Pediatrics 2 Flashcards
What will be formed by failure of obliteration of 2nd brancheal cleft?
BRANCHIAL CYST
- cystic mass in lateral aspect of neck
- may open with fistula
- remove by surgical excision
cystic mass at line of fusion is called?
SEQUESTRATION DERMOID CYST
- usually occurs in external angular in face
- if in neck -> median
- treated -> simple excision of cyst
unilateral sternomastoid mass after birth trauma?
congenital torticollis
- diagnosed by neck US
- head tilt to same side
coarse lymphangioma presenting with large, translucent cystic mass at the side of the neck is called?
CYSTIC HYGROMA
- inject sclerosing agent
What are the types of congenital diaphragmatic hernias?
POSTERIOR HERNIA -> foramen of Bockdalek (commonest type)
ANTERIOR HERNIA -> Foramen of Morgagni (opening of IMA)
DIAPHRAGMATIC EVENTRATION -> weakness
CONGENITAL HIATUS HERNIA
What is the clinical presentation of a congenital diaphragmatic hernia?
ANTENATAL -> maternal polyhydraminos
AT BIRTH -> neonatal cyanosis & respiratory distress
- scaphoid abdomen
- diminished air entry with audible intestinal sounds on chest
- heart sounds heard better on right side
What are the complications of a congenital diaphragmatic hernia?
- ipsilateral pulmonary hypoplasia
- contralateral lung compression
- strangulation
What investigations should be done in order to diagnose a diaphragmatic hernia?
CHEST X-RAY
- bowel in chest
- shift of mediastinum to contralateral side
- dextrocardia
GASTROGRAFIN MEAL
ANTENATAL US
- polyhydramanos
BLOOD GASES
- hypoxia
- hypercapnia
- acidosis
What is the most important step in treatment of congenital diaphragmatic hernia?
PRE-OP PROPER PREPARATION
- NPO
- NGT & IV FLUIDS -> deflate stomach -> decrease lung compression & mediastinal shift
- endotracheal intubation -> if failed -> ECMO
- pulmonary vasodilators
What is the definitive treatment of a congenital diaphragmatic hernia?
OPERATIVE REPAIR AFTER PRE-OP PREP
- open -> subcostal incision OR thoracoscopic -> reduce abdominal contents & fix diaphragmatic defect
What is the cause of esophageal atresia?
failure of recanalization of the foregut
What are the types of esophageal atresia?
- blind upper pouch & fistula of lower pouch -> most common
- upper fistula & lower atresia
- atresia without fistula
- fistula without atresia
- atresia with upper & lower fistula
What are the complications of fistulas?
- lower fistula -> fatal acid pneumonia
- upper fistula -> aspiration of milk & saliva
cause of death -> pneumonia or VACTREL
What is the clinical picture of esophageal atresia?
antenatal -> maternal polyhydraminos
at birth -> CONTINUOUS POURING OF FROTHY SALIVA SINCE BIRTH
-> regurgitation, chocking, cyanosis & cough
- CATHETER TEST -> stops at 10cm from nostrils
- abdominal distention -> due to air entry
- dehydration
- pneumonia
What investigations should be preformed to diagnose esophageal atresia?
X-RAY -> fundic air bubbles increase
GASTROGRAFIN -> shows atresia
How is esophageal atresia treated?
SURGICAL EMERGENCY PRE-OP PREP - NPO & IV fluids - suction of saliva (sump tube) - put baby in prone position to avoid aspiration - warm baby
OPEN THORACOTOMY or THORACOSCOPE
- dissection over TOF & separate it with closure of tracheal defect
- mobilization of both esophageal ends
- if gap is long -> circular or spiral esophagomyotomy of upper pouch
- circular didnt work -> fixation of distal esophageal pouch to paravertebral fascia & marking it with clips -> 2nd set
- last option -> gastric pull up
What is the normal pyloric thickness?
4mm
When is congenital hypertrophic pyloric stenosis diagnosed?
if pyloric thickness is >8mm
What is the pathology in CHPS?
PYLORUS
- hypertrophy of circular muscle fibers
- end abruptly in duodenum
- fades gradually towards the stomach
STOMACH
- dilated with hypertrophy of its walls
- gastritis -> due to stasis
INTESTINE
- collapsed & empty
What is the clinical presentation of CHPS?
age of presentation -> peak at 1 month VOMITING - non-bile stained - projectile - occurs after feeding - does not respond to anti-emetics - followed by hunger pain CONSTIPATION FAILURE TO THRIVE
What are the signs of CHPS?
- dehydration
- recurrent chest infections
- upper abdominal distention
- olive-like palpable mass at right hypochondrium
What are the complications of CHPS?
- recurrent chest infections
- dehydration
- metabolic & electrolyte disturbances
hypokalemia
hyponatremia
hypocalcemia
metabolic alkalosis
paradoxical aciduria
What is the most diagnostic method of investigation is case of CHPS?
ABDOMINAL US -> >8mm pylorus
next is Gastrografin meal
- dilated stomach
- delayed emptying
- string sign
What is the main line of treatment in CHPS?
PRE-OP PREP
- NPO
- NGT & washing of stomach
- IV fluids
- correction of dehydration
- correct metabolic & electrolyte disturbance
- manage chest infection
RAMSTEDT’S PYLOROMYOTOMY
- better by laparoscopy
- incision involves serosa & musculosa till mucosa bulges
What are the types of biliary atresia?
- correctable -> portion of extrahepatic tree only is occluded
- noncorrectable -> intra & extrahepatic atresia
presents as NEONATAL OBSTRUCTIVE JAUNDICE
What are the investigations & treatment of biliary atresia?
investigations -> US, HIDA scan, ERCP
treatment
- correctable -> KASAI OPERATION (Roux en Y hepatico-jejunostomy)
- non-correctable -> liver transplant
What biliary tree anomaly is PRECANCEROUS?
CHOLEDOCAL CYST - congenital cystic dilatation of intra/extrahepatic biliary system has 5 types I -> Fusiform (extra) II -> saccular (extra) III -> abnormal fusiform (most distal part of CBD) IV -> multiple intra&extra hepatic V -> multiple intrahepatic
How does a a choledocal cyst present?
- childhood obstructive jaundice
- RT hypochondrial swelling
- +/- pain
predisposition to cholangiocarcinoma
How is a choledocal cyst treated?
- EXTRA-HEPATIC -> excision of the cyst with Roux-en-Y choledocho-jejunostomy
- INTRA-HEPATIC (Caroli’s disease) -> liver transplant
What are the indications of circumcision?
- religious indication
- phimosis (pinpoint opening of foreskin)
- paraphimosis (obstruction of urethra)
- recurrent balanitis (recurrent inflammation of smegma)
What are the contraindications of circumcision?
- bad general condition
- hemophilia
- hypospidius
When should circumcision be preformed?
- 1 week of life -> maternal clotting factors
- after 40 days -> neonate developed clotting factors
Should anesthesia be used in circumcision?
- < 1 year -> no or local anesthesia
- > 1 year -> general anesthesia
What are the complication of circumcision?
- bleeding
- under correction
- over correction
- injury of glans or urethra
