Paediatrics Flashcards
Dermoid cyst
Dermoid cysts develop at an area where fusion of
sections of the embryo has occurred, and are most
common in the midline of the neck, at the external
angle of the eye, and behind the pinna. They should be removed to prevent secondary infection.
Cleft lip and palate
This is one of the more common congenital anomalies, occurring in 1 in 600 live births.
The face forms during the fifth to the eighth week,
from the maxillary and mandibular prominences of
the first branchial arch.
They grow and fuse together, and if this fusion is incomplete, unilateral or bilateral cleft lip may arise.
The palate develops after the eighth week, and
fusion occurs between the primary palate (the anterior section of the premaxilla and attached four front teeth) and the secondary palate (the hard and soft palate).
The palate may be cleft posteriorly only, a cleft soft palate, or it may extend anteriorly to include the hard palate, cleft palate only, and more commonly it extends further anteriorly to join up with either a unilateral or bilateral cleft lip.
Surgical correction of cleft lip and palate needs to
take the embryological origins and in particular the
blood supply into consideration, in order to allow an
optimum repair and subsequent growth.
Cystic hygroma
The primitive lymph sacs develop in the mesenchyme in the sixth week, and the largest is in the neck, and should resolve, but persistence and sequestration produces a multicystic swelling within the neck which is a lymphangioma, a benign hamartoma (overgrowth of normal tissue), which is also called a cystic hygroma when it occurs in the neck.
Occasionally this is very large and causes respiratory distress in the neonatal age group, but more usually is just a soft swelling in the neck which may extend into the axilla, or even the chest.
A degree of spontaneous resolution can be
hoped for, but often it comes to surgical debulking – a difficult prospect because of the multicystic nature, which makes it difficult to be sure that every bit of the abnormal tissue is removed. If there is a haemangiomatous element as well as the lymphangiomatous part, spontaneous resolution is unlikely.
An MRI scan is recommended to delineate the full extent and nature of the lesion, and the normal structures which are involved, to help plan surgical excision.
Congenital diaphragmatic hernia
The diaphragm develops between the thoracic and abdominal cavity,
finished before the end of the eighth week.
As the embryo folds and carries the primitive heart and septum transversum caudally and ventrally, it carries part of the yolk sac dorsally to develop as the foregut. The lateral mesenchyme develops into the pericardioperitoneal canals,
The motor nerve supply C3–5. can cause shoulder tip pain (which area is also supplied by C4).
Diaphragm develops from the fusion of four
parts
The diaphragm develops from the fusion of four
parts:
• The septum transversum (the fi brous central
tendon);
• The mesentery of the foregut (the area adjacent
to the vertebral column becomes the crura and
median part);
• Ingrowth from the body wall (the peripheral
muscular portion); and
• The pleuroperitoneal membrane (a small dorsal
part).
VACTERL
VACTERL:
• Vertebral anomalies (e.g. hemivertebrae);
• Anorectal anomalies (e.g. imperforate anus);
• Cardiac anomalies;
• Tracheal anomalies (e.g. fi stula, tracheomalacia);
• Esophageal anomalies (the American version!);
• Renal anomalies; and
• Limb anomalies (e.g. radial aplasia).
Pyloric stenosis
The stomach develops from a simple tubular part of the foregut by localised dilatation.
The mesentery which suspends the stomach from the posterior abdominal wall enlarges and becomes the greater omentum. The exit of the stomach into the duodenum is the pyloric canal.
Pyloric stenosis presentation
The baby usually presents after 10–50 days (most commonly 3–5 weeks),
Non-bilious projectile vomiting.
Diagnosis and treatment of pyloric stenosis
The diagnosis is made by feeding the baby, to relax the baby. The visible peristalsis may be seen, feel for the pylorus, which can be felt as a olive like lump in the right upper quadrant,
Ramstedt pyloromyotomy
Pancreas development
The pancreas develops from two outgrowths of the foregut, one ventral and one dorsal.
Due to rotation, the ventral bud and the adjacent gallbladder and common bile duct rotates so that the ventral and dorsal buds lie adjacent to each other and fuse. The two ducts also usually fuse, and the main pancreatic duct enters the duodenum adjacent to the entry of the common bile duct at the ampulla of Vater.
Annular pancreas - outgrowth of ventral duodenum
Duodenal atresia presentation
duodenal atresia present in the first few days of Babies with duodenal stenosis present later
The most common part of the duodenum to be obstructed is just distal to the ampulla of Vater, and so the vomit is most likely to be bilestained. Plain abdominal x-ray in duodenal atresia reveals a ‘double bubble’ – the first bubble being air in the distended stomach, and the second bubble being air in the distended duodenum.
can lead to polyhydramnios.
Duodenal atresia treatment
laparotomy, and a duodenoduodenostomy – which is the most physiological operative correction.
Malrotation
abdominal x-ray may show the small bowel on the right of the abdomen, and the large bowel on the left,
This requires urgent resuscitation, and then laparotomy and correction, before a volvulus occurs.
a contrast meal will demonstrate the position of the duodenojejunal flexure and subsequent lie of the jejunum.
Meconium ileus
Meconium ileus is the term given to ileal obstruction
due to inspissated meconium in the terminal ileum in neonates. Management includes treatment of the obstruction, and then investigation and treatment of the baby for cystic fibrosis.
Anterior abdominal wall defects
These comprise exomphalos and gastroschisis.
Treatment of anterior abdominal wall defects
replace the exteriorised bowel/organs into the abdominal cavity,
feed the baby intravenously .
Umbilical remnant: Meckel’s diverticulum
The vitello-intestinal duct is the remnant of the yolk sac
It should completely obliterate during the sixth week, but may persist If it persists
completely, leads to the Meckel’s diverticulum, which arises from the terminal ileum.
The classical description in adults is that it is present in 2% of the population, is 2 inches (5 cm) long, and 2 feet (60 cm) from the ileocaecal valve.
Meckel’s diverticulum treatment
Asymptomatic - ileal muscosa
Symptomatic - gastric muscosa
may undergo peptic ulceration with subsequent bleeding. It may present with diverticulitis (like appendicitis), or with adhesion/
band obstruction and volvulus because of its persistent attachment to the umbilical cord.
Management is by excision
Umbilical remnant: Urachus
The urachus is the embryonic remnant of the connection between the urinary bladder and the allantois at the umbilicus
can also persist, from which urine comes. Treatment is by surgical excision after accurate diagnosis.
normally urachus is obliterated to form the median umbilical ligament
Hirschsprungs disease
Absence of ganglion cells in the myenteric plexus
Histology of Hirschsprungs disease
As well as aganglionosis, the other
histological abnormality is hypertrophied nerve trunks in the bowel wall, which stain densely for acetylcholinesterase.
Full thickness rectal biopsy
Treatment of Hirschsprungs disease
Treatment is aimed at decompression of the bowel,
either with a stoma in ganglionic bowel just proximal to the transitional zone, or by regular washouts, until a defi nitive operation is performed.
Ano-recatal pull through
Epispadias
This is very rare, the urethral opening being on the
dorsal surface of the penis. It is usually associated with more complex penile anomalies, which may require very complex surgical correction.
Hydrocele
If the PPV remains patent, a congenital hydrocele is found clinically
If the PPV is large enough, it allows abdominal contents to prolapse into the scrotum – an inguinal hernia. Surgical correction of congenital inguinal hernia and PPV ligation are two of the commonest paediatric surgical procedures.
Undescended testis
Failure of the testis to descend fully is known as an
undescended testis. This can lead to the testis being located anywhere along the line of normal descent, down the posterior abdominal wall, in the inguinal canal or in the upper scrotum.
Ectopic testis
If the testis descends, but to an abnormal position, it is called ectopic.
Ectopic testes are very uncommon, but can be found in the perineum, in the upper part of the femoral triangle in the thigh, at the base of the penis, or in the anterior abdominal wall. An undescended testis usually has a patent processus vaginalis associated with it.
Orchidopexy
The surgical procedure for an undescended testis,
an orchidopexy, is similar in principle to a hernia operation. The operation is done via a groin approach, the testis is located, and separated from the PPV. The testis is then mobilised further on the spermatic cord to allow enough length for the testis to be placed in a scrotal pouch.
Causes of the absence of Vas deferens
Can be unilateral or bilateral
Cystic Fibrosis CFTR gene mutation are the cause in 40% of cases
Sometimes due to unilateral renal agenesis
Aetiology of biliary atresia
Biliary tree lumen is obliterated by an inflammatory cholangiopathy causing progressive liver damage
Clinical featres of biliary atresia
Well in the first few weeks of life
No FHx
CONJUGATED BILIRUBIN
Jaundice in infants > 14 days in term ( > 21 days in pre term)
Pale stool
yellow urine
Associated with cardiac malformation, polysplenia and situs invertus
Ix for biliary atresia
Conjugated bilirubin
Ultrasound of the liver
Hepato-iminidiacetic radionuclide scan
Mx of biliary atresia
Nutritional support
Kasai procedure (Roux-en-Y portojejunostomy
If this fails, liver transplant
Causes of bilious vomiting in neonates
Duodenal atresia (AXR - double bubble) - few hours after birth
Malrotation (medial DJ flexure, abnormal orientation of SMA and SMV) 3-7 days after birth
Jejunal / ileal atresia (AXR air fluid level) within 24 hours of birth
Meconium ileus (AXR air fluid levels, sweat test) 24-48 hours after birth
Necrotising enterocolitis (AXR dilated bowel loops - 2nd week of life
Where do bronchogenic cysts typically arise from?
Anamolous development of the ventral foregut
Where are bronchogenic cysts most commonly found?
Midline, most often in the region of the carina (sometimes with attachment to the tracheobronchial tree)
Presenting symptoms of bronchogenic cysts
Asymptomatic or present with respiratory symptoms early in the neonatal period
What is the most common type of foregut cysts?
Enterogenous cysts
Ix for bronchogenic cysts
Antenatal USS, others are detected on conventional CXR
Once suspected, CT
Treatment of bronchogenic cysts
Thorascopic resection, if very young wait until 6 weeks
Describe choanal atresisa
Posterior nasal airway occluded by soft tissue or bone
Associated with coloboma
Improves with crying
Tx: fenestration procedures designed to restore patency
Most common congenital deformity affected orofacial structures
Cleft lip and palate
Most common variants of cleft lip and palate?
Isolated cleft lip
Isolated cleft palate
Combined cleft lip and palate (most common)
Aetiology of cleft lip
Disruption of the muscles of the upper lip and nasolabial region
(nasolabial, bilabial and labiomental)
left more common than right
Aetiology of cleft palate
Cleft palate occurs as a result of non fusion of the two palatine shelves. Both hard and soft palate may be involved.
Complete cases are associated with complete separation of the nasal septum and vomer from the palatine processes
Treatment of cleft palate
Surgical reconstruction
At what age is majority of foreskin retractile
16
Define cryptorchidism
A congenital undescended testis that has failed to reach the bottom of the scrotum by 3 months of age
Associations of cryptorchidism
Patent processus vaginalis
Abnormal epididymis
Cerebral palsy
Mental retardation
Wilms tumour
Abdominal wall defects
Treatment of cryptorchidism
Orchidopexy at 6- 18 months of age. The operation usually consists of inguinal exploration, mobilisation of the testis and implantation into a dartos pouch.
After the age of 2 years in untreated individuals the Sertoli cells will degrade and those presenting late in teenage years may be better served by orchidectomy than to try and salvage a non functioning testis with an increased risk of malignancy
Location of ectopic testis in children
the superficial inguinal pouch,
the perineum,
the base of penis,
the opposite side of the scrotum, t
he femoral canal, and the pubopenile region
What is hypospadias?
The urethral meatus opens on the ventral surface of the penis. There is also a ventral deficiency of the foreskin
Features of hypospadias
Absent frenular artery
Ventrally opened glans
Skin tethering to hypoplastic urethra
Splayed columns of spongiosum tissue distal to the meatus
Deficiency of the foreskin ventrally
Management of hypospadias
DO NOT CIRCUMCISE
urethroplasty
prenile reconstruction
if very distal disease, no treatment required maybe
What is the hernia sac often a remnant of in children
the hernia sac is often a remnant of the processus vaginalis and association with undescended testis is recognised
Is direct or indirect more common?
indirect
Treatment for inguinal hernia
inguinal herniotomy
Treatment of inguinal hernia in female vs male
Female - explore both sides
male - only affected side to minimise risk of damage to cord structures
When are neonatal inguinal hernias operated on?
next list
Describe aetiology of intestinal malrotation
Typically the duodenal loop lies to the left of the caecum and therefore lacks 90 o of its 270o rotation. It becomes fixed in this position with peritoneal attachments (Ladds bands)
Signs of intestinal malrotation
Bilious vomiting is the cardinal symptom and sign.
Ix in intestinal malrotation
abdominal ultrasound scan to determine the relationship between the superior mesenteric artery and vein (normally SMA lies to the left of the SMV). This test is complemented with an upper GI contrast series and this aims to establish that the DJ flexure is correctly sited to the left of the vertebral bodies
Tx of intestinal malrotation
Ladds procedure
Describe Ladds procedure
The bowel is returned to the abdominal cavity in the non rotated fashion with the small bowel on the right hand side and the large bowel on the left, the caecum is positioned in the left upper quadrant. Because the caecum is located in this new location many surgeons will also perform an appendicectomy
Describe thyroglossal cysts
Located in the anterior triangle, usually in the midline and below the hyoid (65% cases)
Derived from remnants of the thyroglossal duct
Thin walled and anechoic on USS (echogenicity suggests infection of cyst)
Describe branchial cysts
Incomplete obliteration of the branchial apparatus may result in cysts, sinuses or fistulae
75% of branchial cysts originate from the second branchial cleft
Usually located anterior to the sternocleidomastoid near the angle of the mandible
Unless infected the fluid of the cyst has a similar consistency to water and is anechoic on USS
Describe dermoid cysts
Derived from pleuripotent stem cells and are located in the midline
Most commonly in a suprahyoid location
They have heterogeneous appearances on imaging and contain variable amounts of calcium and fat
Lymphatic malformations in children
Usually located posterior to the sternocleidomastoid
Cystic hygroma result from occlusion of lymphatic channels
The painless, fluid filled, lesions usually present prior to the age of 2
They are often closely linked to surrounding structures and surgical removal is difficult
They are typically hypoechoic on USS
Describe infantile haemangioma
May present in either triangle of the neck
Grow rapidly initially and then will often spontaneously regress
Plain x-rays will show a mass lesion, usually containing calcified phleboliths
As involution occurs the fat content of the lesions increases
Describe nephroblastoma
Usually present in first 4 years of life
May often present as a mass associated with haematuria (pyrexia may occur in 50%)
Often metastasise early (usually to lung)
Treated by nephrectomy
Younger children have better prognosis (<1 year of age =80% overall 5 year survival)
What cells does nephroblastoma originate from?
neural crest
Which fluids should be avoided outside the neonatal period?
Outside the neonatal period saline / glucose solutions should not be given. The greatest risk is with saline 0.18 / glucose 4% solutions
What is a toddler’s fracture?
Oblique tibial fracture in infants
What is a plastic deformity?
Stress on bone resulting in deformity without cortical disruption
What is a greenstick fracture?
Unilateral cortical breach only
What is a buckle fracture
Incomplete cortical disruption resulting in periosteal haematoma only
Salter-Harris fractures
I - Fracture through the physis only (x-ray often normal)
II - Fracture through the physis and metaphysis
III - Fracture through the physis and epiphyisis to include the joint
IV - Fracture involving the physis, metaphysis and epiphysis
V Crush injury involving the physis (x-ray may resemble type I, and appear normal)
Osteogenesis imperfecta
Defective osteoid formation due to congenital inability to produce adequate intercellular substances like osteoid, collagen and dentine.
Failure of maturation of collagen in all the connective tissues.
Radiology may show translucent bones, multiple fractures, particularly of the long bones, wormian bones (irregular patches of ossification) and a trefoil pelvis
Osteopetrosis
Bones become harder and more dense.
Autosomal recessive condition.
It is commonest in young adults.
Radiology reveals a lack of differentiation between the cortex and the medulla described as marble bone.
Intusussception
Ix signs, signs, Tx
Telescoping bowel
Proximal to or at the level of, ileocaecal valve
6-9 months age
Colicky pain, diarrhoea and vomiting, sausage shaped mass, red jelly stool.
Treatment: reduction with air insufflation (unless ileo-ileal - straight to surgery)
Treatment of Hirschsprung’s disease
Treatment is with rectal washouts initially, thereafter an anorectal pull through procedure
Treatment of meconium ileus
Infants who do not respond to PR contrast and NG N-acetyl cysteine will require surgery to remove the plugs
Treatment of Necrotising Enterocolitis
Treatment is with total gut rest and TPN, babies with perforations will require laparotomy
Angle used to determine severity of SUFE
Southwick angle
Features of pyloric stenosis
projectile’ vomiting, typically 30 minutes after a feed
constipation and dehydration may also be present
a palpable mass may be present in the upper abdomen
Biochemical features of pyloric stenosis
hypochloraemic, hypokalaemic alkalosis due to persistent vomiting
Tx of pyloric stenosis
Ramstedt pyloromyotomy
Paraumbilical hernia
These are due to defects in the linea alba that are in close proximity to the umbilicus. The edges of a paraumbilical hernia are more clearly defined than those of an umbilical hernia. They are less likely to resolve spontaneously than an umbilical hernia
Omphalitis
This condition consists of infection of the umbilicus. Infection with Staphylococcus aureus is the commonest cause. The condition is potentially serious as infection may spread rapidly through the umbilical vessels in neonates with a risk of portal pyaemia, and portal vein thrombosis. Treatment is usually with a combination of topical and systemic antibiotics
Umbilical granuloma
These consist of cherry red lesions surrounding the umbilicus, they may bleed on contact and be a site of seropurulent discharge. Infection is unusual and they will often respond favorably to chemical cautery with topically applied silver nitrate
Persistent urachus
This is characterised by urinary discharge from the umbilicus. It is caused by persistence of the urachus which attaches to the bladder. They are associated with other urogenital abnormalities
Persistent vitello-intestinal duct
This will typically present as an umbilical discharge that discharges small bowel content. Complete persistence of the duct is a rare condition. Much more common is the persistence of part of the duct (Meckels diverticulum). Persistent vitello-intestinal ducts are best imaged using a contrast study to delineate the anatomy and are managed by laparotomy and surgical closure
Criteria for paediatric septic arthritis
Kocher criteria
Features of Talipes equinovirus
Equinus of the hindfoot
Adduction and varus of the midfoot
High arch
Anatomical features of talipes equinovirus
Adducted and inverted calcaneus
Wedge shaped distal calcaneal articular surface
Severe Tibio-talar plantar flexion
Medial Talar neck inclination
Displacement of the navicular bone (medially)
Wedge shaped head of talus
Displacement of the cuboid (medially)
Tx for talipes equinovirus
Ponseti method is best described and gives comparable results to surgery
if fails, surgery
Hydrocele
Occur secondary to patent processus vaginalis
Present as fluid filling in scrotum or as cyst of the spermatic cord
Communicating hydroceles are treated by a trans inguinal ligation of the PPV
Cystic hydroceles in older children may be treated with scrotal exploration
(inguinal approach in children, scrotal in adults)
Pathophysiology of vesicoureteric reflux
ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle
therefore shortened intramural course of ureter
vesicoureteric junction cannot therefore function adequately
Ix for vesicoureteric reflux
VUR is normally diagnosed following a micturating cystourethrogram
a DMSA scan may also be performed to look for renal scarring
