special case of superior vencava syndrome

1. JOINT ROUND
UNIT III, Dept OF PAEDIATRICS
J.J. HOSPITALS
-BY Dr. NISHANTTAWARI (JR I)

2.  SAMEER, 9YRS OLD MUSLIM MALE CHILD 2ND BY ORDER OF BIRTH,
BORN OUT OF NON-CONSANGUINEOUS MARRIAGE HAILING FROM
RAIGAD, MAHARASHTRA, BROUGHT BY PARENTSWITH COMPLAINTS
OF-
BREATHING DIFFICULTY SINCE 5 DAYS
SWELLINGOVERTHE FACE AND CHESTWALL SINCE 5 DAYS

3.  HOPI-
PATIENTWAS DOINGWELL 5 DAYS BACKWHEN HE STARTED
FEELING DIFFICULTY IN BREATHINGWHICHWAS INSIDIOUS IN
ONSETAND GRADUALLY BECOME PROGRESSIVEAND NOW MORE
IN SUPINE POSITION.THERE IS NO H/O SORETHROAT, NO H/O
HEMOPTYSISOR COUGHING IN SUPINE POSTION, NO H/O FEVER.
PARENTSALSO NOTICED SWELLINGOVERTHE FACE AND ALSO
OVERTHE BOTH SIDEOF UPPERTHIRD OF STERNUM SINCE LAST 5
DAYSWHICH IS PROGRESSING SLOWLY. NO H/O CHEST PAIN, NO
SYNCOPALATTACK, NO H/O BLUISH DISCOLORATIONOF PALMS/
SOLES. BUT PATIENTWAS COMPLAININGOF PALPITATIONSWITH
SWEATING SOMETIMES.

4.  PAST HISTORY-
PATIENTWAS HAVING SIMILARCOMPLAINTSA MONTH BACKWHICH
WASTEMPORARILY RELIEVEDON MEDICATIONS PRESCRIBED BY SOME
LOCAL PRACTIONER.
BUT PATIENT AGAIN GETSTHE SIMILAR EPISODE 15 DAYS BACK. SOTHAT
2D ECHO DONEAT SOME OTHER CENTERWHICH IS S/OVALVULARAND
INFUNDIBULAR STENOSISOF PULMONARYVALVE.AND HENCE
REFERREDTO HIGHER CENTER FOR FURTHER MANAGEMENT.
 BIRTH HISTORY-
BORNTO R & I MOTHER BY LSCS I/V/O OLIGOHYDRAMNIOS.
NO NICU/ PICUADMISSIONS.

5.  IMMUNISATION HISTORY-
IMMUNISEDAS PER SCHEDULETILL DATE
 FAMILY HISTORY-
NOT SIGNIFICANT
 DIETARY HISTORY-
MIXED DIET.
ADEQUATE DIETARY INTAKE.

6.  DEVELOPMENTAL HISTORY-
ACHIEVEDALL MILESTONESAT APPROPRIATE AGE.
 SOCIOECONOMIC HISTORY-
LOWER MIDDLECLASSOF MKS SCALE.
 ANTHROPOMETRY-
OBSERVED EXPECTED Z SCORE
Ht 132 cm 135cm -1TO 0
Wt 30.44 kg 32 kg -1TO 0

7.  ON GENERAL EXAMINATION-
PATIENTWAS CONSCIOUSAND ORIENTEDAND EXAMINED IN SITTING
POSITION.
AFEBRILE
GC- FAIR
HR- 94/min, PERIPHERAL PULSES+
RR- 30/min
BP- 108/70 mmHg IN RT ARM.
SpO2- 98%
MILD PALLOR, CLUBBING + (GR II), THROAT- NOT CONGESTED
NO ICTERUS/CYANOSIS/OEDEMA/ LYMPHADENOPATHY.
NO NEUROCUTANEOUS MARKERSWERE SEEN.
 LOCALLYTHERE IS SWELLING IN MANDIBULARAND
SUBMANDIBULAR REGION.AND ALSO OVERTHE BOTH SIDE OF
UPPERTHIRD OF STERNUM.

8.  HEADTOTOE EXAMINATIONWAS NORMAL.
 SYSTEMIC EXAMINATION-
 CARDIOVASCULAR SYSTEM-
INSPECTION-TRACHEA CENTRAL
SWELLINGOVERTHE PRECORDIUM.
APICAL IMPULSEAT 4RTH ICS JUST LATERAL TO NIPPLE
PALPATION- INSPECTORY FINDINGSCONFIRMED.
NOTHRILL, NO HEAVE.
AUSCULTATION- S1 S2 NORMALLY HEARED.
SOFT SYSTOLIC MURMUR+ PROMINENT IN
PULMONARYAREA ANDAPEX.
 REST SYSTEMIC EXAMINATIONWAS NAD.
PROVISIONAL DIAGNOSIS: ? LUDWIG’SANGINAWITH PULMONARY

9. CLINICAL PICTURE OF PATIENT SHOWING SUBMANDIBULAR SWELLING

10. INITIAL MANAGEMENT
 ROUTINE BLOOD INVESTIGATIONWEREWITH IN NORMAL LIMITS.
 INITIALLY PATIENT HAD BEEN STARTED ON INJ. CRYSTALLINE
PENICILLINWITHO2 BY MASKWITH PROPPED UP POSITIONAND
NEBULISATION I/V/O SUBMANDIBULAR SWELLINGAND DIFFICULTY
IN BREATHING.
 AND ON ANTIFAILURETREATMENT WITH PROPRANOLOL,AS
PREVIOUS 2D ECHO REPORT DATED 27 NOV 2015WAS S/O
PULMONARYVALVULAR & INFUNDIBULAR STENOSISWITH PEAK
GRADIENT OF 80mmHg.WITHTRIVIALTR.

11.  ON CHEST X-RAY PA-VIEW-
BOX SHAPED HEARTWAS SEEN.

12.  SO CHEST X-RAY LATERALVIEWWAS DONE.
ANDTHE FINDINGWAS ANTERIOR MEDIASTENAL HAZINESSWITH
HEART PUSHED BEHIND.

13.  SO, REPEAT 2D ECHO WAS DONE BY EXPERT HANDS DATED 14/12/15
S/O-
SUPERIORVENA CAVAL OBSTRUCTIONWITH RVOT OBSTRUCTION
(PROBABLY EXTRA-CARDIAC).
ANDADVICEDAN URGENTCARDIAC CT AND CT CHEST.

14.  SO, URGENT CARDIAC CT WAS DONEAT J.J. HOSPITALS ON
15/12/15 S/OWELL DEFINED HOMOGENEOUSLY ENHANCING ISO-
TO HYPO-DENSE LESION INANTERIOR AND SUPERIOR
MEDIASTINUMWHICH IS COMPLETELY ENCASINGTHE SVC AND
INNOMINATEVEIN. IT IS SEEN EXTENDING INTOTHE SVC
CAUSING INTRALUMINALCONTRAST FILLING DEFECT
SUGGESTING INVASION. FINDINGSARE S/O MALIGNANT
NEOPLASM. ?LYMPHOMA, ?GERM CELLTUMOUR. HPE
CORRELATION SUGGESTED.

15. CARDIAC CT S/O MASS IN ANTERIOR MEDIASTENUM PUSHING THE HEART
BACKWARDS.

17. MEDIASTENAL MASSES
 MEDIASTINUM IS DIVIDED INTO 3 SECTIONS.

18.  THERE ARE SEVERALTYPESOF MEDIASTINAL MASSES,WITHTHEIR
CAUSES LINKEDTOWHERETHEY FORM INTHE MEDIASTINUM:
ANTERIOR MEDIASTINUM:
 GERMCELL- MAJORITY OFWHICH ARE BENIGN (70%) & ARE FOUND
IN BOTH MALESAND FEMALES.
 LYMPHOMA- MALIGNANTTUMOURSTHAT INCLUDES BOTH
HODGKIN’STUMOURAND NON-HODGKIN’S LYMPHOMA.
 THYMOMAANDTHYMIC CYST-THE MOST COMMONCAUSE OF A
THYMIC MASS.THE MAJORITY OFTHYMOMAARE BENIGN LESIONS
THAT ARE CONTAINEDWITHINA FIBROUSCAPSULE. HOWEVER, 30%
OFTHESES CAN INVADE CAPSULE.
 THYROID MASS- USUALLY A BENIGNGROWTH SUCH AS A GOITER.

19. MIDDLE MEDIASTINUM:
 BRONCHOGENICCYST-A BENIGNGROWTHWITH RESPIRATORY
ORIGIN.
 LYMPHADENOPATHY
 PERICARDIAL CYST- A BENIGNGROWTHTHAT RESULTS FROM AN
“OUT POUCHING” OFTHE PERICARDIUM (HEART’S LUNG).
 TRACHEALTUMOURS-TRACHEAL NEOPLASMSAND NON-
EUPLASTIC MASSES SUCHASTRACHEOBRONCHOPATHIA,
OSTEOCHONDROPLASTICA (BENIGNTUMOURS)
 VASCULARABNORMALITIES INCLUDINGAORTICANEURYSMSAND
AORTIC DISSECTION.

20. POSTERIOR MEDASTINUM:
 EXTRAMEDULLARY HAEMATOPOIESIS- EXPANSIONOF BONE
MARROW IN SEVEREANAEMIA.A RARE CAUSE.
 LYMPHADENOPATHY.
 NEUROENTERICCYST MEDIASTINAL- A RARE GROWTHTHAT
INVOLVES BOTH NEURALAND GASTROINTESTINAL ELEMENTS.
 NEUROGENIC NEOPLASM MEDIASTINAL- MOSTCOMMONCAUSE OF
POSTERIOIR MEDIASTINALTUMOUR. 70%ARE BENIGN.

21. SYMPTOMS:
 40 % OFTHE PEOPLESWITH MEDIASTINAL MASSES HAVE NO
SYMPTOMS. MOSTOFTHEM OFTEN DISCOVEREDON CXR
PERFORMED FOR OTHER PURPOSE.WHEN SYMPTOMSARE PRESENT
THEY ARE OFTEN A RESULT OF COMPRESSIONOF SURROUNDING
STRUCTURE, SUCH AS SPINALCORD, HEART OR PERICARDIUM.AND
MAY INCLUDE-
COUGH, SHORTNESSOF BREATH , CHEST PAIN, FEVER, CHILLS, NIGHT
SWEATS,WEIGHT LOSS, HOARSENESS, LNpathy,WHEEZING STRIDOR.

22. DIAGNOSIS
 CHEST X RAY.
 CT OFTHE CHEST OR CT-GUIDED NEEDLE BIOPSY.
 MRI OF CHEST.
 MEDIASTINOSCOPYWITH BIOPSY.
TREATMENT
 DEPENDSOFTHETYPE OF MASSAND ITS LOCATION.

23. REFERENCES
 Weissleder R, wittenberg J, Harisunghani MG. Primer of diagnostic
imaging. Mosby Inc. (2003) ISBN:0323023282
 Brown LR, Aughenbaugh GL. Masses of Anterior mediastinum: CT
and MR imaging.AJR am J Roentgenol. 1991;157 (6): 1171-80
 ShadA Magrath I. Malignant Non- Hodgkin’s Lymphoma In Children.
Principles and Practice of Pediatric Oncology, 3rd Edition, Lippincott-
Raven Publisher’s , pp545-587.
 Nelson’sTextbook Of Pediatrics,Volume-2, 20th Edition from R.
Kleigman, B. Stanton, St Geme, Schor: p2424f