A surgeon should have three diverse properties in his person. That is to saie, a harte as the harte of a lyon. His eyes like the eyes of a hawke, and the handes of a woman.
JOHN HALLE (1529–68)
An imperative task for the GP is not only to diagnose surgical conditions in infancy and children as early as possible, but to be aware of the degree of urgency and the optimal times for intervention. In many instances the emphasis should be placed on a non-surgical solution using natural resolution with time and simple ‘tricks of the trade’.
The neonate’s head may become distorted because of the position in utero or after the passage through the birth canal. The head shape can recover to a normal shape within about 8 weeks following birth. If the abnormal shape persists consider deformational plagiocephaly or craniostenosis.
Plagiocephaly (flat head sydrome)
This is asymmetry of the skull with a normal head circumference. The shape can be likened to a tilted parallelogram (see FIG. 92.1); it is the most common cause of an abnormal head shape. On the side with the flat frontal area, the ear and the parietal eminence sit more posteriorly. It affects 1 in 5 infants and is either congenital or acquired and often results from the infant sleeping in one position, usually on their back. There is usually no impairment of cerebral development or intellect. If the sutures are ridged or the sleeping position causation is ruled out, a skull X-ray should be performed. Management involves initially changing the side to which the child usually faces for sleeping, then regularly changing sides and encouraging time in the prone position while awake. If not responsive, a cranial remodelling helmet can be tried—best from 4 to 8 months.1
Plagiocephaly, congenital or acquired. The long axis is deflected from the saggital plane. In this case the right ear is more posterior.
This is premature fusion of one or more sutures of the cranial vault and base, which act as lines of growth. The abnormality of head shape depends on the sutures involved. The diagnosis is confirmed by radiography. Prompt referral to a paediatric craniofacial surgeon is necessary as planning for possible complex surgery, best at 5 to 10 months, is required.
This condition, which is due to an imbalance between the production and absorption of CSF, usually caused by obstruction to circulation, requires early referral for diversion of ventricular fluid. Prognosis is generally good with early intervention and regular supervision.
Macrocephaly and microcephaly
Macrocephaly and microcephaly are defined as a head circumference greater than the 97th percentile and less than the 3rd percentile respectively. Infants whose head circumference measurements cross these percentile lines require expert assessment and investigation. It is appropriate to undertake regular head circumference measurements in the early childhood years.
EARS, NOSE, FACE AND ORAL CAVITY
The ears are almost adult size and firmness by 5 to 6 years of age but the ear cartilage is not strong enough to cope with surgery under 3 years. For this reason, and because it is best to correct the problem when the child is in a position to support a decision to operate, the optimal time for surgical correction is after 5 to 6 years. It may be possible to correct an ear deformity by moulding the ear with tape or splinting within the first 6 months of life.1
It is best to refer any facial deformity as soon as it is detected.
This dermoid cyst, which has a readily identifiable constant position, lies in the outer aspect of the eyebrow. It is noticed in infancy as it progressively enlarges. Excision is advisable, but check with ultrasound examination because of the possibility of an intracranial extension.
Cleft lip and cleft palate
Congenital clefts of the lip and palate occur in approximately 1:600 of all births. It is very important that the simplest and least obvious form should be recognised in time for adequate repair. This is the submucus cleft, frequently not recognised in infancy because the palate appears to be intact.2 The submucus cleft can be diagnosed on close inspection as the uvula is bifid and there is a deep groove in the midline of the palate covered only by mucous membrane. The ideal age for repair of the cleft lip is under 3 months of age. Secondary surgery can then be performed at various ages. The repair of the palate, which requires preliminary diagnostic ultrasound, is best performed before the child begins to speak. The optimal time is 6 to 12 months of age.
Rhinoplasty is best deferred to late adolescence. If performed early there is a higher incidence of secondary surgery.
Choanal atresia may be unilateral, leading to delayed diagnosis, or bilateral, where there is no instinctive reaction to breathe through the mouth, leading to asphyxia. Since the obstruction is usually by a very thin membrane one side can be perforated with a urethral sound as an emergency procedure.
Septoplasty can be considered if the problem is symptomatic.
Tongue tie (ankyloglossia)
The ideal time to release the ‘tie’ is in infancy, under 4 months.3 As the frenulum is thin and avascular, simple frenulotomy by snipping with sterile scissors (with care) is advisable (see FIG. 92.2). Otherwise surgery should be left until after 2 years of age. The conditions may not be noticed until later in life. A useful guideline is a strong family history of speech problems corrected by tongue tie surgery.
Tongue tie release in infant
This common condition can get recurrently infected with pus discharge from a small opening immediately anterior to the ear at the level of the meatus in front of the upper crus of the helix. It also causes cosmetic problems. It is not a branchial sinus. It can be associated with kidney abnormalities. Refer when diagnosed for surgical excision, although it can be left alone if it is causing no problems.
This is a rare condition and is located inferior to the external auditory meatus or anterior to the sternomastoid muscle. The opening may discharge mucopus. A skin tag or cartilage remnant may be present. Refer when diagnosed for excision.
A squint is rarely obvious in the first weeks of life, but tends to show up when the baby learns to use the eyes, from about 2 weeks to 3 or 4 months of age. However, it may appear late, even as an adult. Vision, which is present at birth, continues to develop until 7–8 years of age.
Constant or true squint is one that is permanent—always present.
Latent squint is one that only appears under stressful conditions such as fatigue.
Transient squint is one that is noticeable for short periods and then the eye appears normal.
Alternating squint is one that changes between the eyes so the child can use either eye to fix vision.
Pseudosquint is not a true squint but only appears to be one because of the shape of the eyelids, i.e. broad epicanthic folds.
A useful way to differentiate a true squint from a pseudosquint is to observe the position of the light in the eyes (corneal reflections) when a torch is shone into them from about 40 cm away. This light reflex will be in exactly the same position in both eyes in the pseudosquint but in different spots with the true squint.
If one eye is ‘lazy’ (that is, not being used), it is standard practice to wear a patch (maybe on glasses) over the good eye for long periods in order to use the inactive eye and have both eyes eventually capable of vision.
The two serious squints are the constant and alternating ones, which require early referral. Transient squint and latent squint usually are not a problem.
Always refer children with strabismus (squint) when first seen to exclude ocular pathology such as retinoblastoma, congenital cataract and glaucoma, which would require emergency surgery.
Children with strabismus (even if the ocular examination is normal) need specialist management because the deviating eye will become amblyopic (a lazy eye with reduced vision, i.e. ‘blind’, if not functioning by 7 years of age). The younger the child, the easier it is to treat amblyopia; it may be irreversible if first detected later than school age. Surgical correction of a true squint is preferred at 1–2 years of age.
Blocked nasolacrimal duct
hard painless lump (2–3 cm long) within sternomastoid muscle
tight and shortened sternomastoid muscle
usually not observed at birth
appears at 20–30 days of age
associated torticollis—head turned away from but tilted towards the tumour
restricted head rotation to side of tumour
Most tumours resolve spontaneously within 1 year. The mother or baby’s carer should be reassured and the child referred to a physiotherapist early. The mother or carer should frequently gently massage the lump, rotate the head to the side of the lesion and then side-flex (stretch) away from that side. Repeat several times twice daily. Encourage the baby to look towards the affected side. If surgery for a persistent fibrotic shortened muscle is required it is best before 12 months.
Older children can present with torticollis and a tight, short fibrous sternomastoid muscle. It is associated with rotation of the head to the affected side, hemihypoplasia of the face and a wasted ipsilateral trapezius muscle. It requires surgical repair.
This is the most common childhood midline neck swelling. It moves with swallowing and tongue protrusion. It is prone to infection, including abscess formation. The cyst and its tract are best excised before it becomes infected.
Lymphatic malformation/lymphangioma/cystic hygroma
These usually present as soft cystic tumours of the neck, face or oral cavity. They resemble clusters of vesicles and are often poorly localised. Some have visible red dots due to haemangiomatous inclusions. If located in the floor of the mouth or peripharyngeal area they endanger the airway and can precipitate an emergency requiring surgery. Surgery is advisable in the early years.
BIRTHMARKS AND SKIN TUMOURS
Infantile haemangioma (strawberry naevus)
See CHAPTER 91. These start soon after birth as a red pinpoint lesion and grow rapidly for the first 6 months, then involute and become pale. Full resolution may take several years. Reassure parents and demonstrate how to stop any bleeding by applying pressure. Possible treatment options include oral or intralesional steroids, vascular laser, interferon and surgery. Surgical intervention is usually not necessary. Exceptions are locations in critical areas such as periorbital, nose, lips and face. Refer lesions on the eyelid early since visual obstruction can lead to amblyopia. Stridor accompanying a haemangioma on the face is suggestive of laryngeal haemorrhage, so refer urgently.
Capillary vascular malformation (port wine stain)
These are present from birth and surgical intervention is inadvisable. They may be treated by pulsed dye laser, which is best initiated as early as possible as the response is best in the first 2 years4 (see CHAPTER 91).
These are aggregations of abnormal subcutaneous veins that may infiltrate deeper tissues. In the past these lesions were treated surgically, but now the emphasis is on specialised sclerosant agents injected under fluoroscopic guidance and specialised laser techniques. Referral to Vascular Malformation Clinics in larger centres is worthy of enquiry.
These appear sometimes as skin lesions because of the red discolouration on the surface of the tumour. Management is as described above.
These have to be treated on an individual basis. If giant naevi they can be dermabraded at ideally less than 6 weeks.
Benign juvenile melanoma (Spitz naevus)
These pigmented lesions, which typically appear on the face, are usually surgically excised because of their rapid growth and family concerns.
CHEST AND BREAST DISORDERS
If necessary surgery should be performed in late adolescence after breast development is complete. It can take the form of a unilateral implant, different-sized bilateral implants or unilateral breast reduction.
Reduction surgery should also be delayed until breast growth is complete, at late adolescence.
This is not to be confused with pseudogynaecomastia due to fat in obese preadolescents. However, gynaecomastia in thin boys does occur and requires referral for assessment if it cannot be attributed to drugs such as oestrogen. If it develops in the pubertal stage, gynaecomastia may resolve spontaneously within 1 or 2 years. If necessary, simple mastectomy can be performed, if no cause can be found.
Subareolar hyperplasia in boys
This presents as a firm discoid subareolar lesion similar to premature breast hyperplasia of girls. It typically occurs at about 12–14 years. There is no indication for surgical treatment. Give an explanation with reassurance that the problem will dissipate.
Chest wall skeletal deformity
Surgical correction is best performed in adolescence.
This syndrome is an absent sternal head of pectoralis major with associated chest wall deformity plus a hypoplastic or absent breast and nipple–areolar complex. Surgical correction can be undertaken from 10 to 20 years.
CONGENITAL HEART DISORDERS
GPs have an important role in the diagnosis of congenital heart disorders as many of the affected infants develop cyanosis, murmurs or congestive heart failure. Early diagnosis and intervention helps prevent serious problems such as bacterial endocarditis and paradoxical emboli.
Ventricular septal defect (VSD)
VSD is the commonest congenital heart lesion (1:500 births).
The defect connects the two ventricles with a L → R shunt (see FIG. 92.4).
Ventricular septal defect: defect in muscle wall
Symptoms and signs depend on size of hole. All have a palpable thrill at the left sternal edge and a pansystolic murmur down right sternal edge.
Small VSD (‘maladie de Roger’): harsh murmur, usually asymptomatic and closes spontaneously.
Larger VSD: symptoms appear in infancy.
Breathlessness on feeding and crying (i.e. early CHF)
Recurrent chest infections
Failure to thrive
Heart failure from about 3 months with large defects
Refer early, especially if failure—early surgery performed by 6 months but can be performed at any age from the newborn period. A patch can close the defect through open-heart surgery. Some may be closed by sealing with an occlusive device through a percutaneous cardiac catheter. A cardiologist will make the appropriate decision. As a general rule about 50% of all VSDs will close spontaneously. The membraneous type, unlike the muscular type, is less likely to close spontaneously.
Atrial septal defect (ASD)
In ASD the defect connects the two atria with two distinct types—ostium secundum with holes higher in the septum (most common) and ostium primum with holes lower in the septum (more serious) (see FIG. 92.5). Signs are a mid-systolic murmur in the pulmonary area, a split 2nd sound and a loud P2. An echocardiogram is diagnostic.
Atrial septal defect: primum defect
Symptoms are uncommon in infancy and childhood with ostium secundum but heart failure with pulmonary hypertension develops early with ostium primum.
Refer these patients early. Prophylactic antibiotics are needed for patients with ostium primum. Follow other cases with regular echocardiograms and growth/development monitoring. Closure is advisable where there is evidence of a troublesome shunt. Options are repair by direct surgical suture or an insertion of a patch or a device closure using a self-expanding ‘double umbrella device’ manipulated into the defect via cardiac catheterisation.
All patients require prophylactic antibiotics before procedures.
The ductus fails to close after birth. A loud, continuous machinery murmur is heard. Symptoms relate to shunt size. The child presents with a murmur with possible respiratory infections, failure to thrive and heart failure. Refer for possible surgical closure by ligation. Alternatives include device closure with placement of an occlusive device or by embolisation coils.
This usually presents in infancy with heart failure. Refer for early surgery to remove the narrowed portion of the aorta.
HERNIAS AND GENITAL DISORDERS
These usually present in the first 3 to 4 months with an incidence of 1 in 50 males and 1 in 500 females (see FIG. 92.6).
Inguinal and femoral hernias should be referred urgently as early surgery is advisable to avoid the high risk of bowel incarceration or strangulation and ovarian entrapment and ischaemia in females. It is useful to follow the ‘6–2’ rule (see CHAPTER 112).
Scrotal hydroceles are painless cystic swellings around the testis. The opening of the processus vaginalis is narrow and often closes spontaneously. Two types can be identified—slack, often bilateral, which disappear within 12 months, and tense, often unilateral, which often persist after the first year. Ninety per cent resolve by 18 months of age; for those that persist, referral is recommended with a view to surgical intervention if present for longer than 2 years.
Testes can still descend up to 3 months after birth. Refer by 6 months with a view to correction between 9 and 12 months but definitely before 2 years (see CHAPTER 112).
Refer to disorders of the penis (see CHAPTER 113). Look for other abnormalities. Refer as soon as possible if the child is not producing a good urinary stream. Non-urgent cases should be evaluated by 6 months with a view to surgery at around 12 months; these patients should not be circumcised.
The foreskin and circumcision
For more detail refer to penile problems (see CHAPTER 113). If not circumcised in the neonatal period it is best performed under general anaesthetic after 6 months of age following consultation, counselling and with the consent of both parents.
Real phimosis is uncommon and almost all cases of tight foreskin with narrowing of the preputial orifice resolve naturally. Treatment with corticosteroid creams should be considered (see CHAPTER 113). Probably the only indication for circumcision is persistent difficulty in passing urine.
Management of this painful condition is outlined in CHAPTER 113.
Surgery is not usually required for umbilical herniae as most close naturally by 4 years of age (95% resolve spontaneously by 2–3 years). Refer for possible repair if still present at 4 years of age. A good guideline is that if the hernial orifice is greater than 1 cm at 12 months then surgical intervention is a possibility. It is usual to operate at 4–5 years.
This is due to a defect in the linea alba adjacent to the umbilicus proper. Most lie just above the umbilicus. The defect is felt like an elliptical slit with firm edges. Spontaneous closure rarely occurs and they are more likely to incarcerate. Refer for operation at any age preferably after 6–12 months.
An epigastric hernia (not to be confused with divarification of the rectus muscles) lies between the umbilicus and the xiphisternum. It is unlikely to close naturally, is likely to incarcerate and causes pain by strangulation of herniated fat. Indications for repair are pain (reproducible on palpation of the hernia) and cosmetic.
Anal fissures are often seen in infants and toddlers with uncomfortable defecation and minimal bright bleeding. The anal mucosa is split in the midline either anterior or posterior. It is caused by the passage of hard stool. The fissure usually heals within a few days.
Fused labia (labial agglutination)
Labial fusion is caused by adhesions considered to be acquired from perineal inflammation (see CHAPTER 107). They are certainly not present at birth. Most authorities recommend no treatment if the child can void readily and allow natural healing to occur.
CHILDHOOD LEG AND FOOT DEFORMITIES
Developmental dysplasia of hip
Detected by clinical examination (Ortolani and Barlow tests) and ultrasound examination (see CHAPTER 65).
Infants are usually treated successfully by abduction splinting (e.g. Pavlik harness).
Open reduction may be required, especially in older babies and toddlers.
Most are physiological (which are symmetrical) and improve with age.
Consider rickets in children at risk.
Toddlers are usually bow-legged until 3 years of age.
Resolve spontaneously by 3 years except in severe cases.
Monitor intercondylar separation (ICS): distance between medial femoral condyles.
Refer when ICS >6 cm at 4 years, not improving or asymmetric (see FIG. 92.7).
Postural variance of lower limbs
Knock knees (genu valgum)
Most are physiological and children are usually knock-kneed from 2–8 years (maximal 3–4 years).
Running is awkward, but improves with time.
Reassure parents about spontaneous improvement.
Monitor intermalleolar separation (IMS): distance between medial malleoli.
Refer if IMS >8 cm (see FIG. 92.7).
In-toeing does not cause pain or affect mobility.
Causes of in-toeing (see FIG. 92.8) are metatarsus varus, internal tibial torsion and medial femoral torsion. Children with femoral torsion tend to sit in a characteristic ‘W’ sitting position (see FIG. 92.9). These features are compared in TABLE 92.1.5
The classic ‘W’ position of femoral torsion (inset hips)
Table 92.1In-toeing in childhood ||Download (.pdf) Table 92.1 In-toeing in childhood
| ||Metatarsus varus ||Internal tibial torsion ||Medial femoral torsion |
|Synonyms ||Metatarsus adductus || ||Inset hips |
|Age at presentation ||Birth ||Toddler ||Child |
|Site of problem ||Foot ||Tibia ||Femur |
|Examination ||Sole of foot bean-shaped ||Thigh–foot angle is inwards ||Arc of hip rotation favours internal rotation |
|Management ||Observe or cast ||Observe and measure ||Observe, rarely surgery |
|Resolution (usually by) ||3 years ||3–4 years ||8–9 years |
|When to refer if not resolved ||3 months after presentation ||6 months after presentation ||8 years after presentation |
Have restricted internal rotation of hip due to an external rotation contracture
Exhibit a ‘Charlie Chaplin’ posture between 3 and 12 months—up to 2 years
Child weight-bears and walks normally
No treatment required as spontaneous resolution occurs
Surgery may be necessary in older children.
Club foot (congenital talipes equinovarus)
Most abnormal-looking feet in infants are not a true club foot deformity; the majority have postural problems referred to as ‘postural talipes’ such as talipes calcaneovalgus, metatarsus varus and postural talipes equinovarus. Such conditions are usually quite mobile and mild, and all resolve spontaneously without treatment. True club foot deformity is usually stiff and severe, and requires orthopaedic correction.5
Inset hips (medial femoral torsion)
In children with inset hips, the femur tends to rotate inwards especially when the child is about 5–6 years old and is normal up to 12 years. The children tend to sit in a ‘W’ position (see FIG. 92.9). Fortunately, most children outgrow this condition before the age of 12.
Flat feet (pes plano valgus)
The majority are physiological and are usually hereditary. All newborns have flat feet but 80% develop a medial arch by their sixth birthday and most by 11 years.5 The presence of the arch can be demonstrated to parents by the tiptoe test. The arch can be seen better when the feet are hanging in the air and even better still when the child is standing on tiptoes (see FIG. 92.10). Flat feet are present in about 10% of teenagers. No treatment is required unless painful and stiff. Good roomy footwear is important. Studies in California have shown no benefit from wearing orthoses or other forms of arch supports. Arches develop naturally.
The tiptoe test for flat feet
Usually the third toe curls inward under the second toe so that the second toe lies above the level of the first and third toes. The toes can usually be straightened if necessary, so ignore the problem until 2 years. Refer if necessary to have a severe deformity corrected by flexor tenotomy.
A summary of optimal signs for surgical intervention in children’s surgical disorders is presented in TABLE 92.2.
Table 92.2Optimal times for surgery/intervention in children’s surgical disorders ||Download (.pdf) Table 92.2 Optimal times for surgery/intervention in children’s surgical disorders
|Disorder ||Surgery/intervention |
|Squint (fixed or alternating) ||12–24 months: absolutely before 7 years |
|Tongue tie ||3–4 months or 2–6 years |
|Ear deformity ||After 6 years |
|Cleft lip ||Less than 3 months |
|Cleft palate ||6–12 months |
|Inguinoscrotal lumps: ||Best assessed before 6 months |
| || |
Surgery best at 6–12 months
Don’t leave >12 months
| || |
ASAP, especially infants and irreducible hernias
Reducible hernias: the ‘6–2’ rule
Birth–6 weeks: surgery within 2 days
6 weeks–6 months: surgery within 2 weeks
Over 6 months: surgery within 2 months
| ||ASAP |
| ||Surgery within 4 hours (absolutely 6 hours) |
| ||Leave to 12 months, then review (often resolve; if not, repair by 2 years) |
| ||Leave and review |
|Other hernias: |
| || |
Leave to age 4
Surgery after 4 if persistent (tend to strangulate)
Never tape down
| ||Any age—best after 6 months |
| ||Any age—best after 6 months |
|Leg and foot development problems: |
| ||Most treated successfully by abductor splinting (e.g. Pavlik harness) |
| || |
Normal up to 3 years
Usually improve with age: refer if ICS > 6 cm
| ||Normal 3–8 years, then refer if IMS > 8 cm |
| ||No treatment unless stiff and painful |
| ||Refer 6 months after presentation if unresolved |
| ||Leave to 8 years, then refer if unresolved |
| ||Refer 3 months after presentation if unresolved |