Paeds Flashcards
(186 cards)
1
Q
What are the types of craniosynostosis?
A
Scaphocephaly, plagiocephaly, brachycephaly, trigonocephaly, pachycephaly, cloverleaf
Scaphocephaly (sagittal - most common), plagiocephaly (unilateral lambdoid or coronal - harlequin eye), brachycephaly (bicoronal), trigonocephaly (metopic), pachycephaly (lambdoid), cloverleaf (sagittal, coronal and lambdoid)
2
Q
What syndromes are associated with craniosynostosis?
A
Apert, Carpenter, Crouzon, choanal atresia, hypophosphatasia, sickle cell, thalassaemia
Associations include various syndromes and conditions.
3
Q
What is the management for craniosynostosis?
A
Refer to plagiocephaly clinic, low-dose CT of the skull with 3D reformats for surgical planning
Management often involves specialized clinics.
4
Q
What is the difference between cephalohaematoma and subgaleal haematoma?
A
Cephalohaematoma: traumatic subperiosteal haematoma; Subgaleal haematoma: haemorrhage in potential space, lethal emergency
Cephalohaematoma does not cross sutures, while subgaleal haematoma does.
5
Q
What are the findings for incomplete partition type 2 (Mondini malformation)?
A
Cochlear hypoplasia, enlarged vestibule, enlarged vestibular aqueduct
Normal basal turn of 1.5 instead of 2.5 turns.
6
Q
What is the degree of tonsillar descent in Chiari I/II?
A
> 6mm below McRae line
McRae line is defined between the basion and opisthion.
7
Q
What are the differential diagnoses for tonsillar descent in Chiari I/II?
A
Paget, fibrous dysplasia, RA, rickets, OI, osteomalacia, achondroplasia, cleidocranial dysostosis, hyperparathyroidism
A variety of conditions can mimic tonsillar descent.
8
Q
What are the findings in septo-optic dysplasia?
A
Absent septum pellucidum, abnormal/hypoplastic optic chiasm and nerves, schizencephaly
Schizencephaly occurs in about 50% of cases.
9
Q
What associations are linked with grey matter heterotopia / focal cortical dysplasia?
A
Agenesis of the corpus callosum, pachygyria, schizencephaly, polymicrogyria, Chiari II
These conditions often co-occur with grey matter abnormalities.
10
Q
What is the pathology of grey matter heterotopia / focal cortical dysplasia?
A
Abnormal grey matter due to shortening or prolonged migration from the germinal zone to the pia
This affects the normal distribution of grey matter.
11
Q
What are the subtypes of grey matter heterotopia / focal cortical dysplasia?
A
Periventricular nodular, band, subcortical nodular, pial
Each subtype has distinct characteristics.
12
Q
When does normal myelination complete in the brain?
A
Occipital white matter at 12 months, frontal at 18 months
Myelination occurs in a specific sequence throughout brain development.
13
Q
What are the types of lissencephaly?
A
Type 1 (undermigration), Type 2 (overmigration)
Type 1 is characterized by a smooth brain appearance.
14
Q
What genetic mutations are associated with lissencephaly?
A
LIS1 (chr 17) and doublecortin (DCX)
These mutations result in the classical pattern of lissencephaly.
15
Q
What is the life expectancy for individuals with lissencephaly?
A
10 years
This is generally due to associated complications.
16
Q
What are the types of schizencephaly?
A
Open lip (80%), closed lip (20%)
Open lip is more severe than closed lip.
17
Q
What are the associations of schizencephaly?
A
Absent septum pellucidum, bilateral schizencephaly, hypothalamic-pituitary axis abnormality
These associations highlight the broad impact of schizencephaly.
18
Q
What is the pathology of colpocephaly?
A
Disproportionate prominence of occipital horns of lateral ventricles
This is due to a wide range of congenital insults.
19
Q
What are the clinical presentations of colpocephaly?
A
Visual, cognitive, motor abnormalities, seizures
These symptoms reflect the neurological impact of colpocephaly.
20
Q
What is the pathology of porencephaly?
A
Intracranial cystic degeneration and encephalomalacia, lined by white matter
This occurs due to cerebral insult after 24 weeks.
21
Q
What findings are associated with aqueductal stenosis?
A
Funnelling of cerebral aqueduct, severe hydrocephalus, thinned cortical mantle
The third and lateral ventricles are affected, while the fourth ventricle is normal.
22
Q
What are the differential diagnoses for vascular malformations?
A
Lymphatic, venous, AVM/AVF
Differentiating these requires careful analysis on imaging.
23
Q
What are the characteristic findings of vascular malformations?
A
Fluid-fluid levels, phleboliths, minimal mass effect
These findings can help in diagnosing the type of vascular malformation.
24
Q
What is the pathology of vein of Galen malformation?
A
Persistence of AVF between choroidal arteries and median prosencephalic vein
This leads to blood shunting and failure to regress.
25
What are the types of vein of Galen malformation?
Choroidal, mural
## Footnote
Each type presents at different ages.
26
What is the clinical presentation of vein of Galen malformation?
High output cardiac failure, cranial bruit, hydrocephalus, developmental delay
## Footnote
These symptoms can result from the hemodynamic changes.
27
What is the grading system for germinal matrix haemorrhage?
1 - caudothalamic groove, 2 - <50% ventricles, 3 - >50% ventricles + ventriculomegaly, 4 - parenchymal extension
## Footnote
Each grade indicates severity and potential outcomes.
28
What is the mortality rate per grade for germinal matrix haemorrhage?
1 = 3%, 2 = 10%, 3 = 20%, 4 = 35%
## Footnote
Higher grades correlate with increased mortality risk.
29
What causes intraparenchymal extension in germinal matrix haemorrhage?
Venous infarction due to compression of deep terminal veins
## Footnote
This occurs as a result of expanded blood-filled ventricles.
30
What is the germinal matrix?
Embryological structure located lateral to lateral ventricles that gives origin to neural and glial cells
## Footnote
It is particularly fragile and prone to hemorrhage.
31
When does the germinal matrix regress?
34-36 weeks
## Footnote
This is a critical period in fetal development.
32
What is the grading for periventricular leukomalacia?
1 - periventricular echogenicity, 2 - small periventricular cysts, 3 - increased periventricular echogenicity with extensive cysts, 4 - extensive subcortical cysts
## Footnote
Each grade reflects the severity of white matter injury.
33
What is the pathology of periventricular leukomalacia?
Due to hypoxic-ischaemic injury at the watershed areas
## Footnote
This is commonly seen in premature infants.
34
What are the findings in ANEC?
T2/DWI hyperintensities within the thalami, putamina, internal and external capsules, cerebellum, brainstem
## Footnote
These findings are indicative of acute necrotizing encephalopathy.
35
What are the features of X-linked adrenoleukodystrophy?
ALD, metachromatic, Alexander, Canavan
## Footnote
Each type presents with distinct imaging characteristics.
36
What findings are associated with CMV infection?
Periventricular/temporal cysts, calcification, white matter inflammation
## Footnote
Infection during the first trimester can lead to neuronal migration disorders.
37
What is the pathology of cerebellitis?
Viral or post-vaccination in children <6 years
## Footnote
Various viruses including coxsackie and measles may be involved.
38
What are the differential diagnoses for paediatric posterior fossa masses?
Pilocytic astrocytoma, ependymoma, diffuse midline glioma, ATRT
## Footnote
Medulloblastoma is the most common posterior fossa mass.
39
What is the epidemiology of medulloblastoma?
3-7 years in childhood or 30-40 years in adults
## Footnote
The age distribution is significant for diagnosis.
40
What are the subtypes of medulloblastoma?
WNT, SHH, group 3, group 4
## Footnote
Each subtype has distinct clinical and prognostic implications.
41
What are the histopathological features of medulloblastoma?
Small round blue cells with Homer Wright rosettes
## Footnote
These features are crucial for diagnosis.
42
What is the management for medulloblastoma?
MRI of entire neuraxis before biopsy
## Footnote
This approach helps in assessing the extent of disease.
43
What are the subtypes of ependymoma?
Posterior fossa, supratentorial, spinal cord
## Footnote
Each subtype has different locations and implications.
44
What is the management for desmoplastic infantile ganglioglioma/astrocytoma?
Surgical resection and chemo, good prognosis
## Footnote
Despite high mortality risk from the procedure, outcomes are generally favorable.
45
What is the pathology of diffuse midline glioma?
WHO grade 4 in young children, H3-K27 altered
## Footnote
This tumor type has a poor prognosis.
46
What age group is primarily affected by choroid plexus papilloma/carcinoma?
85% occur under 5 years old
## Footnote
This age distribution is key for diagnosis.
47
What is the pathology of choroid plexus papilloma/carcinoma?
Frond/cauliflower-like appearance, lined by columnar/cuboidal epithelium
## Footnote
This resembles normal choroid plexus structures.
48
Where do choroid plexus papilloma/carcinoma typically occur in children vs adults?
Lateral ventricles in children, fourth ventricle in adults
## Footnote
This distinction is important for surgical planning.
49
What are the differential diagnoses for retinoblastoma?
PHPV, Coat's disease, retinopathy of prematurity, retinal detachment
## Footnote
These conditions can present similarly to retinoblastoma.
50
What is the differential diagnosis for orbital masses in retinoblastoma?
Capillary haemangioma, lymphangioma, venous varix, cavernoma, lymphoma, rhabdomyosarcoma, sarcoid, mets IgG4, Erdheim Chester
## Footnote
Multiple conditions can mimic the appearance of retinoblastoma.
51
How often is retinoblastoma bilateral?
30-40% bilateral, 5% trilateral
## Footnote
This is associated with mutations in the RB tumour suppressor gene.
52
What are the clinical presentations of hypothalamic hamartoma?
Gelastic seizures, visual problems, precocious puberty
## Footnote
These symptoms can significantly impact quality of life.
53
What are the subtypes of rhabdomyosarcoma?
Embryonal, alveolar, pleomorphic
## Footnote
Each subtype has distinct characteristics and prognoses.
54
What are the common locations for rhabdomyosarcoma?
Head and neck, genitourinary, extremities
## Footnote
The head and neck region is the most common site.
55
When would you not perform a biopsy for rhabdomyosarcoma?
If paratesticular
## Footnote
This is due to the high likelihood of malignancy in this location.
56
What are the aggressive variants of haemangioma?
Kaposiform haemangioendothelioma, epithelioid haemangioendothelioma
## Footnote
These variants can have significant clinical implications.
57
What age group is most affected by haemangioma?
Fetal and neonates; most frequent liver neoplasm in infants <6 months
## Footnote
This highlights the importance of early diagnosis.
58
What are the common locations for haemangioma?
Liver, musculoskeletal
## Footnote
These sites are critical for clinical evaluation.
59
What is the differential diagnosis for haemangioma?
Hepatoblastoma, neuroblastoma mets
## Footnote
Differentiation is important for management.
60
What is the differential diagnosis for juvenile nasopharyngeal angiofibroma (JNA)?
Haemangioma, sarcoma
## Footnote
These conditions can present similarly in imaging.
61
Where does JNA originate?
Sphenopalatine foramen
## Footnote
It can extend into the nasopharynx and pterygopalatine fossa.
62
What is the blood supply for JNA?
External carotid (internal maxillary, ascending pharyngeal, palatine), internal carotid
## Footnote
Understanding the blood supply is essential for surgical planning.
63
What is the management for JNA?
Pre-operative embolisation, ENT referral for surgical resection
## Footnote
This approach helps minimize bleeding during surgery.
64
Retropharyngeal abscess: Organisms?
Group A Strep, S aureus, H influenzae, anaerobes, TB
65
Retropharyngeal abscess: Management?
IV antibiotics +/- transoral surgical drainage
66
Epiglottitis: Age group?
3-6 years
67
Epiglottitis: Organisms?
H influenzae, group A Strep
68
Fibromatosis colli: Cause?
Due to birth trauma (forceps, difficult labour, malposition), resolves in 4-8 months
69
Thyroglossal duct cyst: Management?
Ultrasound of thyroid bed to look for normal thyroid. Surgical resection (<1% undergo malignant transformation into papillary thyroid carcinoma)
70
Tethered cord: Causes?
Closed spinal dysraphisms, arachnoid adhesions, diastematomyelia
71
Diastematomyelia: Subtypes?
1 - duplicated dural sac with common midline osseous/fibrous spur, vertebral anomalies, symptomatic. 2 - single dural sac, less marked impairment
72
Caudal regression syndrome: Subtypes?
1 - high conus termination above L1, 2 - low-lying tethered cord below S2
73
Caudal regression syndrome: Associations?
Anorectal anomalies, diastematomyelia, myelomeningocele, tethered cord, Currarino triad, VACTERL, OEIS
74
Currarino (ASP) triad: What is it?
Anorectal malformation, sacrococcygeal defect, presacral mass
75
RDS: When does the humerus head ossify?
Earliest at 37 weeks
76
RDS: Complications?
Pulmonary interstitial emphysema, bronchopulmonary dysplasia
77
Pulmonary interstitial emphysema: Risk factors?
Reduced lung compliance, prematurity, low birth weight, meconium aspiration syndrome, pneumonia
78
Pulmonary interstitial emphysema: Mechanism?
Mechanical ventilation in first weeks of life causes increased pressure and poor compliance, alveolar rupture, air escapes into interstitium and lymphatics
79
Meconium aspiration: Ddx in a term neonate?
TTN, pneumonia
80
Aspirated foreign body: Next step?
Expiratory radiograph if cooperative, if not then lateral decubitus with hyperlucent lung down
81
Aspirated foreign body: How is a decubitus film performed?
Plate in front, x-ray shot from the back
82
Bronchiolitis: Organism?
RSV
83
Round pneumonia: Pathology?
Collateral airways not developed
84
Oesophageal atresia with tracheo-oesophageal fistula: Associations?
VACTERL, CHARGE, T21, T18, intestinal atresias, pyloric stenosis, annular pancreas
85
VACTERL: What is it?
Constellation of congenital anomalies, need 3 or more of: vertebral anomalies, anal atresia, cardiac anomalies, cleft lip, tracheo-oesophageal fistula, renal anomalies, radial ray, polydactyly, hydrocephalus
86
TOF: Types?
A - oesophageal atresia, B - proximal fistula/distal atresia, C - proximal atresia/distal fistula, D - double fistula with intervening atresia, E - isolated (H-type) fistula
87
TOF: Management?
Echo and renal ultrasound, bronchoscopy, surgical correction
88
CPAM: Associations?
Sequestration, renal agenesis, polyhydramnios, hydrops, adenocarcinoma, pleuropulmonary blastoma
89
CPAM: Pathology?
Failure of normal bronchoalveolar development
90
CPAM: Classification?
1 - large cysts, 2 - <2cm, 3 - microcysts <5mm, 4 - unlined cyst, 0 - lethal
91
CPAM: Further imaging if patient needs surgery?
CT chest with contrast
92
Sequestration: Intralobar vs extralobar?
Intralobar: 75%, Extralobar: 25%
93
CLO: Associations?
Aberrant left pulmonary artery, VSD, PDA, ToF
94
CLO: Most common lobe?
LUL
95
Pleuropulmonary blastoma: Subtypes?
1 - cystic, 2 - mixed, 3 - solid
96
Appropriate position of UAC?
T8-T10 or L3-L5
## Footnote
UAC stands for umbilical artery catheter
97
Appropriate position of UVC?
Inferior atriocaval junction
## Footnote
UVC stands for umbilical vein catheter
98
Differential diagnosis for cardiomegaly?
Ebstein, cardiomyopathy, ASD/VSD/PDA, heart failure
99
Types of ASD associated with cardiomegaly?
* Secundum (most common, associated with T21)
* Primum
* Sinus venosus (associated with PAPVR)
* Coronary sinus type
* PFO
100
Epidemiology of VSD and ASD?
VSD more common than ASD and presents in infancy; ASD in adulthood
101
Features of Tetralogy of Fallot?
* VSD
* Overriding aorta
* RVH (elevated apex)
* Pulmonary hypoplasia (pulmonary bay)
102
Other congenital cyanotic diseases associated with Tetralogy of Fallot?
* Increased vascularity (TAPVR, TGA, TA, single ventricle)
* Decreased vascularity (ToF, Ebstein)
103
Types of TAPVR?
* Supracardiac (50%, left vertical vein, snowman sign)
* Cardiac (30%, drainage into coronary sinus)
* Infracardiac
* Mixed
104
Associations of right aortic arch?
CHD, heterotaxy
105
Other vascular rings/slings associated with right aortic arch?
* Aberrant subclavian
* Innominate artery compression
* Double aortic arch (most common vascular ring)
* Pulmonary sling (anterior oesophageal impression)
106
Next step in case of dextrocardia?
Echo (small 5% risk of cardiac anomalies)
107
Findings in pneumomediastinum?
Spinnaker sign
108
Differences between pneumomediastinum, pneumopericardium, and pneumothorax?
* Pneumomediastinum: thymic wing sign, haystack sign, gas doesn't shift on movement
* Pneumopericardium: gas in pericardial sac doesn't rise above pericardial reflection, gas shifts immediately
* Pneumothorax: visible pleural edge
109
Causes of pneumomediastinum?
* Trauma
* Oesophageal or tracheobronchial perforation
* Vigorous exercise
* Asthma
* Barotrauma
* Infection
* Connective tissue disorders
* Spontaneous
110
Management of pneumomediastinum?
Conservative unless tension (then requires drain)
111
How to differentiate lymphoma from thymus?
* Thymus is fat density
* Insinuates between structures (no mass effect)
* Conforms to inner ribs (undulating lateral margin)
* Forms inferior notch with cardiac border
* Sail sign
* No tracheal deviation
112
Next step for thoracic neuroblastoma?
MRI to look for spinal involvement and pre-op planning
113
Differential diagnosis for hepatoblastoma?
* Infantile hepatic haemangioma
* Mesenchymal hamartoma (<2yo)
* Neuroblastoma mets
* HCC (>5yo with cirrhosis)
* Undifferentiated embryonal sarcoma (>5yo)
114
Associations of hepatoblastoma?
* Wilms
* Beckwith-Wiedemann
* Hemihypertrophy
* FAP
* Fetal alcohol syndrome
* Gardner
* Glycogen storage disease
* Biliary atresia
115
Natural history of infantile hepatic haemangioma?
Starts in utero, rapid proliferative growth in first 6 months of life, then regress and involute
116
Markers for infantile hepatic haemangioma?
Vascular endothelial growth factor (VEGF)
117
Ultrasound findings for biliary atresia?
* Triangular cord sign
* Gallbladder ghost triad (absent gallbladder, irregular/lobular contour, lack of smooth echogenic mucosal lining)
118
Nuclear medicine findings for biliary atresia?
Tc-99m HIDA scan shows hepatic uptake with no excretion into the bowel at 24 hours
119
Age group for neuroblastoma?
Congenital to 5 years
120
Common locations for neuroblastoma?
* Adrenals (35%)
* Retroperitoneum (30%, including organ of Zuckerkandl)
* Posterior mediastinum (20%)
* Neck
* Pelvis
121
Associations with neuroblastoma?
* Hirschsprung
* DiGeorge
* Beckwith-Wiedemann
* NF1
122
Genetics of neuroblastoma?
* Amplification of N-myc oncogene
* Deletion of chr1
123
Management of neuroblastoma?
* Correlate with urine catecholamines
* Staging CT CAP (single phase)
* Bone scan for metastases
* MIBG to monitor progress
* Paediatric oncology referral for surgery + chemo
124
Theranostic MIBG tracers?
* I-123 with I-131
* Ga-68 DOTATATE with Lu-177 DOTATATE
125
Staging systems for neuroblastoma?
Evans or International Neuroblastoma (post-op) staging system
126
Differential diagnosis for Wilms tumor?
* Neuroblastoma
* Mesoblastic nephroma (solid)
* Cystic nephroma (cystic)
* Clear cell sarcoma
* Rhabdoid tumour
* AML
* RCC
* Ossifying renal tumour of infancy
* Lymphoma
127
How to differentiate Wilms from neuroblastoma?
* Wilms: usually 4yo, never congenital, well-circumscribed, claw sign
* Neuroblastoma: usually <2yo, can occur in utero, poorly marginated
128
Typical age group for Wilms tumor?
3-4 years
129
Associations with Wilms tumor?
* Beckwith-Wiedemann
* WAGR
* Denys-Drash
* Sotos
* Cryptorchidism
* Nephroblastomatosis
130
Genetics of Wilms tumor?
2% familial, due to WT1 and WT2 genes (chr11)
131
Management of Wilms tumor?
* Staging CT CAP (single phase)
* Bone scan for metastases
* Paediatric oncology referral for nephrectomy and chemo
132
Staging for Wilms tumor?
* Stage 1: kidney only
* Stage 2: local spread
* Stage 3: LN or confined to abdomen
* Stage 4: haematogenous mets
* Stage 5: bilateral renal involvement
133
When is nephrectomy possible for Wilms tumor?
Stage 1 and 2
134
Prognosis for Wilms tumor?
Cured in 90%
135
Follow-up for Wilms tumor patients?
Ultrasound every 3 months for 2 years, check contralateral kidney for nephroblastomatosis
136
Management of nephroblastomatosis?
Follow up ultrasound until 8 years old to check for growth suggestive of transformation to Wilms
137
Typical age group for mesoblastic nephroma?
Most diagnosed within 3 months (90% within 1 year)
138
Differential diagnosis for cystic nephroma?
* MCDK
* Cystic Wilms
139
Typical age group for cystic nephroma?
Boys 3 months to 5 years and middle-aged women
140
Genetics of cystic nephroma?
DICER1 mutation
141
Nuclear medicine findings for MCDK?
MAG3 or DTPA shows obstructive uropathy
142
Differential diagnoses for bilateral enlarged echogenic kidneys in ARPKD?
* Duplex kidneys
* Glomerulonephritis
* Acute tubular necrosis
* Acute cortical necrosis
* Acute interstitial nephritis
143
Associations of ARPKD?
* Congenital hepatic fibrosis
* Caroli disease
* Multiple biliary hamartomas
* Beckwith-Wiedemann
* Meckel Gruber
144
Other renal variants associated with duplex kidney?
* Agenesis
* Supernumerary
* Horseshoe
* Cross fused ectopia
* Pancake
* Pelvic
* Persistent fetal lobulation
* Hypertrophied column of Bertin
* Accessory renal arteries
* Retroaortic/circumaortic left renal vein
145
What is the Weigert-Meyer rule?
Upper moiety has inferomedial ectopic insertion into a ureterocele and is obstructed; lower moiety has normal insertion and refluxes
146
Associations of vesicoureteric reflux?
* Duplex collecting system
* Ureteral obstruction
* Posterior urethral valves
147
Grading of vesicoureteric reflux?
* 1: ureter
* 2: renal pelvis
* 3: dilatation of ureter and pelvicalyceal system
* 4: tortuous ureter, blunting of fornices
* 5: severe dilatation, loss of papillary impressions
148
Pathology of vesicoureteric reflux?
Due to abnormal maturation of VUJ resulting in a short distal ureteric submucosal tunnel
149
Management of vesicoureteric reflux?
* Renal ultrasound
* MAG 3
* Prophylactic antibiotics
* Endoscopic injection of bulking/sclerosing agent
* Surgical referral for potential reimplantation
150
Complications of vesicoureteric reflux?
* UTI/pyelonephritis
* Renal scarring
* Renal failure
151
How to perform an MCU?
* Check indication
* Check prophylactic antibiotics
* Nurse/mother assistance to stabilize child
* Sterile technique
* 5Fr feeding tube used as a catheter
* Water-soluble contrast administered
* Intermittent screening images
152
Describe types of renal nuclear medicine scans for PUJ obstruction?
* MAG3: secreted, glomerular filtration and tubular function
* DMSA: tubular function for scarring
* DTPA: filtered, glomerular filtration
153
Associations of posterior urethral valves?
* Aneuploidy (T21)
* Bowel atresia
* Craniospinal defects
154
Pathology of posterior urethral valves?
Failure of regression of the mesonephric duct forms a thick valve-like membrane
155
Associations of cryptorchidism?
* Prematurity
* IUGR
* Maternal smoking/alcohol/diabetes
* Noonan syndrome
156
Prognosis for cryptorchidism?
Most descend within first 3 months; orchiopexy if not descended after 1 year
157
What is the testicular appendage?
Remnant of embryonic Mullerian (paramesonephric) duct
158
What are the X-ray signs of pneumoperitoneum?
Rigler sign, football sign, triangle sign, falciform ligament sign, urachus sign, hepatic edge sign
## Footnote
These signs indicate the presence of free air in the peritoneal cavity.
159
What are the risk factors for NEC?
Prematurity, congenital heart disease, perinatal asphyxia, IUGR, placental insufficiency
## Footnote
These factors increase the likelihood of developing necrotizing enterocolitis.
160
What is the pathology of NEC?
Ischaemic and infective causes, inflammation from mucosa leading to transmural necrosis, most common in terminal ileum
## Footnote
Gas-forming organisms are likely involved in the infection.
161
What are the ultrasound findings in hypertrophic pyloric stenosis?
Single muscular wall >3mm, pyloric transverse diameter >13mm, longitudinal measurement >15mm
## Footnote
These measurements indicate hypertrophy of the pylorus.
162
What ultrasound probe is used for hypertrophic pyloric stenosis?
High frequency (6-10 MHz) linear probe
## Footnote
This probe provides the necessary resolution for visualizing the pylorus.
163
How can you differentiate hypertrophic pyloric stenosis from pylorospasm?
Pylorus appears sonographically normal, can distend the stomach with fluid to open the pylorus
## Footnote
This distinction is important for accurate diagnosis.
164
What is the typical clinical presentation of hypertrophic pyloric stenosis?
4-8 weeks old with projectile vomiting, paradoxical aciduria
## Footnote
These symptoms are characteristic of the condition.
165
What are the risk factors for hypertrophic pyloric stenosis?
First-born, male, C-section, family history
## Footnote
These factors contribute to the likelihood of developing the condition.
166
What is the management for hypertrophic pyloric stenosis?
Paediatric surgical referral for pyloromyotomy
## Footnote
This procedure relieves the obstruction caused by the hypertrophied pylorus.
167
What is the incidence of malrotation and volvulus?
1:500 births
## Footnote
This statistic highlights the relative rarity of the condition.
168
What are the associations with malrotation and volvulus?
Duodenal atresia/stenosis/web, gastroschisis, omphalocele, biliary atresia, pancreatic hypoplasia/agenesis, congenital diaphragmatic hernia (CDH), heterotaxy
## Footnote
These associated conditions can complicate the clinical picture.
169
How would you perform an upper GI contrast study?
Contrast (10mL omnipaque 300) via NGT, low dose fluoroscopy, patient supine, then right decubitus for pylorus visualization
## Footnote
This method helps in assessing for malrotation and volvulus.
170
What is the normal position of the DJ flexure?
Passes midline to left of L2 vertebral body pedicle, D2-D4 is retroperitoneal
## Footnote
Understanding normal anatomy is crucial for identifying malrotation.
171
What are Ladd bands?
Fibrous peritoneal tissue that attach the caecum to the retroperitoneum in the RLQ, passing over D2 in malrotation
## Footnote
These can cause extrinsic obstruction in malrotation.
172
What is a differential diagnosis for the double bubble sign in duodenal atresia?
Duodenal web, annular pancreas, malrotation/volvulus
## Footnote
These conditions can present with similar radiological findings.
173
What are the associations with duodenal atresia?
VACTERL, Down syndrome (T21), annular pancreas
## Footnote
These syndromes often have concurrent anomalies.
174
What is the pathology of duodenal atresia?
Failure to canalize at 11-12 weeks, compared to distal small bowel atresias due to ischaemic insult
## Footnote
This developmental defect leads to obstruction.
175
What features favor ileocolic intussusception over ileoileal?
Larger anteroposterior diameter >2.5cm, hyperechoic core (mesenteric fat), thicker outer hypoechoic wall, greater interssusceptum length (>3cm), internal lymph nodes
## Footnote
Recognizing these features aids in diagnosis.
176
What is the age group most affected by intussusception?
3 months to 3 years
## Footnote
This demographic is particularly vulnerable.
177
What is the management for intussusception in a child?
Inform parents, paediatric surgeons, air reduction enema
## Footnote
This approach is critical for timely intervention.
178
How would you perform an air enema for intussusception?
Ensure no complications, hydrate child, informed consent, insert Foley catheter, insufflate at 4L/min, retry if necessary
## Footnote
This procedure is a non-invasive treatment option.
179
What are the differential diagnoses for microcolon in meconium ileus?
Ileal atresia, anal atresia, Hirschsprung, meconium plug
## Footnote
Identifying the underlying cause is essential for management.
180
How would you perform a lower GI contrast study in meconium ileus?
Contrast via 8F catheter in rectum, low dose fluoroscopy, follow contrast progress, can roll from left to right if needed
## Footnote
This technique helps visualize the bowel.
181
What are the associations with meconium plug syndrome?
Maternal diabetes, magnesium (given in pre-eclampsia), Hirschsprung, cystic fibrosis
## Footnote
Recognizing these associations aids in diagnosis.
182
What are the subtypes of Hirschsprung disease?
Short segment (75%), long segment (15%), total colonic aganglionosis (8%), ultrashort segment
## Footnote
Each subtype has different implications for treatment.
183
What is the management for Hirschsprung disease?
Full thickness rectal biopsy 2cm above dentate line
## Footnote
This biopsy confirms the diagnosis.
184
What is the Rule of 2s for Meckel diverticulitis?
2% of population, 2 feet from ileocaecal valve, 2 inches in length, symptomatic by 2 years, 2% symptomatic, 2 types of ectopic tissue
## Footnote
This mnemonic is useful for remembering key facts about Meckel diverticulum.
185
What is the embryology of Meckel diverticulitis?
Failed involution of the vitelline duct, true diverticulum on antimesenteric side of bowel
## Footnote
Understanding its embryological origin helps in diagnosis.
186
What nuclear medicine study is used for Meckel diverticulitis?
Tc99m pertechnetate if ectopic gastric mucosa
## Footnote
This study helps in identifying ectopic tissues.
