acute osteomyelitis.
    • Inspection will reveal erythema, swelling and often blistering of the skin.
    • Movement is painful and the patient is frequently prexial.
    • Usually the patient has already been x-rayed in casualty or by the referreding doctor and the x-rays seem to be normal.

    There are no radiological changes in acute osteomyelitis. A normal x-ray is entirely consistent with acute infection in the bone

    • All patients presenting with acute inflammation over the leg will be admitted.
    • Blood cultures and blood count will be taken.
    • Analgesia will be prescribed.
    • Intravenous antibiotics (currently Cefril) will be prescribed.
    • The limb will be elevated to heart height.
    • Temperature will be monitored 4-hourly.
    • Failure to settle implies either resistance to the prescribed antibiotic or the need for drainage.
    • Patients must not be referred to orthopaedic surgery without a consultant seeing the patient.
  • Oesophageal atresia

    GENERAL

    • 87% of children with oesophageal atresia will have an associated tracheo-oesophageal fistula.
    • 50% of children with oesophageal atresia will have associated anomalies.
    • Patients with fistulae are at risk of pulmonary soiling from saliva, feeds and refluxing gastric juice.
    • Many of these children are, additionally, premature.

    DIAGNOSIS

    • Clinical suspicion must follow all premature births and stiff naso-gastric tube should be passed to exclude the diagnosis.
    • Other clinical features include the “frothy” baby, respiratory distress, epigastric distension, or the recognition of an associated anomaly, eg. anorectal malformation.
    • Never perform contrast studies in patients in whom the diagnosis is suspected.
    • Diagnosis is confirmed by a plain chest x-ray with an opaque tube in the upper oesophagus.
    • No other investigation is necessary or desirable.
    • Investigation may be required to define associated abnormalities.

    TRANSPORT

    • The key to success is to prevent pulmonary soiling.
    • Pass a double lumen tube into the upper pouch and provide continuous suction.
    • The baby should be kept warm, IV fluids and supplementary oxygen prescribed.
    • Cimetidine may be given to reduce the acidity of gastric reflux.

    MANAGEMENT

    • Oesophageal atresia is not a surgical emergency. The patient’s condition must be optimised.
    • Continuous upper pouch suction (double lumen tube, 20 cm H20), antibiotics, oxygen, cimetidine, etc. must be continued.
    • Associated anomalies must be sought and their significance assessed.
    • The patient should not leave thenursery for studies. Order BSU’s.
    • Chromosomal lesions are common and may preclude operative treatment.
    • The anaesthetic team should be consulted early.
    • Facilities for post-operative ventilation must be secured.
  • Pyloric stenosis

    • Pyloric stenosis is a biochemical emergency. It is not a surgical emergency.
    • The cardinal symptom is bile-free vomiting. Many patients also have diarrhoea.
    • Signs include visible peristalsis in the epigastrium and a palpable “tumour”.

    INVESTIGATIONS

    • Ultrasound – look for thickness of pyloric muscle, length of channel.
    • Electrolytes – usually a severe hyponatraemic, hypochloraemic alkalosis.
    • Barium meal should rarely be necessary. Only if consultant radiologist concurs.
    • Urea and electrolytes.

    MANAGEMENT

    • Establish IV line: If impossible use initial intraosseous infusion.
    • Catheterise.
    • Insert nasogastric tube.
    • Start IV 5% Dextrose Saline.
    • Give initial bolus of 20 ml/kg and repeat prn.
    • Check urine is passed. Rate required 1 ml/kg/hr.
    • Check pH of urine.
    • When urine has been seen to be passed, add KCl 20 meq/L.
    • Titrate rate of infusion against urine output.
    • Recheck urea and electrolytes every 4-6 hours.
    • Plan surgery when bicarbonate is less than 30 m mol/L.

    OPERATION

    • Only performed when patient is biochemically normal.
    • Ramsted’s pyloromyotomy.

    POST-OPERATIVE CARE

    • This depends upon the operating surgeon and the severity of illness.
    • Discuss with the surgeon.
    • Ensure clear instructions are left with the nursing staff.

    SUGGESTED READING

    • Infantile hypertrophic pyloric stenosis in the Third World environment. Emmink B, Hadley GP, Wiersma R. S Afr Med J 1992:82;168-170.
    • Lesions of the stomach. Dudgeon DL. In: Ashcraft & Holder. Paediatric Surgery.
  • Rabies

    DEFINITION

    • An acute viral illness of central nervous system affecting most mammals causing a fatal encephalomyelitis.
    • Transmission is usually via contamination with virus-laden saliva of bites from rabid animals.

    EPIDEMIOLOGY

    Rabies exists in 2 forms – 

    • urban, propagated by non-immunised domestic dogs and cats.
    • sylvatic form propagated by rats, monkeys, mongooses and meercats.

    Human-to-human transmission has been reported via organ transplantation and human bites.

    25,000-30,000 deaths are reported world-wide annually.

    PATHOGENESIS

    • The virus spreads centripetally up the nerve to the central nervous system. Viraemia does not play a role.
    • Virus replicates in the grey matter and spreads centrifugally along the autonomic nerves to reach other tissues – salivary glands, adrenal medulla, kidneys, lung, liver, skeletal muscle, skin and heart.
    • Incubation period is variable – 10 days to 1 year (or longer) (mean 1-2 months). The time period varies with amount of virus introduced and thesite of the bite.
    • Two clinical forms are identified –

    Paralytic rabies – dumb rabies
    Fulminant encephalitis – furious rabies most common

    MANIFESTATIONS

    • There are 4 clinical stages –

    Prodrome – headache, nausea, malaise, vomiting and often paraesthesia at the 
    site of inoculation.

    Acute encephalitis – marked by excessive motor activity, excitation, agitation, hallucinations, opistotonic posturing, seizures, lucid periods set shorter as disease progresses.
    Profound dysfunction of brainstem centres producing the classical features of rabies encephalitis, cranial nerve palsies, difficult deglutition and increased slivation.
    Death (recovery rare).

    DIAGNOSIS

    • Routine full blood count and chemistry are usually normal.
    • Isolation of virus from infection secretions, saliva, CSF, tissue.
    • Serological demonstration of acute infection.
    • Demonstration of viral antigen in infected tissue.
    • Fluorescent antibody staining of coroneal impression scars, skin biopsies.

    DIFFERENTIAL DIAGNOSIS

    • Other viral encephalitis – Guillian Barre Syndrome.
    • Allergic encephalomyelitis to rabies vaccine.
    • Psychosis and behavioural disorder.
    • Typhoid and tetanus.

    POST-EXPOSURE PROPHYLAXIS AND TREATMENT

    • Prophylaxis – any person who sustains a broken skin injury (category 3 exposure) living in an endemic area when the animal is not identified must be given active vaccination and passive immunisation –

    Category 1 – Discuss with officer-on-duty
    Category 2 – Rabies help line

    No prophylaxis –

    • Known animal – vaccination record produced.
    • Animal captured, remains well after 10 days of observation

    Management –

    Local wound therapy

    • Generous scrubbing of wound with soap then irrigation with water
    • Antitetanus toxoid
    • Penicillin

    Passive immunization

    • 20 IU/kg RIG or 40 IU/kg equine RIG (Rabigam 150 IU/ml)
    • ?into wound, rest intragluteus muscle

    Active vaccination 

    • Anti-rabies vaccine is also given at the same time
    • HDVC – human deploid cell vaccine (Vero-Rab)
    • Dose 0.5 cc into deltoid muscle x5 doses

  • Snake bite

    • Poisonous snakes have fangs. Non-poisonous snakes do not.
    • Paired puncture or scratch wounds are indicative of a bite by a venomous snake.
    • All snakes have cytotoxic, neurotoxic and haematotoxic components in their venom. The proportions of these components varies between species.
    • Antivenom is a horse serum product and potentially lethal.
    • Tourniquets cause more trouble than snakes. Always note the presence, site and duration of tourniquets.

    MANAGEMENT

    • All children bitten by venomous snakes should be admitted, whether ornot there is objective evidence.
    • All touniquets should be removed.
    • A TEG should be done: abnormality suggests envenomation.
    • Tetanus prophylaxis is given.
    • Coagulation screen is requested, check K+.
    • Local status at wound site is documented.
    • Antibiotics are given.
    • The limb is kept at heart height.

    MONITOR

    • Look for a developing compartment syndrome. Measure ICP if in doubt.
    • Look for extension of oedema/inflammation/necrosis.
    • Fluid balance where limb swelling is progressive.

    ANTIVENOM

    Consider antivenom if clinically deteriorating.

    • Give test dose in conjunction with Phenergan and Hydrocortisone.
    • Dose determined by size of innoculum not size of patient. Start with 2 amps.
    • Give IVI.

    Consider antivenom if neurological signs develop.

    Ventilate early

  • Sjambok injuries

    • The term myorenal syndrome is preferred to the term crush injury or crush syndrome.
    • The syndrome occurs in sjambok, deep burns and crush injuries.
    • The syndrome is dependent on the toxic effect of the iron component of the haemoglobin/myoglobin molecule on the renal tubule.
    • Muscle lysis results in superoxide and hydroxyl radical formation and hyperkalaemia develops rapidly.
    • Both haemoglobin and myoglobin are precipitated in an acid urine (pH <5.6).
    • Myoglobinuria is not always present but a poor prognostic feature (incidence of renal failure 50% when present).
    • The syndrome is dependent on the toxic effect of the iron component of the haemoglobin/myoglobin molecule on the renal tubule.
    • Severity grading is dependent on the presence of urinary pigment, venous bicarbonate, serum electrolytes and renal function.


    PRESENTATION

    • Good venous access, volume replacement, analgesia.
    • Placement of a urinary catheter, urine specimen for dipstix and laboratory assessment for presence of pigment.
    • Full blood count, urea and electrolytes, venous blood gas assessment.

    TREATMENT PROTOCOL ACCORDING TO SEVERITY

    • Use of CVP is confined to patients over 7 years and older and should be discussed with the on-duty consultant before insertion.
    • Injuries graded as massive and major should be discussed with the ICU registrar/consultant on duty in order to secure an ICU bed if possible.