TWO CHILDREN WITH TWO CHILDREN WITH CHYLOTHORAXCHYLOTHORAX

DEPT OF PEDIATRICS & PICUDEPT OF PEDIATRICS & PICUDR. PRASHANTDR. PRASHANT

DR. JANANI SANKARDR. JANANI SANKARDR. BALA RAMACHANDRANDR. BALA RAMACHANDRAN

DR. K. G. RAVIKUMARDR. K. G. RAVIKUMAR

KKCTHKKCTH

CASE -1CASE -1 11 year old boy11 year old boy H/O excision of small mass in axilla at H/O excision of small mass in axilla at

age of 3 years (? Cystic hygroma)age of 3 years (? Cystic hygroma) Admitted at age of 8 years with Admitted at age of 8 years with

bilateral chylothorax (TGL- high) and bilateral chylothorax (TGL- high) and dissolving ribs on left sidedissolving ribs on left side

A huge hemangioma in left A huge hemangioma in left hypochondriumhypochondrium

Bone scan showed increased uptake Bone scan showed increased uptake in ribs, pelvis and shoulder girdlein ribs, pelvis and shoulder girdle

BONE SCAN

FINAL DIAGNOSISFINAL DIAGNOSIS

GORHAM’S DISEASEGORHAM’S DISEASE

TREATMENT RECEIVEDTREATMENT RECEIVED

Frequent drainage of chyle from Frequent drainage of chyle from both side of chestboth side of chest

Octreotide Octreotide ZolendronateZolendronate Interferon-Interferon-αα s.c. 3 million unit s.c. 3 million unit

thrice a weekthrice a week VATS & Decortication on left sideVATS & Decortication on left side Albumin InfusionsAlbumin Infusions Diet (high protein and MCT rich)Diet (high protein and MCT rich)

FOLLOW UPFOLLOW UP

Growth FailureGrowth Failure Almost all ribs of left side had vanishedAlmost all ribs of left side had vanished Respiratory failureRespiratory failure Hypercapnea(pCO2 – max 110)Hypercapnea(pCO2 – max 110) New collection of chyle in left groin New collection of chyle in left groin

with corresponding lesion in the left with corresponding lesion in the left femurfemur

FURTHER TREATMENTFURTHER TREATMENT

Non Invasive Ventilation at homeNon Invasive Ventilation at home Radiation to Lt femur to target the Radiation to Lt femur to target the

new lesionnew lesion Radiation to mediastinum plannedRadiation to mediastinum planned Medical Pleurodesis with Tetracycline Medical Pleurodesis with Tetracycline

attemptedattempted Succumbed to cardiorespiratory Succumbed to cardiorespiratory

arrest and refractory shock within arrest and refractory shock within hours after pleurodesis hours after pleurodesis

Gorham’s diseaseGorham’s disease

Very rare disorderVery rare disorder

Also known as vanishing bone Also known as vanishing bone disease, massive osteolysis, Gorham disease, massive osteolysis, Gorham Stout syndrome.Stout syndrome.

Abnormal vasculogenesis in bones Abnormal vasculogenesis in bones and other tissues of the bodyand other tissues of the body

All options in literature were All options in literature were attempted on this boyattempted on this boy

Usually die by 2Usually die by 2ndnd decade decade

Our boy was diagnosed at 8 years Our boy was diagnosed at 8 years and died at 12 years of ageand died at 12 years of age

CASE -2CASE -2

3 years old girl3 years old girl

H/O lymphedema of both legs at birth.H/O lymphedema of both legs at birth.

Intermittent watery copious diarrhoea since Intermittent watery copious diarrhoea since birthbirth

Intestinal biopsy done at day 19 of life, Intestinal biopsy done at day 19 of life, showing showing

lymphangiectasia.lymphangiectasia.

Started on MCT rich formulaStarted on MCT rich formula

FINAL DIAGNOSISFINAL DIAGNOSIS

INTESTINAL INTESTINAL LYMPHANGIECTASIALYMPHANGIECTASIA

MANAGEMENTMANAGEMENT

Admitted many times for albumin Admitted many times for albumin transfusion. (every 2-3 weeks)transfusion. (every 2-3 weeks)

Pleural tap was also done 2 timesPleural tap was also done 2 times

FOLLOWUPFOLLOWUP

Growth failureGrowth failure

Lymphedema both lower limbsLymphedema both lower limbs

Episodes of laryngeal edema with Episodes of laryngeal edema with inspiratory stridorinspiratory stridor

RECENT ADMISSIONRECENT ADMISSION

Came with severe respiratory Came with severe respiratory distress with right chylothorax with distress with right chylothorax with fever.fever.

Intubated in view of impending Intubated in view of impending respiratory failure.respiratory failure.

Pig tail catheter was inserted in Pig tail catheter was inserted in thoracic cavitythoracic cavity

Improved with albumin infusion, Improved with albumin infusion, octreotide and removal of fluid.octreotide and removal of fluid.

Extubated and doing well Extubated and doing well

Treatment options in Treatment options in ChylothoraxChylothorax

MEDICALMEDICAL

OctreotideOctreotide Interferon-Interferon-αα PleurodesisPleurodesis

SURGICALSURGICAL

Thoracocentesis Thoracic duct

ligation Pleuro-peritoneal

shunt Decortication &

VATS

Thank YouThank You