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Paediatric Surgery > Neonatal Surgery > Flashcards

Neonatal Surgery Flashcards

1
Q

What forms the primitive gut embryologically

A

Embryonic disc folds in the cephalocaudal direction and transversely to form the endoderm-lined cavity of the primitive gut

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2
Q

What are the divisions of the primitive gut

A
  • Pharyngeal gut
  • Foregut
  • Midgut
  • Hindgut
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3
Q

From where are the muscular contributions of the gut derived

A

Mesoderm

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4
Q

What does the foregut give rise to

A
  • Trachea
  • Oesophagus
  • Stomach
  • Duodenum
  • Liver
  • Pancreas
  • Spleen
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5
Q

What results from abnormal closure of the oesophagotracheal septum

A

Tracheo-oesophageal fistula

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6
Q

From where is the liver derived

A

Hepatic diverticulum - endodermal outgrowth from the distal end of the foregut

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7
Q

From where is the uncinate process of the pancreas derived

A

Ventral pancreatic bud

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8
Q

When is the connection formed between the rectum and the outside and how does this occur

A
  • Week 9

- Rupture of the anal membrane underlying the anal pit

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9
Q

Describe Gastroschisis

A
  • Defect in the abdominal wall to the right of the umbilicus
  • Bowel is eviscerated, thickened and matted
  • No sac
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10
Q

What is Gastroschisis associated with

A

Intestinal atresias (10%)

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11
Q

How is Gastroschisis treated

A

Preformed spring-loaded silos allows the bowel to reduce into the abdomen by gravity. The defect can then be closed.

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12
Q

Describe exomphalos (omphalocele)

A

Hernia inclusive of bowel into the base of the umbilical cord, i.e. covered by a sac

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13
Q

How is exomphalos (omphalocele) categorised

A
  • Major = defect >5cm

- Minor = defect <5cm

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14
Q

What is exomphalos (omphalocele) associated with

A
  • Trisomies

- Cardiac defects

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15
Q

How is exomphalos (omphalocele) treated

A

Closure of the defect in one or more stages (if the abdomen is not large enough to accept the bowel then it can be left to epithelialise and the contents be gradually reduced before 1 year)

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16
Q

List the VACTERL conditions

A
  • Vertebral anomalies
  • Anorectal anomalies
  • Cardiac
  • Tracheo-Esophageal
  • Renal
  • Limb
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17
Q

What maternal feature is associated with oesphageal atresia

A

Maternal polyhydramnios

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18
Q

How does oesophageal atresia present

A

Present at birth with a ‘mucousy’ baby, choking or turning blue on feed

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19
Q

What is oesophageal atresia

A

A section of the oesophagus is missing resulting in it ending in a blind-ended pouch

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20
Q

What percentage of babies with oesophageal atresia will have a tracheo- oesophageal fistula

A

75%

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21
Q

How is oesophageal atresia diagnosed

A

Confirmed by trying to pass and NG tube and taking a CXR. There are tube coils in the upper thorax. Gas in the stomach indicates a fistula between the trachea and distal oesophagus.

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22
Q

How is oesophageal atresia treated

A

Disconnection of the tracheo-oesophageal fistula and anastomosis of the upper and lower oesophagus through a right thoracotomy

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23
Q

What are the complications associated with oesophageal atresia surgery

A
  • Anastomotic leak
  • Anastomotic stricture
  • GORD
  • Recurrent fistula
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24
Q

What chromosomal abnormality is associated with duodenal atresia

A

Downs Syndrome

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25
Q

What is duodenal atresia

A

Congenital absence of complete closure of a portion of the lumen of the duodenum

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26
Q

How does duodenal atresia present

A

At birth with bile-stained vomiting

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27
Q

How is the diagnosis of duodenal atresia confirmed

A

AXR - ‘double bubble sign’ of gas in the stomach and proximal duodenum

28
Q

How is duodenal atresia treated

A

Side-to-side duodenoduodenostomy

29
Q

Describe low and intermediate anorectal anomalies in boys

A
  • Rectum present and passes through a normal sphincter complex
  • Fistulous track to the surface of the perineum, usually anteriorly onto the scrotum
30
Q

How are low and intermediate anorectal anomalies treated in boys

A

If meconium is visible a local ‘cut-back’ procedure can be performed to open the fistula back to the rectum in anticipation of normal continence

31
Q

In high anorectal anomalies, where does the fistulous track develop

A

Rectum to urethra

32
Q

How is faeces diverted in imperforate anus

A

High sigmoid colostomy

33
Q

Describe the pathophysiology of midgut malrotation

A
  • As the physiological midgut hernia reduces, the mesentery usually rotates to bring the caecum to the RIF and the DJ flexure to left of the midline
  • Failure of this rotation leaves the caecum high in the RUQ and the DJ flexure mobile in the midline
34
Q

How is midgut malrotation diagnosed

A

Upper GI contrast study and USS

35
Q

How is midgut malrotation managed

A

Urgent laparotomy to untwist the bowel (Ladd procedure if volvulus is present)

36
Q

How does Meconium ileus present

A

Delayed passage of meconium and abdominal distension

37
Q

How is Meconium ileus treated non surgically

A
  • Gastrograffin enema

- NG N-acetyl cysteine

38
Q

What is the surgical management of Meconium ileus

A
  • Surgical removal of the plugs

- Temporary ileostomies

39
Q

What chromosomal disorder is associated with Hirschsprung’s disease

A

Down’s Syndrome

40
Q

What causes Hirschsprung’s disease

A
  • Failure of ganglion cells (neural crest origin) to migrate down the hindgut
  • Causes a functional obstruction at the junction between normal and aganglionic bowel
41
Q

Describe the distribution of bowel affected by Hirschsprung’s disease

A
  • 80% rectum/sigmoid

- 20% involves entire colon

42
Q

How does Hirschsprung’s disease present

A

Presents in the first few days of life with:

  • Low intestinal obstruction
  • Failure to pass meconium
  • Abdominal distension
  • Bile-stained vomiting
43
Q

How is the diagnosis of Hirschsprung’s disease confirmed

A

Rectal biopsy - no ganglion cells in the submucosa (suction biopsy for neonates; strip biopsy if older)

44
Q

How is Hirschsprung’s disease treated

A

Three-stage surgical procedure:

  1. Defunctioning colostomy with multiple biopsies
  2. Pull-through procedure to bring down ganglionic bowel
  3. Closure of the colostomy
45
Q

What is the main complication of Hirschsprung’s disease

A

Enterocolitis associated with C.diff toxin

46
Q

Describe Necrotising Enterocolitis

A

Acute inflammatory condition of the neonatal bowel that may be associated with areas of bowel necrosis and a systemic inflammatory response syndrome

47
Q

What is the primary risk factor for necrotising enterocolitis

A

Prematurity

48
Q

List the signs and symptoms of necrotising enterocolitis

A
  • Feed intolerance
  • Delayed gastric emptying
  • Abdominal distension, tenderness and ileus
  • Blood PR
  • Peritonitis if perforation occurs
49
Q

How is necrotising enterocolitis managed

A
  • Supportive
  • NBM with parenteral nutrition
  • Surgical resection of necrotic bowel
50
Q

What is the key determinant of survival in necrotising enterocolitis

A

Birthweight

51
Q

How are congenital diaphragmatic hernias categorised

A
  • Posterolateral (Bochdalek)

- Anterior (Morgagni)

52
Q

What is the most common type of congenital diaphragmatic hernia

A

Posterolateral (Bochdalek) - 90% left sided

53
Q

How is congenital diaphragmatic hernia managed

A

Repair of the diaphragmatic defect after a few days with either suture or prosthetic gore-tex patch

54
Q

When do the facial primordia appear during development

A

4th week

55
Q

List the 5 facial primordia

A
  1. Frontonasal prominence
  2. Maxillary prominences (paired)
  3. Mandibular prominences (paired)
56
Q

When does the palate develop

A

Between weeks 5 and 12

57
Q

What are the two major groups of cleft lip and palate

A
  1. Clefts involving the upper lip and anterior part of the maxilla, with or without palate involvement
  2. Clefts involving hard and soft regions of the palate
58
Q

What deficiency in pregnancy may be implicated in the development of cleft lip/palate

A

Vitamin B complex

59
Q

What is the risk of having a second affected child with cleft lip compared to the general population

A

4% Vs. 0.1%

60
Q

What causes cleft lip

A

Failure of merging the medial nasal prominence and maxillary prominence

61
Q

What conditions are associated with neonatal intestinal malrotation

A
  • Exomphalos

- Diaphragmatic hernia

62
Q

What condition is associated with a scaphoid abdomen

A

Bochdalek hernia (herniation of the abdominal contents into the chest)

63
Q

What causes unilateral cleft lip

A

Failure of nasolabial muscle ring fusion

64
Q

What structures may a Bochdalek hernia contain

A

Stomach

65
Q

What structures may a Morgagni hernia contain

A

Transverse colon

Paediatric Surgery (5 decks)

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