Dr.Ghaniya K.C , Dr.C.Jayakumar , Dr.Praveena N.Bhaskaran , Dr.Stefy Suuny Jacob , Dr.Navya George , Dr.Shela Sany
4 year old girl, S/P anoplasty for imperforate anus on Day 4 of life, presented with history of multiple admissions for recurrent LRTI and FTT for evaluation. Child also had complaints of recurrent aspiration post feeds.
Let us go back
Day 2 -Ba has respiratory distress due to Meconium aspiration was on CPAP for 2 days.
Day 4 – Child had anoplasty done for imperforate anus
Day 45 – Child had complaints of cough while feeding, was admitted for bronchiolitis /pneumonia and was treated with IV Antibiotics.
At 2 months – Admitted again for breathlessness and managed as pneumonia and, treated with IV antibiotics
At 3 months – Admitted again admitted in ICU
This time work up for Immunodeficiency was done
Lymphocyte subset analysis and GE scintigraphy done were normal,
At 8 months -again Admitted for LRTI
Had similar episodes of LRTI at 1 y 7th month
They opted for Complementary and alternative system of treatment and getting treatment from them
Last episode of LRTI was at 4 years of age again requiring ICU admission
DIFFERENTIAL DIAGNOSIS
◦ TB
◦ Cystic Fibrosis
◦ Bronchiectasis
◦ Immunodeficiency
◦ TEF
CPAM
sequestration
PCD
Vitals were stable at presentation
PICCLE negative
Anthropometry
Weight -B/W 0 and -2SD
Height – B/W 0 and -2 SD
HC – between 0 and +1 SD
MAC – 114 cm
INVESTIGATIONS
TC – 17.38 K
N/L – 39/52
CRP – 5.84
Immunoglobulins (G,A,M,E ) – Within normal range
Pre OPserology test – Negative
Peripheral smear – Mircocytic hypochromic RBC’s with Leukocytosis
MDCT CHEST WITH HRCT- :Left upper lobe small parenchymal nodue with a few small ill defined scattered parenchymal infiltrates are non specific , likely indicating an infectious etiology.
No lung malformations .
No bronchiectasis.
GeneXpert MTB – MTB not detected.
Sweat chloride test – 35.00 mmol/L normal
Contrast swallow study was done in view of suspected TEF, and study showed contrast filled tract noted tracking anteriorly approximately at the level of T2-T3 with a thin line of contrast seen lining the wall of the trachea and bronchi bilaterally- Highly suspicious of TEF
Radiologically no vertebral anomalies in regard to VACTREL syndrome, no vertebral anomalies were noted.
Diagnostic bronchoscopy – A large fistula is seen at 6 o clock on rigid bronchoscopy 4 cms proximal to carina. 21 cms marking at tip of bronchoscope on 5 fr IFT when positioned tip at fistula.
Type H TEF
MANAGEMENT: TOF surgery under GA
TEF: Esophageal atresia (EA) is the most common congenital anomaly of the esophagus, with a prevelance of 1.7 per 10,000 births.
Of these , >90 % have an associated TEF.
In the most common form of EA , the upper esophagus ends in a blind pouch and the TEF is connected to the distal esophagus (Type C ).Exact cause is unknown , associated features include advanced maternal age , European ethnicity , obesity , low socioeconomic status & tobacco smoking.
50 % infants are non-syndromic without other anomalies , and the rest have associated anomalies , most often associated with the vertebral , anorectal , cardiac , tracheal , esophageal , renal , radial (VACTERL) syndrome.
Tracheoesophageal fistula are of five different types:
Type A: There’s no TEF(8%), only EA(pure esophageal atresia). Oesophagus divides into two parts, both portions ending in blind pouches .
Type B is rare form of TEF (2% )of all cases. Here lower portion of esophagus ends in a blind pouch, and the upper portion is connected to trachea a tracheoesophageal fistula.
Type C: Most common (85%)form .Here upper portion of esophagus ends in a blind pouch, and the lower portion is connected to trachea a tracheoesophageal fistula.
Type D: Rarest form of TEF(<1%), a tracheoesophageal fistula connects both the upper and lower portions of esophagus to trachea.
Type E(4%): Here sophagus connects to your stomach normally and is fully intact. However, a tracheoesophageal fistula connects esophagus and trachea.
TAKE HOME MESSAGE: Surgery is not the final step for a child with TEF. Child can present at later stage with strictures , leak and recurrent fistula.