Congenital chylothorax: current perspectives and trends

Figures & data

Table 1 Causes of CC

Idiopathic

Congenital malformation of lymphatics

Lymphangiomatosis

Lymphangiectasia

Atresia of thoracic duct

Cystic hygroma

Associated with syndromes

Down syndrome

Noonan syndrome

Turner syndrome

Gorham–Stout syndrome

Yellow nail syndrome

Abbreviation: CC, congenital chylothorax.

Figure 1 Formation, course, and termination of thoracic duct. Reprinted with permission from Earth’s Lab., Dr Joseph H Volker.¹⁰⁴

Abbreviation: SVC, superior venacava.

Figure 2 Congenital chylothorax: clinical effects.

Figure 3 Bilateral CC, before (A) and after (B) intercostal drain insertion.

Abbreviation: CC, congenital chylothorax.

Table 2 Medications used in the management of CC

Medication	Mechanism of action	Case reports	No. of cases	Outcome	Side effects
Octreotide	Mild vasoconstriction of splanchnic vessels Reduction in intestinal secretions as well as intestinal absorption	Cochrane review Das and Shah^Citation61 19 case reports of 20 neonates	Totally 20 neonates	Successful resolution (n=14) Failure (n=4) Equivocal result (n=1)	None reported (n=12) NEC (n=1) Recurrence (n=1) Mild abdomen distension (n=2) Transient hypothyroidism (n=1)
Sildenafil	PGE5 inhibitor-> increase in cGMP-> facilitate lymphatic vessel growth	Malleske and Yoder^Citation65	One case	Effective resolution	None reported
Povidone-iodine	Enhanced sclerosis Anti-exudative	Brissaud et al^Citation82	Four cases	Effective in three cases with chest tube removal 6–16 days after povidone-iodine (Betadine)	Increased contralateral pleural effusion (n=1) Generalized edema (n=1) None reported (n=1)
		Scottoni et al^Citation84	Five cases	Effective in four cases Median time for resolution – 4 days	None reported
OK-432	Cellular and cytokine mediated	Matsukuma et al^Citation90 Kim et al^Citation89	Two cases Two cases	Median time for resolution – 4 days Median time for resolution – 2–3 days	Tachypnea and fever None reported
Oxytetracycline	Initiation of inflammatory cascade by mesothelial cells	Utture et al^Citation92	One case	Effective resolution in 2 days	None reported

Abbreviations: CC, congenital chylothorax; cGMP, cyclic guanosine monophosphate; NEC, necrotizing enterocolitis.

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