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LEPROSY
Leprosy is quite common in children, but rare under 2 years of age. It is often unrecognised, and 75% of cases regress spontaneously without treatment. Childhood leprosy usually responds rapidly to treatment and reactions are rare. Almost all cases in children are indeterminate or tuberculoid leprosy. Leprosy bacilli are intracellular organisms. Host resistance therefore depends on cellular immunity and not on antibodies. Antibodies are made however, and are responsible for lepra reactions. Prolonged contact with a patient is NOT needed to contract leprosy. The incubation period is usually about 3 years (range 3 months to 40 years).

TYPES OF LEPROSY
Indeterminate
Indeterminate lesions are all flat lesions not showing typical tuberculoid features (clear-cut lesion with sensory loss of 2 or 3 modalities or superficial nerve enlargement). When M leprae first invades the body, the small slightly pale skin patch of indeterminate leprosy develops. Usually cellular immunity destroys all the organisms and the patch disappears spontaneously. But the infection may develop into determinate leprosy: tuberculoid, borderline or lepromatous.

Tuberculoid (TT) - flat or raised

1. 2. A clear-cut skin lesion with well-defined limits, PLUS Loss of at least two of temperature, pain or light touch, OR enlargement of the superficial nerves supplying the area.

Tuberculoid leprosy occurs when cellular immunity is strong, but not strong enough to kill off all the leprosy bacilli. The intense inflammatory reaction prevents bacilli growing and spreading, but rapidly causes swelling, and damages the skin and nerves in which M leprae lives. There are usually one or two hypopigmented lesions with clearly defined edges, small or large. The lesions may be flat or slightly raised, especially round the edges where the bacilli are active. Healing takes place from the centre, which may be flat and returning to normal colour. Skin smears are negative. There is a rapid response to treatment. Reactions are rare. The lepromin skin test is strongly positive.

Borderline (BB) - rare in children

In borderline leprosy, the host resistance is between that of the tuberculoid and lepromatous forms. Some leprosy bacilli are killed, but some spread from skin and nerves to bones, muscles, testes, etc. There are multiple lesions of variable size and shape that may be flat or raised. The lesions are usually shiny and red, the edges slope upwards and the centres are flat. The skin lesions, but not the nerve lesions, are usually the same on each side of the body. Small firm nodules are common on the face. Skin smears are positive from the lesions, but negative from normal skin.

Lepromatous (LL) - very rare in children

Cellular immunity is poor. The bacilli multiply rapidly and spread to the eye, the mucous membranes of the nose, bones, muscles and testes. Lesions are widespread and symmetrical. There is little inflammatory reaction and local tissue damage at first, but later the large number of bacilli causes tissue necrosis and destruction.

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SKIN AND NERVE LESIONS

Skin lesions
1. 2. 3. 4. 5. Hypopigmented macules. Erythematous or hyperpigmented macules. Infiltrated (raised) patches: usually erythematous or hyperpigmented. Desquamation comes with resolution. Lichenoid lesions: pin-head papules on normal skin, macules or infiltrated plaques. Papulonodular lesions: red, firm rounded elevations. Lesions of the nose, mouth and eyes are rare in children.

35% of skin lesions are single; 50% are found on the lower limb, 30% on the arm and forearm and 10% on the trunk.

Nerve involvement
1. 2. 3. The ulna, external popliteal and great auricular nerves are those that are most often enlarged. Loss of sensation occurs. Temperature discrimination is usually affected first, followed by pain then light touch. There is often pain or tenderness of the nerve trunk. Paralysis and deformities are rare in children.

DIAGNOSIS
1. Skin lesions with or without neural involvement Diagnosis is made by: a. the appearance of the skin b. sensory loss in the skin lesions c. nerve enlargement, pain or tenderness. 2. Neural involvement only (an enlarged or tender peripheral nerve). Definite sensory loss (without a history of trauma) is diagnostic of leprosy. Pain or tenderness of a nerve trunk (without a history of trauma) is diagnostic of leprosy, especially if there is a history of contact. An enlarged nerve unilaterally (without a history of trauma) is suggestive of leprosy. If there is a history of contact, start treatment. If there is no history of contact, review every 6 months.

Skin smear
Skin smears are negative in over 90% of cases in children. Infiltrated lesions are occasionally positive. Take smears from the childs lesions, ears and arms. Swab the skin with spirit, then pinch it up between finger and thumb to stop the blood flowing. Make a straight cut 2 mm deep and 5 mm long with a scalpel, then turn the blade quickly and scrape it inside the cut to get some juice (NOT blood) on the blade. Smear the juice on a clean glass slide labelled with the patients name and the date. Juice from 6 or 8 sites can be smeared on one slide. Let the slide dry, then warm the back of the slide briefly over a flame until it is just too hot to hold on the back of your hand. Six or more organisms indicate lepromatous leprosy, 1-5 organisms indicate borderline leprosy, and no organisms are seen in tuberculoid leprosy. The morphological index (MI) is the percentage of solid staining organisms on the slide. The MI falls from 25-75% at diagnosis to 0% after about 6 months of uninterrupted treatment. A stationary or rising MI indicates that the patient is not taking the drugs or that resistance has developed.

Biopsy
A FULL THICKNESS skin biopsy (down to fat) is sometimes helpful in diagnosis, and is very useful for correct classification. Biopsy across the edge of a suspected lesion. Fix the biopsy in FFA (40%

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formaldehyde 10 ml, mercuric chloride 2 g, glacial acetic acid 3 ml, water to 100 ml) for 2 hours. Wash in water, then store in 70% alcohol. If FFA is not available, use normal saline.

The Lepromin (Mitsuda) Intradermal Test

This is not used in Papua New Guinea.

MANAGEMENT
1. Do a skin biopsy for suspected tuberculoid or borderline leprosy (which is most cases in children). Do a skin smear for suspected lepromatous or borderline leprosy. If the diagnosis is certain, start treatment as soon as you have taken the biopsy or smear. Notify the Leprosy Control Officer of the case. Make sure all the necessary paper work is completed. The family must understand: a. that leprosy can be cured, PROVIDING treatment is taken regularly b. how much medicine should be given each dose c. how often medicine should be given d. the importance of reporting quickly if nerve pain, anaesthesia, swelling or fever develop e. that any change of address must be notified and treatment continued. Examine all contacts (family and friends, schoolmates, those who live in the same house) for leprosy.

2. 3.

4.

The treatment of leprosy was reviewed in Lancet 2:487,1988. Most children with leprosy have paucibacillary disease. They can be treated for just 6 months with daily unsupervised dapsone and monthly supervised rifampicin 15 mg/kg (maximum 600 mg). All treatment then stops and the patient remains under observation for 2 years. Patients with multibacillary disease should continue with drug treatment until skin smears are negative (a minimum of 2 years). They are treated with daily unsupervised dapsone and clofazimine, and monthly supervised rifampicin 15 mg/kg (maximum 600 mg) and clofazimine 1 mg/kg. After treatment, observation should continue for 5 years. Dapsone daily dose 3-19 kg: 25 mg (half tab) 20-29 kg: 50 mg (1 tab) 30-49 kg: 75 mg (1.5 tab) 50 kg or more: 100 mg (2 tab) Clofazimine dose Less than 10 kg: 50 mg (1 tab) every second day 10 kg or more: 50 mg (1 tab) every day The treatment of lepromatous leprosy has improved greatly with the advent of combination chemotherapy - monotherapy with dapsone should no longer be used. Patients with lepromatous leprosy have deficient immunity to leprosy, and are very dependent on drug therapy. The treatment of lepromatous leprosy has been modified because of: a high incidence of resistance after 5 to 10 years treatment of lepromatous leprosy with dapsone alone (Br Med J 2:914-915,1977) the persistence of viable dapsone-sensitive organisms despite full dapsone therapy. In decreasing order of potency, bacteriocidal leprosy drugs are rifampicin, clofazimine, ethionamide and dapsone. Rifampicin is the most potent antileprosy drug. Thiacetazone, thiambutosine (DPT) and streptomycin are only bacteriostatic.

LEPRA REACTIONS
There are two forms of reaction. Both are rare in children.

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Down-grading and reversal reactions

The reaction is due to a rapid change in the host-parasite relationship. It is usually seen in tuberculoid and borderline cases. Skin lesions increase in number, become inflammed and may ulcerate. Peripheral nerves may become tender and swollen, then acute paralysis develops. There may be oedema of the hands and feet. In a down-grading reaction, the host resistance becomes weaker. New lesions appear. The lepromin reaction becomes weaker, and tuberculoid lesions change to borderline or lepromatous. In a reversal reaction, the host resistance becomes stronger (this often occurs with treatment). Serial biopsies show fewer bacilli and increased lymphocytes, epithelial cells and giant cells. Borderline lesions change towards tuberculoid, and the lepromin reaction tends to be stronger.

Erythema nodosum leprosum (ENL)

This is caused by an antibody reacting with antigen from disintegrating bacilli. It usually occurs in lepromatous or borderline leprosy. There is little change in the leprosy lesions. Crops of tender red lumps appear every 5-7 days. There is often associated fever, oedema, neuralgia, arthralgia, acute paralysis, lymphadenitis, iritis, irido-cyclitis, epididymo-orchitis or proteinuria.

THE TREATMENT OF LEPRA REACTIONS

Do NOT reduce the dose of dapsone. Thalidomide is rarely used (it is effective, but teratogenic).

A mild reaction (especially indeterminate or tuberculoid leprosy):

1. 2. 3. Advise REST - bed rest, splinting or sling. Give ASPIRIN every 6 hours Give CHLOROQUINE daily for 3 weeks (tablets 150 mg base) Weight 6-9 kg: 10-19 kg: 20-29 kg: 30-39 kg: 40-49 kg: 50 kg or more: 4. 1st week tab 1 tab 1 tab 2 tab 2 tab 3 tab 2nd week tab tab 1 tab 1 tab 1 tab 2 tab 3rd week tab tab tab tab tab 1 tab

If there is no improvement, give CLOFAZIMINE 100 mg a day (every second day if under 10 kg), as well as dapsone. Up to 10 mg/kg of clofazimine can be given each day.

A severe reaction with acute neuritis, iritis or iridocyclitis

1. Give PREDNISOLONE 1 mg/kg/day in 3 divided doses. Reduce the dose gradually when the reaction is controlled. Prednisolone should ONLY be used for acute neuritis or iritis, and NOT for ENL alone. Give CLOFAZIMINE 10 mg/kg/day as well as dapsone. When the reaction is controlled, reduce the dose gradually to 100 mg a day (or every second day in a child weighing less than 10 kg). When all signs of the reaction have gone, gradually withdraw clofazimine completely (leaving the patient still on maintenance dapsone). Treat iridocyclitis with atropine drops and 1% hydrocortisone drops every 6 hours.

2.

3.

DAPSONE RESISTANCE
This is suggested by: no visible improvement after regular treatment for one year, or

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no reduction in the skin smear morphological index after regular treatment for one year, or reappearance of solid rods on smears (relapse) despite regular treatment. Take a skin smear each month for 3 months. Give supervised dapsone. If there is no improvement after 3 months: a. b. arrange with the TB/Leprosy Section of the Health Department for a biopsy to be sent for culture and sensitivities (it may have to go overseas). stop dapsone and give rifampicin 10 mg/kg/day for 12 weeks, and clofazimine 100 mg daily indefinitely.

1. 2. 3.

REFERENCES
Bagshawe A. Trans Roy Soc Trop Med Hyg 83:121-7,1989. Epidemology of leprosy. Browne SG. Leprosy in children. Geneva: WHO, 1976. Editorial. Lancet 2:487-488,1988. Chemotherapy of leprosy. Jopling WH. Handbook of leprosy. 3rd ed. London: Heinemann, 1984.

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LIMP
See also Arthritis, pp.35-38. NEVER IGNORE A LIMP, OR PAIN IN THE HIP OR LEG. IT MUST ALWAYS BE REGARDED AS PATHOLOGICAL.

ACUTE LIMP
Pain in the knee is often due to disease of the hip. X-ray the appropriate area. Consider pyogenic arthritis, osteomyelitis, cellulitis, TB, fracture, Perthes disease, scurvy, rheumatic fever, malignancy and irritable hip. Any febrile child with a painful hip should get chlorampenicol and be referred for aspiration or exploration of the hip.

ACUTE AND CHRONIC LIMP

All ages: 1-2 years: 2-5 years: 5-10 years trauma, osteomyelitis, pyomositis, TB or pyogenic arthritis malignant disease (leukaemia, neuroblastoma, sarcoma) cerebral palsy, CDH, spiral crack fracture tibia monoarticular synovitis, mild spastic hemiplegia irritable hip (aspirate to exclude suppuration if the child is febrile), Perthes disease (osteochondritis: 80% of cases occur in boys, 10% are bilateral, and X-ray shows flattening of the femoral head with a normal acetabulum; cf TB in which the acetabulum in involved). Refer the child to a surgeon slipped upper femoral epiphysis - take a lateral x-ray of both hips and compare the 2 sides.

10-15 years:

Note: The diagnosis of TB of the hip is often missed until irreversible damage has occurred. Always consider the possibility of TB in all age groups.

A LIMP AFTER AN INJECTION

It is quite common to see a child who has had an injection in the buttock for an acute febrile illness, and subsequently developed a weak leg on the side of the injection. Is the weakness due to damage to the sciatic nerve from the injection; or was the febrile illness actually polio, which caused the flaccid paralysis? (This is unlikely now - but still a possibility.) Ask, did the paralysis come on immediately after the injection (sciatic nerve damage), or did it come on several days later (polio)? Polio usually causes wasting of the quadriceps and buttock as well as the calf muscles, the knee jerk is reduced on the affected side, there is no sensory loss, and the child may not be able to walk on the affected leg at all. Sciatic nerve damage does not cause wasting of the quadriceps or buttock, the knee jerks are equal, there is anaesthesia to light touch and pinprick on the lateral side of the foot, and the child can walk - but with the characteristic gait of a patient with foot-drop. An injection given to a child with early poliomyelitis may precipitate paralysis in that limb. This is one of a number of good reasons for avoiding the indiscriminate use of injections in patients with nonspecific febrile illness.

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LIVER BIOPSY
This is a dangerous procedure, and should only be performed with adequate indications. These are very rare in childhood. The best results are obtained by using a spring-loaded modified Trucut needle with ultrasound guidance. LIVER BIOPSY MUST ONLY BE PERFORMED BY EXPERIENCED STAFF. AND IT SHOULD BE DONE UNDER ULTRASOUND GUIDANCE IF POSSIBLE. Do a prothrombin time. If this is 2 seconds or more longer than the control, give vitamin K, 1.0 mg/kg IM and wait 24 hours. Do not do the biopsy if the prothrombin time is still prolonged. The platelet count should be over 100,000. Crossmatch one unit of blood. Drain any ascites before doing a liver biopsy. Ensure that your biopsy needle is SHARP. Over 4 cm liver: use the subcostal route Under 4 cm liver: infant:

older child:

give IV diazepam just before the procedure, and have an assistant compress the chest at the end of expiration to arrest respiration. Use the intercostal route use the intercostal route under relaxant general anaesthesia, unless the child is old enough to co-operate fully (about 12 years or more).

1. 2. 3. 4.

The child lies supine with the right side at the edge of the bed. The right arm is held above the head. Scrub your hands and put on sterile gloves. Subcostal: puncture below and to the right of the xiphoid process in the midclavicular line. Aim towards the left shoulder. Intercostal: puncture at the point of maximum dullness (8-l0th intercostal space) in the mid axillary line. This technique involves puncture of the pleural cavity and must ONLY be used if full expiration can be maintained throughout the procedure. Clean the skin with iodine in a 10 cm radius around the proposed puncture site. Inject 2% plain lignocaine through a 23 gauge needle into skin, subcutaneous tissue, pleura, peritoneum and liver capsule. Make a nick in the skin with a scalpel blade.

5.

MENGHINI ONE SECOND NEEDLE BIOPSY

1. Place the nail in the shaft of the needle with the flat end away from the point. Attach a 5 ml syringe to the needle. Draw up 2 ml of sterile saline into the syringe.

Longitudinal section of the Menghini liver biopsy needle. Note the nail in the shaft of the needle.

2. 3.

Push the needle perpendicularly down to, but not through, the intercostal muscle. Inject 1 ml of saline to clear the needle of any fragments. Commence and maintain firm aspiration. With the chest maintained in expiration, QUICKLY push the needle into the liver, then remove it. Do not rotate the needle. This part of the procedure should take only one second.

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4.

Put the needle into a bottle of 10% formalin, and gently inject a little of the remaining saline from the syringe to free the biopsy.

TRUCUT NEEDLE BIOPSY

This method is more dangerous than the Menghini technique (particularly with an uncooperative patient), but it has a higher success rate and usually yields a better biopsy specimen. It is an improved version of the Vim-Silverman needle. 1. 2. Push the needle into the liver with the chest held in expiration. While holding the main shaft of the needle still, rapidly advance the cutting blade so that it excises the fragment of liver that is resting in the slot on the needle (or release the trigger if a spring-loaded Trucut biopsy gun is being used). Remove the needle, pull back the cutting blade, and carefully remove the biopsy into a bottle of 10% formalin.

3.

VIM-SILVERMAN NEEDLE BIOPSY

This method is more dangerous than the Menghini technique, but has a higher success rate with a fibrotic liver. Have the chest held firmly in expiration.

1. 2. 3. 4.

Introduce the trocar and cannula into the liver. Remove the trocar. Holding the cannula firmly in one hand, push the split needle in rapidly until its head hits the head of the cannula. Hold the split needle stationary and advance the cannula until its tip is level with that of the longer split needle inside the liver. Rotate the cannula and split needle. Remove the split needle and cannula together. The biopsy is held between the two halves of the split needle, and can be freed under 10% formalin.

OBSERVATION
The main risk from liver biopsy is haemorrhage. The pulse rate must be taken hourly for 24 hours after the biopsy. You must be notified if it rises. Be prepared to give the crossmatched blood. There may be some right chest and shoulder tip pain.

REFERENCE
Sherlock S. Diseases of the liver and biliary system. Blackwell.

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LUMBAR PUNCTURE
1. Have the patient held in the CORRECT position by an assistant: a. lie the patient on the side with the back and hips flexed so that the knees are bent up onto the abdomen b. the assistant should hold the child by the shoulders and NOT by the head. Strong flexion of the neck in a small child can cause respiratory obstruction and death c. the childs back should be at the edge of the bed d. the childs back must be vertical - very often the child is rolled slightly onto the front, and this makes your job much more difficult. Note: Ensuring that the child is firmly held in the correct position is essential to success. 2. 3. 4. Scrub your hands. Use sterile gloves, if available. Clean the whole of the lower back and the tips of your left index and middle fingers (that you will use to feel the childs back) with iodine. Find the L3-4 or L4-5 space, at or just above a line joining the two iliac crests. A common mistake is to choose too low a space in small children. Do NOT inject local anaesthetic in babies or children under 20 kg. Use a sterile 21 or 23 gauge disposable needle. Use a 21 gauge scalp vein needle with all but 2 cm of the tubing cut off for babies. DO NOT TOUCH THE SHAFT OF THE NEEDLE WITH YOUR FINGERS. Keep the needle EXACTLY HORIZONTAL and SLOWLY push it in, aiming towards the umbilicus. Push the needle in, in VERY SMALL stages of about 1 mm each. Wait AT LEAST 15 seconds between each stage, and turn the needle a quarter of a turn each way on its long axis (in case the end is blocked). It is easy to push the needle in too far in small children. Go in SLOWLY, and WAIT a long time between each time you push it in. The CSF often comes out very slowly in small children, so you can be in the subarachnoid space without knowing it if you do not wait long enough. Take 1 ml of CSF into each of two labelled bottles. Send the CSF for microscopy, culture, protein and sugar. Laboratory examination of CSF is dealt with on p.70. Take out the needle, lie the child on his front, clean off the iodine, and stick on a small dry swab with a piece of Elastoplast.

5.

6.

7.

8. 9.

10. If you get a traumatic tap, treat the child for meningitis and repeat the lumbar puncture even more carefully 2 days later.

Common causes for a failed or traumatic LP are: the child was not held correctly (it is YOUR job to see this is done properly) the puncture was made too low the needle was not in the exact midline of the spine and not kept exactly horizontal the needle was pushed in too fast and too far without waiting long enough between each stage.

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LUNG ASPIRATION
Lung aspiration is not a routine procedure. However, properly performed, it is comparatively safe: death is uncommon from the procedure, pneumothorax requiring drainage occurs approximately once in every 400 aspirations, and transient haemoptysis once in every 130 aspirations. When reliable bacteriological services are available, lung aspiration is a justifiable diagnostic procedure in children with very severe pneumonia not responding to standard antibiotic therapy. It may also be useful in the diagnosis of pulmonary tuberculosis 1. Take a chest x-ray with AP and right lateral views (or left lateral if the signs are on the left). Aspirate ONLY if there are chest x-ray changes or clear clinical localising signs (crepitations, bronchial breathing or dullness). Consider the lung as upper, middle or lower zones. Aspirate the affected zone from either the front or the back (but not from the side), depending on the lateral x-ray and whether there are more crepitations at the front or back. Aspirate ONLY in the dark areas of the diagram:

2.

3.

4. 5. 6. 7. 8. 9.

Have oxygen and a chest drain available. Have an assistant hold the child sitting up with hands over the head. Clean the skin with iodine. Use a 23 gauge needle on a 10 ml syringe. Draw up 1 ml of sterile saline or culture medium. Have another assistant grasp the childs chest FIRMLY in expiration just before you aspirate. It is most important that the child be held firmly in expiration, because the needle may tear the pleura if the child breathes while the needle is in the lung.

10. Push the needle quickly into the chest to the required depth. Go just above the rib. 11. Inject 0.5 ml saline into the lung. With the needle in the same position, exert maximal suction. Then slowly withdraw the needle over 2 or 3 seconds while still exerting strong suction. 12. Inoculate the saline in the syringe onto the appropriate culture media. 13. Observe the child closely for the next hour for signs of pneumothorax (increasing tachypnoea and cyanosis). There may be transient haemoptysis. 14. Take an AP chest x-ray later in the day or next day to check for pneumothorax.

REFERENCES
Shann F. Lancet 2:537-541,1984. Aetiology of pneumonia. Silverman M. Arch Dis Child 52:925-931,1977. Aetiology of pneumonia. Weech AA. Am J Dis Child 122:277,1971. Lung puncture.

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