CONGENITAL ANOMALIES Flashcards
Bochdalek hernia
Posterolateral diaphragmatic defect
Morgagni hernia
Retrosternal diaphragmatic defect
Eventration
Abnormal elevation a part/entire leaf of the intact diaphragm due to:
Paralysis
Congenital muscular hypoplasia
Atrophy of muscle fibre
Congenital diaphragmatic hernia potential complications
Pulmonary hypoplasia
Pulmonary hypertension
Persistent pulmonary hypertension and persistent feral circulation
Diagnosis of congenital diaphragmatic hernia
PRENATAL- U/S or MRI with fetal surgery
POSTNATAL- Respiratory distress and cyanosis may be suggestive but confirmed on X-ray
Management of congenital diaphragmatic hernia
Stabilisation using ventilation to limited barotrauma (HFOV) and ECMO
Surgical intervention when child is stable
Investigations in hypertrophic pyloric stenosis and diagnostic criteria
U/S
Diagnostic criteria:
Muscle thickness =/> 4mm and a length =/> 16mm
Management of hypertrophic pyloric stenosis
Correct metabolic hypochloraemic hyponatraemic metabolic alkalosis
Surgical Ramstedts pyloromyotomy
Oesophageal atresia and T-O fistula diagnosis
Babygram after failure to pass stiff NG tube and contrast study contraindicated
Oesophageal atresia and T-O fistula management
Reploge tube to prevent further aspiration
Exclude other anomalies
TPN if surgery is delayed
Surgery: Thoracotomy, fistula ligation and end to end anastomosis
Oesophageal atresia and T-O fistula presentation
Maternal polyhydramnios and absence of stomach gas on prenatal U/S
Copious, fine white frothy bubbles of mucus in the mouth and nose that recur despite suctioning
Rattling respiration and episodes of coughing and choking in association with cyanosis
Symptoms worsen during feeding
Abdominal distension 2° to collection of air in the stomach
Duodenal atresia is common in
Trisomy 21
Duodenal atresia diagnosis/ presentation
Polyhydramnios
Bile stained vomitus
Scaphoid abdomen
Types of duodenal atresia
Duodenal stenosis
Fibrous cord atresia
Complete atresia with separation
Duodenal atresia management
Resuscitation and stabilisation
Surgical intervention with duodeno-duodenoplasty
Intestinal malrotation diagnostic investigation
Water soluble contrast study
Intestinal malrotation management
Resuscitation and surgical intervention
Perform Ladd’s procedure with bowel placed in a position of non rotation
Jejunoileal atresia definition
It’s due to intrauterine vascular accident as described by Banard and Louw
Jejunoileal atresia presentation
Polyhydramnios
Bilious vomiting
Abdominal distension
Assoc. anomalies
Jejunoileal atresia diagnosis
Triple bubble appearance on X-ray
Jejunoileal atresia management
Surgical repair with tapering or resection of dilated bowel
Anorectal malformation is due to…
Failure of descent of urorectal septum
Anorectal malformation in males
Perineal fistula
Rectourethral fistula (bulbar and prostatic)
Rectovesical fistula
Imperforate anus without fistula
Rectal atresia
Anorectal malformation in females
Perineal fistula
Vestibular fistula
Persistent cloaca
Imperforate anus without fistula
Rectal atresia
