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