CONGENITAL CYSTIC

ADENOMATOID
MALFORMATION
 HISTORY
• This 3 days old term / AGA / male baby
delivered via labour naturalis on 21.11.09
referred to MMHRC as a case of congenital
diaphragmatic hernia.
• H/O baby cried soon after birth.
• H/O resp. distress since birth.
• No H/O cyanosis/ convulsions.
• CT chest done outside – CONGENITAL
DIAPHRAGMATIC HERNIA
 CONTD…..
• ANTENATAL HISTORY :
• No H/O drug intake/ fever with rash / PIH/
GDM.

• Antenatal USG done at 7 months of GA-

S/O congenital diaphragmatic hernia.
 EXAMINATION
• O/E baby cry ,activity- weak
• colour – pink.
• no congenital anamoly,
• no birth injury,
• respiratory distress +,
• CRT < 3 sec,
•
• RESPIRATORY SYSTEM :
RR – 62 / min
• SCR +, ICR +
• Air entry decreased on left side,
• Movement decreased on left side
• No added sounds
• CARIOVASCULAR SYSTEM :
• HR – 148 / min
• apex shifted to right sie
• S1, S2 +, no murmur
• ABDOMEN :
• Protuberant, not scaphoid
• no organomegaly
• CNS :
AF open – N
• NNR +
 INVESTIGATIONS
• Hb – 18.1 g %
• TC – 8300 cells/cu.mm
• Platelets – 1.6 lakhs
• Sugar – 84 mg %
• Urea – 53 mg %
• Creatinine – 0.8 mg %
• PCV – 48
• BT – 3 min
• CT – 8 min
 CONTD……
• Surgical profile - Normal
• Chest X ray – Opacity over left side with shift of
mediastinum to right side
• 2D ECHO: Congenital heart disease, Small OS
ASD - Left to right shunt, Trivial TR, No
pulmonary hypertension
• CT Chest: Suggestive of Cystic adenomatoid
malformation
• USG Abdomen and Pelvis: Normal study
(Diaphragm intact)
 Management
• Nasal CPAP ventilation
• IV fluid
• IV antibiotics
• Inj. Vit. K
• Inj. Calcium gluconate
 Contd……
• Surgeon’s opinion was obtained
– No surgical intervention was required as the
findings were in favor of CAM. Delayed CT
abdomen and chest for contrast was advised
• CT surgeon opinion was obtained
– Diagnosed as CAM
– Surgery was suggested and need for post
operative ventilation explained
 Contd……
• After getting consent and DIL baby was
taken up for surgery
• Operative diagnosis – CAM of left lingula
and lower lobe
• Left lung lingulectomy and superior basal
segmentectomy wqs done
 Post operative
• Baby was received in NICU with tube and
bag ventilation (accompanied by
anaesthetist) with invasive BP monitoring
and chest tube in situ
• Baby was cold (temp 21.9 C)
• CRT > 3 Sec
• BP 68/36 mm of Hg
• ABG suggestive of metabolic acidosis
• Baby put under warmer connected to
mechanical ventilator support
• Bicarb correction was given
• Poor perfusion - Dopamine started @
12mic/kg/min
• Dehydration corrected frequently with fluid
boluses
• Fentanyl infiusion started for pain control
• BP started falling so dopa was increased
to 15 mics/kg/min
• Inspite of this BP kept falling so NA
infusion started @ 0.6mics/kg/min
• SpO2 started falling (36%)
• ABG showed severe respiratory acidosis
• Ventilator setting changed according to
ABG
• BP kept falling
• Peripheral IV line could not be obtained
hence anaesthetist was called to get a
central line which went into VAIN
• Baby went into cardiac arrest
• Was resuscitated as per latest guidelines
but could not be revived
• Baby declared dead on 27th Nov at 1:45
pm