Chapter 92: Surgical problems in children

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

Craniostenosis

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.

Hydrocephalus

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.

Prominent bat/shell ears

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

Facial deformity

It is best to refer any facial deformity as soon as it is detected.

External angular dermoid

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.

Nasal disorders

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)

Early signs:

• tongue may appear heart-shaped

• infants unable to protrude the tongue over the lower lip

• breastfeeding problems

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.

Pre-auricular sinus

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.

Branchial sinus/cyst/fistula

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.

Strabismus (squint)

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.

Main types of squint

See FIGURE 92.3.

• 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

Refer CHAPTER 91.

Sternomastoid tumour/fibrosis

Features in infants:

• 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.

Thyroglossal cyst

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.

Cervical lymphadenopathy

Refer to CHAPTER 61.

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).

Venous malformations

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.

Lymphatic malformation

These appear sometimes as skin lesions because of the red discolouration on the surface of the tumour. Management is as described above.

Congenital naevi

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.

Breast asymmetry

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.

Macromastia

Reduction surgery should also be delayed until breast growth is complete, at late adolescence.

Gynaecomastia

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.

Poland syndrome

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.

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).

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.

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.

Patent ductus arteriosus

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.

Coarctation of the aorta

This usually presents in infancy with heart failure. Refer for early surgery to remove the narrowed portion of the aorta.

Inguinal hernias

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).

Hydroceles

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.

Undescended testes

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).

Hypospadias

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.

Phimosis

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.

Paraphimosis

Management of this painful condition is outlined in CHAPTER 113.

Umbilical hernia

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.

Umbilical granuloma

Refer CHAPTER 91.

Para-umbilical hernia

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.

Epigastric hernia

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 fissure

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.

Penile problems

Refer CHAPTER 113.

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.

Bow legs (genu varum)

• 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).

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 (pigeon toes)

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

Out-toeing

Infants

• 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.

Curly toes

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.

Refer to CHAPTER 34.

Refer to CHAPTER 60.

It is worthwhile knowing those non-traumatic conditions that demand immediate attention. Danger signs include excessive drooling of frothy secretions, bile-stained vomiting and delayed passage of meconium.

Neonatal gut obstruction—consider meconium ileus, volvulus, small bowel atresia.

Neonatal emergencies^6,7

• Oesophageal atresia: rattling respiratory distress + excessive drooling and secretions + choking with feeding (passage of 10F catheter stops about 10 cm)

  Action: nil orally, oropharyngeal suction, IV fluids

• Diaphragmatic hernia: severe respiratory distress + barrel-shaped chest + scaphoid abdomen (X-rays of chest/abdomen show loops of bowel in chest)

  Action: Give O₂, nasogastric tube (avoid bag and mask)

• Bilious (green vomiting) = bowel obstruction or malrotation (abdominal X-ray)

  Action: nasogastric drainage and refer (do not feed)

• Neonatal intestinal obstruction including volvulus, Hirschsprung disorder, atresia and meconium ileus: bilious vomiting (green bile) + distension + delayed stools

  Action: nasogastric tube, IV fluids, refer

• Imperforate anus and rectum

  Action: refer for surgery on day of birth, anoplasty for low lesions; complex surgery for high lesions

• Bile duct atresia: neonatal jaundice (conjugated bilirubin) (usually 4–6 weeks) → white stools

  Action: refer early for precise diagnosis and surgical correction

• Congenital lobar emphysema: respiratory distress + cyanosis + signs of emphysema

  Action: refer early for urgent assessment and surgery to remove diseased lung

• Congenital cystic disease of the lungs: respiratory distress soon after birth

  Action: as above

• Congenital heart disease (severe forms)

  Action: refer early for medical treatment and assessment

• Exomphalos (intestinal contents in a sac)

  Action: nasogastric tube, IV dextrose drip, temperature control, refer

• Gastroschisis (exposed bowel contents through anterior wall defect)

  Action: as for exomphalos; cover with plastic wrap

• Pierre–Robin syndrome: micrognathia + cleft palate + respiratory obstruction from tongue

  Action: early referral

• Tension pneumothorax

  Action: intercostal needle/catheter with aspiration

• Myelomeningocele and meningocele

  Action: early neurosurgical referral

Note the importance of plain X-rays as urgent first-line investigations and early surgical referral.

• Pyloric stenosis: projectile vomiting weeks 2–6, epigastric ‘tumour’ (CHAPTER 60)

• Torsion of testis: severe groin/low abdominal pain + vomiting

• Intussusception: pallor + abdominal pain (severe and intermittent) + inactivity

• Acute appendicitis: abdominal pain + a/n/v + guarding

• Peritonitis

• Intestinal obstruction: colicky pain + vomiting + distension

• Irreducible inguinal hernia

• Paraphimosis

• Cerebral abscess

• Meckel diverticulum—diverticulitis or haemorrhage

• Various neoplasias