Congenital Cystic Adenomatoid Malformation

CASE PRESENTATION

DR MAHESH TALPALLIKAR (PEADIATRIC SURGRON )DR MANDAR HAVAL (DCH DNB FELLOW IN NEONATOLOGY)

HISTORY

Bhavan Reddy 8 month boy Hindu by religion born of non-consangineous marriage to primigravida mother hailing from Basvkalyan brought by parents with C/O

Fever , cold , cough on & off---1month

Breathlessness ------------------ 1day

ONSET, DURATION, PROGRESS

Patient was apparently alright last month when he had insidious onset fever ,cough ,cold . Fever was mild to moderate grade more at night associated with cough .Cough not associated with expectoration.

ODP cont…

He was treated on OPD basis with oral antibiotics & symptomatic treatment. He improved initially but since 1 day his parents complained breathlessness which was sudden onset . Patient was referred to ASR Solapur .

No H/O repeated respiratory tract infection

No H/O weight loss , failure to thrive

No H/O high grade fever ,productive cough No H/O fever with rash

No H/O bluish discolouration

No H/O foreign body ingestion

PAST HISTORY No H/O exanthematous illness

(Measles),tuberculosis No H/O any other significant

illness in the past.

FAMILY HISTORY No H/O tuberculosis , significant

illness in family member.

BIRTH & PERINATAL HISTORY

Antenatally registered, 2 TT inj taken.

Antenatal USG normal. No H/O DM , HTN , thyroid

disorder in mother No H/O fever with rash ,or any

other illness No H/O any medication , radiation

BIRTH HISTORY Cont…

Full term born vaginally at hospital.

Baby cried immediately after birth.

No resuscitation required . No H/O fever ,convulsion ,icterus

,cyanosis postnatally. No NICU admission.

IMMUNIZATION

Completed according to national schedule till age.

SOCIO-ECONOMIC HISTORY Father – Private business Mother -Housewife

GENERAL EXAMINATION

Conscious , a febrile HR 156/min CRT >3sec RR 68/min - tachypnoea BP 60/46 mm of Hg in Rt arm

supine position No pallor ,cyanosis , clubbing ,

oedema , lymphadenopathy

HEAD TO TOE EXAM Head , face & Neck- No facial

dysmorphic features , fontanel closed.

Eyes , ear ,nose –normal. Mouth , throat – normal. Neck – No neck swelling, no neck

veins visible. Skin , hair , nail – Normal Bone , joints ,spine –normal.

ANTHROPOMETRY

Head circumferance- 44 cm (>50%ile)

Chest circumferance- 41 cm Length – 70 cm (>50%ile) Weight – 7.2 Kg (>15%ile) U/S Ratio – 1.6 : 1

SYSTEMIC EXAMINATION

CVS- S1 S2 heard normal , Apex beat shifted to left No murmur RS- Air entry ABSENT on Rt side. Decreased chest rise on Rt side. No crepts , Tachypnea+, retra Accessory muscles of respiration

active.

SYS EXAM Cont…

P/A- Soft distended ,liver 3cm

palpable. Spleen , kidney – Not palpable. CNS- Tone ,power , reflex – Normal No other signs of neural deficit

INVESTIGATIONS

CBC- TLC 25600 cells/cmm (Neu 85%, Lym 13%, Band forms 2%), Hb 12.3 g/dl ,

Platelet 7,21,000 /cmm X ray chest – Right sided pneumo-

thorax with collapse of right lung , mild displaced mediastinum.

USG Chest + Abdomen- Right side pneumo-thorax with underlying collapsed right lung.

DIAGNOSIS ???

RIGHT PNEMOTHORAX WITH COLLAPSED RIGHT LUNG …!!!!

Xray post ICD (3 Day)

So ICD put & Xray done which showed mild to moderate pleural effusion Rt side with irregular consolidation RT MZ , LZ

Why pnemo-thorax ???

Table 405-1   -- CAUSES OF PNEUMOTHORAX IN CHILDREN

HRCT THORAX done

HRCT SHOWING- Multicystic lesions with thick walls seen involving anterior basal , posterior basal segment seen involving Rt lower lobe S/O Congenital Cystic Adenomatoid Malformation Type 1.

NOW DIAGNOSIS IS…

Congenital Cystic Adenomatoid Malformation Type 1- presenting as Pneumo-thorax

Management

O2 Inhalation. ICD was put. Right lung lobectomy after settling

of pnemothrax done. Inj Antibiotics Symptomatic treatment.

Patient improved & discharged later.

POST-OPERATIVE X-RAY

X-RAY ON DAY BEFORE DISCHAGE

Congenital Cystic Adenomatoid Malformation

Cystic adenomatoid malformation (CCAM) consists of hamartomatous or dysplastic lung tissue mixed with more normal lung, generally confined to one lobe.

This congenital pulmonary disorder occurs in ∼1-4/100,000 births.

CCAM cont…

Etiology- The lesion probably results from an

embryologic insult before the 35th day of gestation, with maldevelopment of terminal bronchiolar structures.

Histologic examination reveals little normal lung and many glandular elements. Cysts are very common; cartilage is rare.(cartilage if insult occurs later -10th -24th wk )

CCAM Cont…

Histological types- Type 1 (50%) is macrocystic and

consists of a single or several large (>2 cm in diameter) cysts lined with ciliated pseudostratified epithelium. The wall of the cyst contains smooth muscle cells and elastic tissue. One third of cases have mucus-secreting cells. Cartilage is rarely seen in the wall of the cyst. This type has a good prognosis for survival.

CCAM Cont…

Type 2 (40%) is microcystic and consists of multiple small cysts with histology similar to that of the type 1 lesion. Type 2 is associated with other congenital anomalies and carries a poor prognosis.

CCAM Cont…

Type 3 (<10%), the lesion is solid with bronchiole-like structures lined with cuboidal ciliated epithelium and separated by areas of nonciliated cuboidal epithelium. This lesion carries the poorest prognosis and can be fatal.

DIAGNOSIS

Antenatal USG- Prenatal ultrasonographic findings

are classified as macrocystic (single or multiple cysts >5 mm) or microcystic (echogenic cysts <5 mm).

CT Thorax- Allows accurate diagnosis and sizing

of the lesion.

Clinical manifestation

Patients can present in the newborn period or early infancy with respiratory distress, recurrent respiratory infection, and pneumothorax.

Patients with smaller lesions are usually asymptomatic until mid-childhood, when episodes of recurrent or persistent pulmonary infection or chest pain occur.

Treatment Surgery- Sarcomatous and carcinomatous

differentiation has been described in patients with CCAM, so surgical resection by 1 yr of age is recommended to limit malignant potential. The mortality rate is <10%.

Another indication for surgery is to rule out pleuropulmonary blastoma, a malignancy that can appear radiolographically similar to type I CCAM.

THANK YOU !!!!!!