PART COMMON PAEDIATRIC SURGICAL PROBLEMS

Transcription

1 PART 9 COMMON PAEDIATRIC SURGICAL PROBLEMS Ch009-F10280.indd 261 7/27/2007 5:35:23 PM

2 Ch009-F10280.indd 262 7/27/2007 5:35:23 PM

3 Common surgical conditions in children S. W. Beasley 9.1 The penis and foreskin The glans of the uncircumcised penis is protected by a layer of loose skin called the foreskin or prepuce. The amount of foreskin present varies among boys. At birth, and for many years afterwards, it is normal for part or all of the undersurface of the foreskin to be adherent to the glans penis. This adherence slowly separates during childhood. Forcible retraction of the foreskin before it is ready can damage the glans and may cause secondary phimosis. Therefore, the foreskin should not be retracted forcibly unless a circumcision is being performed. Spontaneous separation of these adhesions is normally complete by puberty. Smegma Smegma accumulates beneath the adherent foreskin. It appears as asymmetrical accumulations of yellowtinged material predominantly in the coronal groove beneath the foreskin (Fig ). There may be sufficient smegma to produce a noticeable swelling, which may be misdiagnosed as a dermoid cyst or tumour. It is often misinterpreted as being mid-shaft because a small child s coronal groove may be a long way from the tip of the foreskin. Smegma is normal, and is released spontaneously as the foreskin separates from the glans penis. When it is released, it may be associated with some redness and irritation of the foreskin for a day or so: this, too, is a normal process. Balanitis Infection can develop beneath the foreskin and, if severe, pus may appear from the end of the foreskin. Balanitis is often associated with phimosis. Infection may cause considerable redness and swelling of the penile shaft, necessitating treatment with either topical or oral antibiotics. Phimosis In phimosis the opening at the tip of the foreskin has narrowed down to such a degree that the foreskin cannot be retracted (Fig ). The external urethral meatus is not visible. Phimosis must be distinguished from the normal adherence of the foreskin to the glans. In most boys, phimosis can be treated by application of steroid ointment (e.g. betamethasone valerate ointment) to the tight, shiny part of the foreskin. This usually obviates the need for circumcision. However, marked previous inflammation, infection, skin splitting and balanitis xerotica obliterans can lead to marked scarring of the foreskin and phimosis, and in many of these children the only reasonable treatment is circumcision. Sometimes the severity of phimosis is such that there is ballooning of the foreskin on micturition, and on rare occasions it may even cause urinary retention with a distended bladder. A degree of phimosis is common in infancy but tends to resolve spontaneously in the first few years of life, and is not considered abnormal in this age group. Paraphimosis Paraphimosis occurs when a mildly phimotic foreskin has been retracted over the glans and has become stuck behind the coronal groove, causing oedema of itself and the glans penis (Fig ). It is a painful and progressive process. Treatment involves gentle manipulation of the foreskin forwards, which may require a general anaesthetic. Circumcision is not performed at this time, but a few children may need it subsequently if the phimosis does not respond to topical application of steroid ointment. Hypospadias It is important to recognize hypospadias when it is present (Fig ). The foreskin looks square and hangs off the penis, and the shaft of the penis is bent ventrally. The two main problems in hypospadias are: the location of the urethra (which can be found on the ventral side of the shaft of the penis, proximal to its correct position) chordee (ventral angulation of the shaft and glans) Correction of chordee to straighten the penis is required to allow later successful sexual function. 263 Ch009-F10280.indd 263 7/27/2007 5:35:23 PM

4 9.1 COMMON PAEDIATRIC SURGICAL PROBLEMS A Fig In phimosis, the foreskin is narrowed and cannot be retracted. B Fig A. The normal foreskin with accumulation of smegma beneath it. The swellings caused by the smegma are in the region of the coronal groove. B. On retraction, smegma appears as accumulations of material beneath a foreskin that has not yet separated from the glans. Fig Paraphimosis: the foreskin has become stuck behind the coronal groove. 264 The operation is usually performed as a single-stage procedure at 9 12 months of age, often as day surgery. Circumcision is absolutely contraindicated in hypospadias because the skin of the prepuce is used during repair of the hypospadias. Severe hypospadias may be indicative of an intersex abnormality. For example, when there is penoscrotal hypospadias and a bifid scrotum, the scrotum should be examined carefully for testes, because some of these children may be females with congenital adrenal hyperplasia; the labioscrotal folds are labia rather than scrota, and the presumed urethral opening may in fact be the entrance to the vagina (Ch. 19.3). Circumcision The indications for circumcision remain controversial. In many countries, circumcision has been abandoned in the neonatal period because of its relatively high complication rate. Apart from the risk of septicaemia and meningitis when performed in the relatively immunologically immature neonate, there are a number of problems that may occur during circumcision at any age. These include removal of too much or too little foreskin, postoperative bleeding and infection. Haemorrhage postoperatively occa- Ch009-F10280.indd 264 7/27/2007 5:35:23 PM

5 COMMON SURGICAL CONDITIONS IN CHILDREN 9.1 Epispadias In epispadias, the urethra opens on to the dorsal aspect of the base of the penis. Epispadias is part of a spectrum of lower abdominal wall defects in which ectopia vesicae (bladder exstrophy) and cloacal exstrophy are the most severe forms. Boys with epispadias are often incontinent of urine because the sphincter of the bladder neck is also deficient. Clinical example James was a 7-year-old boy who presented following two episodes of balanitis. He also complained of discomfort on micturition. Examination revealed a tight foreskin that could not be retracted; the urethral meatus could not be seen. After 1 month of topical application of betamethasone ointment four times a day to the tight part of the foreskin, he was able to fully retract it. Circumcision was not necessary. B A Fig In hypospadias the ventral shaft of the penis is angulated and shortened (chordee), the urethral meatus is ventrally placed and the foreskin is defi cient on the underside. A. Ventral aspect. B. Appearance from above. sionally requires surgical reintervention. The most troublesome and common complication of circumcision is abrasion and ulceration of the sensitive glans penis, particularly near the urethral meatus. As the meatal ulceration heals it may produce meatal stenosis and require a meatotomy to re-establish an adequate urinary stream. The inguinoscrotal region Inguinal hernia After the testis has descended into the scrotum during the seventh month of pregnancy, the canal down which it migrates, the processus vaginalis, should obliterate. Failure of obliteration of the processus vaginalis may produce an inguinal hernia, a hydrocele or an encysted hydrocele of the cord. A widely patent proximal processus vaginalis allows bowel (and, in girls, the ovary as well) to enter the inguinal canal, producing a reducible lump in the groin called an indirect inguinal hernia (Fig ). This occurs in about 2% of live male births but is less frequent in girls. The greatest incidence is in the first year of life. The usual presentation is that of an intermittent swelling, overlying the external inguinal ring, that has been noticed by a parent. At times it may appear to cause discomfort. It is most likely to be obvious during an episode of crying or straining, and in infants may be seen during nappy changes. Inguinal hernias should be repaired as soon as practicable. Strangulation of inguinal hernias is common, particularly during the first 6 months of life. Strangulation can be recognized when the groin swelling becomes irreducible. If left untreated, a strangulated hernia may damage the incarcerated bowel and, occasionally, by compressing the testicular vessels may lead to testicular atrophy. For this reason, an immediate attempt should be made to reduce the hernia manually. This is done by first disimpacting 265 Ch009-F10280.indd 265 7/27/2007 5:35:24 PM

6 9.1 COMMON PAEDIATRIC SURGICAL PROBLEMS Fig Large bilateral inguinal hernias. 266 the hernia at the external inguinal ring, and then reducing it along the line of the inguinal canal. Fortunately, most hernias that become stuck can be reduced manually; the hernia can then be repaired as an elective procedure within a few days. This is best done in a specialist paediatric surgical centre. Hydrocele A hydrocele presents as a painless cystic swelling around the testis in the scrotum (Fig ). It contains peritoneal fluid that has tracked down a narrow but patent processus vaginalis. It transilluminates brilliantly. When the hydrocele is lax, the testis can be felt within it. The upper limit of the hydrocele can be demonstrated distal to the external inguinal ring, distinguishing it from an inguinal hernia, where the swelling extends through the external inguinal ring. There is no impulse on crying or straining. Hydroceles are common in the first few months of life, do not cause discomfort and usually disappear spontaneously. Surgery is only indicated if the hydrocele persists beyond 2 years of age. Undescended testis Undescended testis (or cryptorchidism) is a term used to describe the testis that does not reside spontaneously in the scrotum. Undescended testes occur in about 2% of boys, being more common in premature infants. Spontaneous descent of the testis is unlikely beyond 3 months post-term. Cryptorchidism is important to detect because it will result in Fig A right hydrocele. reduced fertility if left untreated. It is suspected that the higher temperature to which an undescended testis is subject impairs spermatogenesis. The diagnosis is made by examining the inguinoscrotal region. Normally, the testis should be found within the scrotal sac. In cryptorchidism the scrotum looks empty (Fig ). The testis is milked down the line of the inguinal canal towards the scrotum and is pulled gently towards the scrotum. If the testis cannot be brought into the scrotum or will not remain there spontaneously it is considered undescended. Clinically, it may be difficult to distinguish a retractile testis from an undescended testis. In most normal boys the testis resides in the bottom of the scrotum, but the cremasteric reflex, which is prominent during mid-childhood, may cause it to move upwards, sometimes completely out of the scrotum. A retractile testis found outside the scrotum initially can be brought down into the normal position and should stay there spontaneously, at least until the cremasteric reflex is stimulated (Table 9.1.1). An undescended testis will not stay in the scrotum spontaneously and usually cannot even be coerced beyond the neck of the scrotum. It is often smaller than a normal testis on the other side. Undescended testes should be brought down into the scrotum surgically between 9 and 12 months of Ch009-F10280.indd 266 7/27/2007 5:35:25 PM

7 COMMON SURGICAL CONDITIONS IN CHILDREN 9.1 Table Comparison of undescended and retractile testes Feature Undescended testis Retractile testis Can be brought fully to bottom of scrotum No Yes Remains in scrotum spontaneously for a period before retracting No Yes Resides spontaneously in scrotum at times No Yes Normal size Normal or small Normal Fig An acutely painful scrotum in a child is most likely to be caused by torsion of an appendix testis or torsion of the testis. Fig Undescended right testis. age. Unfortunately, in many boys the diagnosis is not made until the child is older. The later the testis is brought down, the more likely it is that there will be damage to spermatogenesis. Orchidopexy is performed as a day case procedure. In general, the results are excellent when the procedure is performed by a specialist paediatric surgeon. The acutely painful scrotum There are a number of conditions that cause an acutely painful or enlarged scrotum (Fig ), of which torsion of a testicular appendage is the most common, and torsion of the testis itself the most important (Table 9.1.2). In both conditions the boy complains of severe pain in the scrotum. In the early stages of torsion of a testicular appendage, a blueblack pea-sized swelling which is extremely tender to touch may be seen through the skin of the scrotum near the upper pole of the testis. Palpation of the testis itself causes no discomfort. Later, a reactive hydrocele develops, the tenderness becomes more generalized and the clinical features may make it difficult to distinguish from torsion of the testis. Where torsion of the testis has occurred, both the testis and the epididymis are exquisitely tender (unless necrosis has already occurred) and the testis may be lying high within the scrotum. In older boys the pain radiates to the ipsilateral iliac fossa and may be associated with nausea and vomiting, producing symptoms similar to those of appendicitis, which highlights the importance of always examining the scrotum in boys presenting with lower abdominal pain. 267 Ch009-F10280.indd 267 7/27/2007 5:35:25 PM

8 9.1 COMMON PAEDIATRIC SURGICAL PROBLEMS Table Causes of an acutely painful scrotum Condition Comment Frequency Torsion of testicular appendix Peak age 11 years >75% Unilateral tenderness Torsion of testis Peaks in neonatal and adolescent age groups 20% Surgical emergency Epididymo-orchitis Usually in infancy Rare Association with urinary tract abnormalities Idiopathic scrotal oedema Usually in young child Rare Bilateral oedema Testes not tender 268 Treatment Urgent surgical exploration of the scrotum is required to untwist the testis and epididymis and to suture both testes to prevent subsequent torsion. A completely necrotic testis should be removed. A torted and infarcted testicular appendix should be removed. In this situation the testis should be checked to make sure that it has not twisted but otherwise it requires no treatment. When a testis has twisted the contralateral testis should also be examined and pexed to prevent it from twisting at a later date. Other causes of scrotal pathology Epididymo-orchitis is unusual in children: it is most often seen during the first year of life, where it may signify an underlying structural abnormality of the urinary tract. For this reason investigation involves a renal ultrasound and micturating cystourethrogram. Examination of the urine may show leukocytes and bacteria. Mumps orchitis is extremely rare prior to puberty. In idiopathic scrotal oedema there is painless boggy oedema of the whole scrotum and the testes are completely non-tender. Testicular malignancy is occasionally seen in leukaemia or with a primary testicular neoplasm. Abnormalities of the umbilicus The umbilical cord desiccates and separates several days after birth, allowing the umbilical ring to close. Sometimes the stump of the cord may become infected, the umbilical ring may not close, or there may be remnants of the embryonic channels that pass through the umbilicus before birth (Table 9.1.3). Table Abnormalities of the umbilicus Abnormality Umbilical hernia Comment Exomphalos See Chapter 11.5 Gastroschisis See Chapter 11.5 Umbilical hernia Umbilical sepsis ( omphalitis ) Umbilical granuloma Ectopic bowel mucosa Patent vitellointestinal duct Patent urachus Common, most resolve Asymptomatic Skin covered Neonatal, serious condition Common, treat with silver nitrate Often pedunculated Treat with silver nitrate Sinus opening at umbilicus Communication with ileum Discharges faecal fl uid and gas Communication with bladder Discharges urine Failure of the umbilical ring to close after birth produces an umbilical hernia (Fig ). Umbilical hernias are common in neonates but most close spontaneously in the first year of life. The skin overlying the umbilical hernia never ruptures and strangulation of the contents is virtually unknown. The swelling will become tense when the infant cries or strains. Umbilical hernias normally do not cause pain. No treatment is required during the first few Ch009-F10280.indd 268 7/27/2007 5:35:25 PM

9 COMMON SURGICAL CONDITIONS IN CHILDREN 9.1 band attaching the ileum to the deep surface of the umbilicus may cause intestinal obstruction. A Meckel s diverticulum represents persistence of the ileal part of the duct. Vitellointestinal duct remnants are excised. Urinary discharge from the umbilicus suggests a persistent communication with the bladder in the form of a patent urachus. Sometimes it may produce a cystic mass or abscess in the midline just below the umbilicus. Urachal remnants should be excised. The anus and perineum A variety of unrelated conditions affect the anus and perineum in children. Fig Umbilical hernia. years of life. If the hernia is still present after the age of 3 years it can be repaired as a day surgical procedure. Discharge from the umbilicus Discharge from the umbilicus may be pus, mucus, urine or faeces. An umbilical granuloma is a common lesion that first becomes evident after separation of the umbilical cord. There is a small accumulation of granulation tissue in the umbilicus, accompanied by a seropurulent discharge. If it has a definite stalk it can be ligated without anaesthesia, but most often it is treated by topical application of silver nitrate. Ectopic bowel mucosa has a similar appearance but has a smooth, red, glistening surface and discharges mucus. It is treated in the same way. Persistence of part or all of the vitellointestinal (omphalomesenteric) duct produces one of a number of abnormalities, which usually present in early infancy but may not be evident for some years. Complete patency of the tract allows ileal fluid and air to discharge from the umbilicus. Persistence of one part produces a sinus or cyst, which may become infected to form an abscess and may discharge pus. A vitellointestinal Anal fissure These are usually seen in infants and toddlers when passage of a hard stool splits the anal mucosa, causing sharp pain and often a few drops of bright blood. The condition is of little consequence and the fissure usually heals within days. Examination of the anal margin shows a split in the epithelium anteriorly or posteriorly in the midline. An anal fissure may occur in an older child, in which situation it tends to be related to constipation. The child gets severe pain on defaecation and becomes reluctant to defaecate, further worsening the constipation. Treatment is directed at overcoming the underlying constipation. A stool softener and lubricant, e.g. paraffin oil, may be helpful. A chronic indolent, often non-painful, fissure away from the midline may indicate inflammatory bowel disease, such as Crohn disease. Perianal abscesses These are most likely to occur in the first year of life from infection of an anal gland. The abscess points superficially, a centimetre or two from the anal canal. The abscess should be drained and the fistula between the abscess and the anal canal laid open to reduce the likelihood of recurrence. Rectal prolapse Rectal prolapse tends to occur in the second and third years of life in otherwise normal children. The rectum prolapses during defaecation and returns spontaneously afterwards. In some, manual reduction is required. The prolapsed mucosa may become congested and bleed but causes little discomfort. Clinically, it needs to be distinguished from prolapse of a benign rectal polyp (a benign hamartomatous lesion seen in children) and the apex of an intussusception 269 Ch009-F10280.indd 269 7/27/2007 5:35:25 PM

10 9.1 COMMON PAEDIATRIC SURGICAL PROBLEMS 270 (the child would have other symptoms of intussusception). The passage of time, and treatment of any underlying constipation, is all that is required in the majority of toddlers. Occasionally, a sclerosant is injected into the submucosal plane of the rectum for persistent cases. In a few patients there is an underlying organic cause for the rectal prolapse. Usually the reason is obvious, as in paralysis of anal sphincters in spina bifida and sacral agenesis, undernourished hypotonic infants, bladder exstrophy, cloacal exstrophy, following surgery for imperforate anus, or malabsorption. Labial adhesions This common condition is often detected on routine examination in infant girls, or noted incidentally by parents. The epithelium of the labia minora has fused in the midline; this may sometimes cause discomfort on micturition and may be associated with urinary infection. Labial adhesions are never present at birth. Asymptomatic adhesions require no treatment and resolve as the girl gets older as a result of rising levels of oestrogen. Gentle lateral traction on the labia may assist their separation if this is necessary because of problems with micturition or urinary infection. Adhesions have a tendency to recur after separation. The neck Lesions of the neck fall into two broad groups: developmental anomalies and acquired lesions. The exact location of the lesion will usually provide a clue as to its nature. Midline neck swellings The most common midline neck swelling in children (Table 9.1.4) is a thyroglossal cyst. Typically, there is a swelling overlying and attached to the hyoid bone that moves on swallowing and tongue protrusion. It may become infected to form an abscess with overlying erythema of the skin. The thyroglossal cyst and the entire thyroglossal tract is best excised before it becomes infected. Excision must include the middle third of the hyoid bone (Sistrunk operation), otherwise recurrence is common. Ectopic thyroid tissue is a less common cause of a midline neck swelling. Clinically, it may be difficult to distinguish from a thyroglossal cyst. If suspected preoperatively, a thyroid isotope scan will clarify the distribution of all functioning thyroid tissue. Table Midline neck swellings Cause of swelling Thyroglossal cyst Ectopic thyroid Submental lymph node/abscess Dermoid cyst Goitre Cystic hygroma Comment Congenital dermoid cysts can occur along any line of fusion, including the neck, where they are situated in the midline. A midline cervical dermoid is occasionally mistaken for a thyroglossal cyst. It contains sebaceous material surrounded by squamous epithelium. The most common congenital dermoid cyst is the external angular dermoid, which is found at the orbital margin. Dermoid cysts enlarge slowly and it is appropriate for them to be removed. Cystic hygromas are congenital hamartomas of the lymphatic system. They vary greatly in size and may involve the front of the neck or extend to one or both sides asymmetrically. Some complex cystic hygromas may contain cavernous haemangiomatous elements and may extend upwards into the floor of the mouth or downwards into the thoracic cavity. They may enlarge rapidly from viral or bacterial infection, or from haemorrhage. Depending on their extent and location, the airway may be compromised, leading to life-threatening respiratory obstruction. Surgery involves excision or debulking of the lesion. In some situations they are injected with sclerosants. Lateral neck swellings Most common (80% of midline neck swellings) Moves with tongue protrusion and swallowing Attached to hyoid bone May be only thyroid tissue present Do thyroid isotope scan Check inside mouth for primary infection Other cervical lymph nodes may be enlarged Small, mobile, non tender Yellow tinge through skin In subcutaneous layer Lower neck Hamartoma Usually evident from birth May be extensive Most lateral neck swellings are acquired, being due to infection of one or more of the cervical lymph Ch009-F10280.indd 270 7/27/2007 5:35:26 PM

11 COMMON SURGICAL CONDITIONS IN CHILDREN 9.1 nodes. Persistently enlarged cervical lymph nodes are normal in children with frequent upper respiratory infections: they represent a normal response to infection (i.e. reactive hyperplasia) and require no treatment. Lymph nodes may enlarge rapidly and become tender during active infection but usually settle with rest, analgesia and antibiotics as required. In children aged 6 months to 3 years lateral cervical lymphadenitis may progress to abscess formation: the lymph nodes enlarge over 4 5 days and become fluctuant, although deeper nodes may not exhibit fluctuation. The overlying skin becomes red. Treatment involves incision and drainage of the abscess under general anaesthesia. MAIS lymphadenitis Cervical lymphadenitis due to atypical mycobacterial infection is common in preschool children. The MAIS (Mycobacterium avium, intracellulare, scrofulaceum) infection produces chronic cervical lymphadenitis and collar stud abscesses (so called MAC or Mycobacterium avium complex) and usually affects the jugulodigastric, submandibular or preauricular lymph nodes. The involved lymph node increases in size over several weeks before erupting into the subcutaneous tissue as a collar stud cold abscess. Eventually, if untreated, it may cause purple discoloration of the overlying skin and will ulcerate through the skin to produce a chronic discharging sinus. MAIS infections respond poorly to antibiotics. Treatment involves surgical removal of the collar stud abscess and excision of the underlying infected lymph nodes. Lymph node tumours Primary tumours involving the lymph nodes occur in older children. Both Hodgkin and non-hodgkin lymphomas may involve cervical lymph nodes. Rarely, other tumours may metastasize to the cervical lymph nodes, e.g. neuroblastomas and nasopharyngeal tumours. beneath the anterior border of the sternomastoid near its upper third. They may become infected and should be removed. Sinuses or fistulas usually arise from the second branchial cleft, although sometimes the first and third clefts are responsible. Torticollis Torticollis, or wry neck, has many causes in childhood (Table 9.1.5). A sternomastoid tumour presents in the third week of life when the parents notice a hard lump in the neck or that the head cannot be turned to one side. The head is flexed slightly to the side of the shortened sternomastoid muscle, and is turned to the contralateral side. There may be a history of breech delivery or forceps delivery. There is a hard, painless swelling, usually 2 3 cm long, in the shortened sternomastoid muscle (Fig ). Sometimes the whole muscle may be involved. Table Causes of torticollis Cause Sternomastoid tumour Postural torticollis Comment Not present at birth Present at 3 weeks of age Tight, shortened sternomastoid muscle Most resolve without treatment Present at birth Disappears in months From intrauterine position Cervical hemivertebrae Imbalance of ocular muscles (strabismus) Lateral cervical lymphadenitis Tumours Atlanto-occipital subluxation Benign paroxysmal torticollis of infancy Branchial remnants Branchial remnants arise from the branchial arch system. A variety of abnormalities occur, including branchial cysts, branchial sinuses, branchial fistulas and persistent cartilaginous remnants. Branchial fistulas are present from birth but, because the opening is so tiny, they may not be noticed for some years. A drop of mucus or saliva may be observed leaking from the external orifice near the anterior border of the sternomastoid muscle in the lower neck. Branchial cysts present later in childhood with a mass Fig Sternomastoid tumour in torticollis. 271 Ch009-F10280.indd 271 7/27/2007 5:35:26 PM

12 9.1 COMMON PAEDIATRIC SURGICAL PROBLEMS Rotation of the head to the side of the tumour is limited. Plagiocephaly and hemihypoplasia of the face may develop in subsequent months. The tumour dis appears within 9 12 months in the vast majority of affected infants. Where fibrosis persists and causes permanent shortening of the muscle with persistent torticollis, the sternomastoid muscle should be divided. Occasionally, older children present with torticollis due to a short, tight and fibrous sternomastoid muscle; the ipsilateral shoulder is elevated, there may be compensatory scoliosis, and the child has difficulty rotating the head towards the affected side. These children require surgical division of the muscle. 272 Ch009-F10280.indd 272 7/27/2007 5:35:26 PM