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MRCS A(My Own) > Congenital Diseases > Flashcards

Congenital Diseases Flashcards

1
Q

number one cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years.

A

Meckel’s Diverticulum

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2
Q

Asymptomatic diverticula

A

Diverticulosis

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3
Q

Meckel diverticulum rule of 2

A

the Meckel diverticulum
occurs in about 2% of the population,
is about 2 inches in length,
is usually located within 2 feet of the ileocecal valve, and
usually presents before 2 years of age

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4
Q

Vitello-intestinal/Omphalomesenteric duct disappears

A

disappears entirely by weeks 8-10 of gestation.

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5
Q

Adrenal cortex zone and hormones

A

zona glomerulosa (ZG), fasciculata (ZF), and reticularis (ZR), responsible to produce aldosterone, cortisol, and sex steroid hormone

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6
Q

pain in 9 IBS

A

low dose tricyclic antidepressants if pain is a dominant symptom

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7
Q

Hernia with lung hypoplasia

A

Bochdalek

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8
Q

If absent Vas deferens and consistent chest infection
Think of

A

Cystic fibrosis

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9
Q

<5 year child with severe pain during defecation with post bright red blood

A

Anal fissure

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10
Q

current jelly type stool in which disease

A

Inflammatory bowel disease

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10
Q

current jelly type stool in which disease

A

Intussception

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11
Q

cherry red masss protruding out of anal canal

A

Juv3nile polyps as FAP

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12
Q

> 10 years old child with pain in internal rotation

A

Slipped femoral epiphysis
So get Hip Xray

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13
Q

More common in extended breech babies is which disease

A

Developmental dysplasia of the hip

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14
Q

Hip pain referr3d to knee
What diagnosis

A

If between 5 to12 years then it’s PERTHES
If it’s in obese male then it’s Slipped upper femoral epiphysis

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15
Q

flattened femoral head in xray

A

Perth3s disease

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16
Q

Heterogenous above hyoid bone neck mass

A

Dermoid

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17
Q

Mass with fat, Calcium and hair in a young age boy

A

Dermoid

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18
Q

Most commo. Branchial cyst origin site

A

2nd branchial cleft

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19
Q

Suprahyoid vs infrahyoid neck mass in children

A

Supra: Dermoid
Infra: Thyroglossal duct

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20
Q

Lesion on ant vs post site of SCM muscle

A

Ant: Branchiwl cyst near angle of mandible
Post: lymphatic malformation

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21
Q

Age for cystic hygromq

A

Less than 2 years

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22
Q

calcified phleboliths in a lesion

A

Infantil3 hemangioma

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23
Q

Exomphalos and diaphragmatic herniae are commonly associated with

A

Intestinal malrotation

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24
Q

Ligament of Treitz

A
  1. stabilize the duodenojejunal flexure and prevent it from being displaced.
  2. Transi5ion between forgut and midgut
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25
Q

UGIB vs LGIB is through which

A

Ligament of treitz attachment at duodenojejunal flexure.

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26
Q

Keypoints of esophageal atresia

A
  1. Distal Tracheaoesophageal fistula is the commonest variant
  2. High incidence of polyhydramnios.
  3. Absence of gastric fluid on antenatal U/s.
  4. High incidence of of associated imperforate anus.
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27
Q

Malrotation surgery

A

ladds procedure

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28
Q

Hirschsprung’s disease

A

Absence of ganglion cells from myenteric and submucosal plexuses

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29
Q

Diagnosis of Hirschsprung’s disease

A

Full thickness rectal biopsy

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30
Q

Surgeryfor Hirshprung

A

ANORECTAL PULLTHROUGH

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31
Q

VACTERL association is with

A

Esophageal atresia

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32
Q

Biliary atresia Rx

A

Kasai Procedure

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33
Q

main risk factor of Necrotising enterocolitis

A

Prematurity

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34
Q

most common cause of meconium plugs

A

cystic fibrosis

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35
Q

ISt line RX of Meconium ileus

A

Intestinal lavage

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36
Q

Ectopic testes site

A

base of penis, femoral triangle and perineum and superficial inguinal pouch

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37
Q

IF an ectopic lateral testes then surgery should be

A

Open Orchidopexy

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38
Q

Problems that occur during feeding are more suggestive of proximal pathology of which

A

Esophageal atresia

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39
Q

posterior urethral valves is associated with

A

Patent Urachus

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40
Q

cherry red lesions surrounding the umbilicus

A

Umbilical granuloma

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41
Q

commonest cause of Omphalitis

A

Staphylococcus aureus

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42
Q

Rx of Umbilical granloma

A

chemical cautery with topically applied silver nitrate.

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43
Q

Persistence of part of the vitello-intestinal duct

A

Meckels diverticulum

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44
Q

Time taKen by umbilical hermia to close spontaneously

A

B /w 12 months and three years)

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45
Q

congenital defects cryptorchidism is associated with

A

Patent processus vaginalis
Abnormal epididymis
Cerebral palsy
Mental retardation
Wilms tumour
Abdominal wall defects (e.g. gastroschisis, prune belly syndrome)

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46
Q

congenital undescended testis is one that has failed to reach the bottom of the scrotum by which age

A

3 months of age

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47
Q

Time when orchidopexy needs to be done in undescendent testes

A

6-18months

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48
Q

Loss of sertoli cells due to undescendant testes

A

2years

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49
Q

When orchidectomy becomes better than orchidopexy in undescended testicle

A

In late teens when nothing is Present to besalvaged

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50
Q

Rx Of indirect inguinal hernia in children

A

Herniotomy
as post wall is usually normal and its due to Patent processus vaginalis

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50
Q

Rx Of indirect inguinal hernia in children

A

Herniotomy
as post wall is usually normal and its due to Patent processus vaginalis

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51
Q

why need of bilateral exploration of indirect inguinal hernia in children

A

As most of them are bilateral but not in males

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52
Q

Why only affected side is operated in males but bilateral in females in in direct inguinal hernia in children

A

BcZ risks of cord and testicular injury in males do not justify this approach and therefore only the symptomatic side is treated.

53
Q

Causes of non bile stained vomiting in infancy

A

Infantile hypertrophic pyloric stenosis Gastro-oesophageal reflux
Feeding difficulties (technique/volume

54
Q

Causes of bile stained vomiting in infancy

A

Malrotation with volvolos
Duodenal atresia
Hirschsprung’s disease
Anorectal malformations
Meconium ileus (cystic fibrosis)
Necrotising enterocolitis

55
Q

Dx of Pyloric stenosis

A

Test feed or USS
Olive like mass is Palpable

56
Q

The first line treatment for uncomplicated meconium ileus is

A

enemas of either dilute gastrograffin or N-acetyl cysteine.

57
Q

Rx of Palpable vs non palpable undescendent testes after 6 months

A

Palpable: Orchidopexy
Impalpable: Laproscopy

58
Q

Torsion of testes most common time

A

Torsion of a testicular or epididymal appendage char acteristically affects boys just before puberty

59
Q

If UTI in children then what test to do always

A

As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI

60
Q

Diagnostic test for Vesicoureteric Reflux

A

micturating cystourethrogram

61
Q

Cause of unilateral cleft lip

A

failure of nasolabial ring fusion

62
Q

most common congenital deformity affecting the orofacial structures.

A

cleft lip and palate

63
Q

Mainstay Rx of cleft Lip and palate

A

Surgical reconstruction

64
Q

Pierre Robin sequence

A

isolated cleft palate
retrognathia and
a posteriorly displaced tongue (glossoptosis

65
Q

Primary vS Secondary Palate

A

Primary is infront of incisive foramen
Secondary is behind it

66
Q

Cleft palate is present due to

A

Cleft palate results in failure of fusion of the two palatine shelves.

67
Q

Which part if hard palate contains greater palatine neurovascular bundle.

A

maxillary fibromucosa is thick and contains the greater palatine neurovascular bundle.

68
Q

Inguinal hernia in infants

A

Inguinal hernia in infants = Urgent surgery
After 1 year of age the complication rate reduces significantly

69
Q

Pleuroperitoneal hernia through which foramen

A

foramen of Bochdalek: through the dome of the diaphragm posteriorly.

70
Q

Most common location of hypoSpadias

A

Distal and ventral surface of Penis

71
Q

Bilious vomiting in neonates

A

Bilious vomiting in neonates is a surgical emergency and is intestinal malrotation and volvulus until otherwise proven.

72
Q

What excess procedure is done in Ladds procedure of malrotation

A

Appendicectomy as caecum is now placed in upper right Quadrant

73
Q

Two tests done in melrotation and their purpose

A

1• Abdominal u/s: determine the relationship between the superior mesenteric artery and vein (normally SMA lies to the left of the SMV
2. upper GI contrast study: To check that DJ Flexure is correctly sited to left of vertebra in case midgut volvolvs has occured.

74
Q

one of colicky abdominal pain, together with a mass on clinical examination.

A

mostly think of intusscaption

75
Q

Common GI congenital issue in Down syperonne babies

A

Drodenal atresia

76
Q

Rx of Duodenal atresia

A

Duodenoduodenostomy

77
Q

Persistent vitello-intestinal ductsdiagnosis

A

contrast study to delineate the anatomy

78
Q

Common sites of Necrotising Entercolitis

A

Terminal ileum
caecum
distal colon

79
Q

Tests to done to rule outbiliary atresia or Physiological jaundice

A

Conjugated bilirubin

80
Q

Diagnosis of Biliary atresia

A
  1. u/S to rule out extrahepatic causes
  2. Hepato-iminodiacetic acid radionuclide scan (good uptake but no excretion usually seen)
81
Q

Rx of biliary atresia

A

Roux-en-Y portojejunostomy (Kasai procedure).

82
Q

Biliary atresia is confused with

A

Alagille syndrome autosomal dominant disorder

83
Q

Pyloric stenosis typically presents in

A

second to fourth weeks of life with vomiting,

84
Q

Electrolyte diS order in Pyloric stenosis

A

hypochloraemic, hypokalaemic alkalosis due to persistent vomiting

85
Q

Buckle fractures features

A

Buckle fractures will show periosteal haematoma formation only, no cortical disruption

86
Q

Toddlers fracture

A

Oblique tibial fracture in infants

87
Q

Osteogenesis imperfecta X-ray findings

A

Translucent bones
Multiple fractures, particularly long bones
Wormian bones (irregular patches of ossification)
Trefoil pelvis.

88
Q

Surgery for uncomplicated umbilical hernia

A

After 3 years of age
Not before that because strangulation is rare

89
Q

Location of cystic hygroma

A

Cystic hygromas are soft and transilluminate. Most are located in the posterior triangle.

90
Q

Cyanosis worst during feeding

A

Choanal atresia

91
Q

Midline cystic mass with air fluid level in infants

A

Bronchogenic cyst

92
Q

Midline cyst@ carina with air fluid levels

A

Bronchogenic cysts

93
Q

Bronchogenic cyst forms due to

A

anomalous development of the ventral foregut.

94
Q

Rx of Bronchogenic cysts

A

Thorascopic resection
after six weeks of age

95
Q

poor muscular development and hyper-mobile fingers at young age

A

Osteogenesis imperfecta

96
Q

Common orthopedic issue caused by breech delivery

A

Developmental dysplasia of the hip

97
Q

Rx of evelopmental dysplasia of the hip atearly age

A

Arthrodesis

98
Q

Developmental dysplasia of the hip diagnosed by

A

Barlow and Ortolani tests

99
Q

Perthes disease vs developmental dysplasia of the hip

A

In latter one have shallow acetabulum

100
Q

Most common other anomaly with hypospadias

A

Although it’s mostly isolated but if with other disease then it’s with Cryptorchidism

101
Q

Wilm’s Tumor affect which age group

A

1-4years of age

102
Q

Some features of Nephroblastoma(Wilms Tumor)

A

right sided loin pain
lethargy
haematuria
Pyrexia
Large mass in right upper quadrant

103
Q

Branchial cyst location and derivation

A

Branchial cysts are usually located laterally and derived from the second branchial cleft.

104
Q

Bronchial cyst location

A

In ant triangle
near angle of mandible

105
Q

Midline neck mass

A

Thyroglossal cyst below hyoid
Dermoids above hyoid

106
Q

Ant vs post triangle mass

A

Ant: Branchial cyst
Post: Lymphatic malformation I.e Cystic hygroma

107
Q

USS of Branchial Cyst

A

Anechoic on USS unless infected

108
Q

Fluid of choice in children with no hemodynamic instability aka maintenance

A

Any isotonic crystalloid
Both Hartmann or 0.9% Normal saline can begiven

109
Q

Indications for IV fluidsin children

A

Resuscitation and circulatory support

Replacing on-going fluid losses

Maintenance fluids for children for whom oral fluids are not appropriate

Correction of electrolyte disturbances

110
Q

Giving fluid in children causes which electrolyte abnormality

A

Hyponatraemia

111
Q

Harrison Sulcus is present @which rib area

A

6th coastal cartilage

112
Q

Some features of Rickets

A

Bowing of leg
Rickettary rosary
Harrison sulcus
widening and cupping of epiphysis on X-ray

113
Q

Most common abd emergency in children under 1year of age

A

M. C is inguinal hernia then Intussusception

114
Q

Diseasewhich Present in Istweek of life

A

Pylorir stenosis
Malrotation

115
Q

Hyperactivity and short stature are associated with

A

Perthes disease

116
Q

Xray of slipped upper femoral epiphysis

A

displacement of the femoral epiphysis inferolaterally.

117
Q

spiral fractures are caused by which type of injury

A

spiral fractures are usually the result of rotational injury

118
Q

Surgery for biliary atresia

A

Kasai Procedure

119
Q

Marble bone disease

A

Osteopetrosis

120
Q

. If post rib # in neonates

A

Non accidental injury as post rib fracture is extremely rare in neonates

121
Q

If breech baby with normal Barlow and ortolani tests then

A

Get a hip USS to rule out developmental dysplasia of hip

122
Q

which intestinal disorder with down syndrome

A

Duodenal atresia

123
Q

Kocher criteria for septic arthritis?

A

Kocher’s ‘WIFE’ is:

WCC >12
Inability to weight bear
Fever
ESR >40
= >90 chance of septic arthritis

124
Q

commonest organism ofseptic arthritis

A

Staph aureus

125
Q

Youngboy with Bloody diarrhoea and weigh loss

A

Inflammatory bowel disease

126
Q

Investigation of inflammatory bowel disease

A

Endoscopy

126
Q

Investigation of inflammatory bowel disease

A

Endoscopy

127
Q

Early plain x-ray changes in Perthes Disease:

A

Widening of the joint space.
Sub chondral linear lucency.

128
Q

Sistrunk procedure

A

Thyroglossal excision of the cyst and track

129
Q

Diseases associated with intussception

A

polyps, lymphadenopathy and cystic fibrosis

130
Q

Diagnosis of intussectionmade by

A

Ultrasound

MRCS A(My Own) (28 decks)

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