2. 1 day old neonate
Antenatal History of anterior neck swelling
Confirmed postnatally
3. Maternal age 26 years
Second gravida with one alive child
Previous LSCS
No other significant Medical/Surgical issues
Regular follow up while in Antenatal period
4. Clinical assessments normal
Fetus has large neck mass on USG in 37th week
Mild Polyhydramnios
Antenatal diagnosis was cystic hygroma
6. 8.03.2011
Well defined hypo echoic mass
Dimensions 5.5 X 6 cm
located in cervical region
Lateral to neck vessels
7. Guarded prognosis was explained
Continuation of pregnancy was advised
Mode of delivery was to be decided upon
obstetric indications
Antenatal procedures not feasible
8. FTLSCS at JLNHRC on 19/3/2011 12:50 pm
Baby had massive neck mass
Cried immediately after birth
Had mild respiratory distress in supine position
APGAR score at 1 min – 7
5 min - 8
9. Birth of high risk newborn anticipated
Instruments for securing airways were ready had the
baby deteriorated further
Kept in thermo neutral environment
Dried and wrapped in warm clothing's
Oro-nasal suctioning were performed
Distress was alleviated in right lateral position
10. Initial stabilization assured in labour room
Necessary equipments kept ready in NICU
Transported with warm clothes
Airway positioning while transporting assured
Case was informed to pediatric surgeon
11. › Euthermic
› No other dysmorphic features
› CRT < 2 sec
› Heart rate 122/min
› Respiratory Rate 40/min
› BP 58/32 (40) mm Hg
› Anthropometry
12. › Respiratory system - B/L breath sounds distinct
equally heard
conducted sounds +
Inspiratory stridor
› CVS – S1S2 +, no murmurs
› Per Abdomen – soft, non distended, non tender
› CNS – NNR present
› Ext genital – female, normal
13. › Solitary neck mass
› Large –10x8x6 cms
› Midline, encroaching
towards right
› Non pulsatile
› Skin - normal
14. Palpation confirmed
the findings
Temp – normal
Tenderness – absent
firm to hard mass
Not compressible
Well encapsulated
15. Mobile in all the
directions
Not adherent to
underlying structures
Not adherent to skin
Trans illumination- absent
Not pulsatile
No thrills or hum
16. Thermo neutral environment with servo control
Airways management and positioning
Fluid and electrolyte
Nutrition
Respiratory and Hemodynamic monitoring
18. › Solid mass
› Well encapsulated
› Few areas of cystic degeneration
› Few stipulated calcifications
› No large calibre vessels inside the tumor
› Neck vessels pushed laterally
› Tracheal displacement +
19. ENT consultation
› airway status
› need of elective/emergent intubation
› Intra operative help
ENT Opinion
› No pressure effect on trachea
› No need of emergent intubation
› CT scan
› FNAC
20. FNAC avoided due to
› possible risk of hemorrhage within the mass
leading to airway compromise.
› Non representative areas aspirated
› Limited sensitivity of FNAC
22. Localisation of mass
Characterisation of nature of lesion
Airway column assesment
Relationship with major neck vessels
23.
24.
25. 3 mm section thickness plain and post
contrast
Base of skull to diaphragm
26.
27.
28.
29.
30.
31.
32. A large 6.5x6x5 cm sized heterogenous
Mildly enhancing mass lesion wnich is well
encapsulated containing scattered nodular
calcification seen involving neck anteriorly
and on rt side
Supreiorly upto submandibular space
Inferiorly supraclavicular region
Displacing airway column on left and
major vessels posteriorly possibility of
cervical teratoma.To be correlated with
clinical and histopathological findings
33. Serum alpha feto-protein on day 2- 83,000 ng/ml
Normal range = 100000 to 125 ng/ml from neonatal
to infancy
44. Specimen of 6.5x6x3.5
cm received.
O/S- nodular with
retracted capsule.
45. C/S- shows lobulated grey
white mass predominantly
solid with multiple small cysts .
Cysts are of varying size from
1mm to1cm diameter filled
with mucinous material.
Few cartilagenous area,
slimy area & bony spicules
were present in solid part of
the mass.
53. • Multiple sections studied from tumour shows mature as well as
immature elements derived from all 3 germ layers.
• Mature elements comprise of nests of squamous cells, glands,
mature cartilage, occasional bony tissue, neural tissue &
smooth muscle tissue.
• Immature elements include neuroepithelial elements,
occasional group of blastemal cells & immature cartilage in
myxoid stroma. Mitosis is in the range of 2/10HPF. Normal
thyroid tissue is not seen in the section studied.
• Impression:- ABOVE FEATURES FAVOUR IMMATURE CERVICAL
TERATOMA (Grade –II)
54. 0 Mature solid teratoma
I Abundance of mature tissues, intermixed with loose
mesenchymal tissue with occasional mitoses; immature
cartilage; tooth anlage
II Fewer mature tissues; rare foci of neuroepithelium
with common mitoses, not exceeding three 40X
fields in any one slide
IIIFew or no mature tissue ; numerous neuroepithelial
elements, merging with a cellular stroma occupying
≥four 40X fields
55. Greek word – monstrous tumour
Derived from all three embryonic germ layers-ectoderm,
endoderm and mesoderm
Can occur anywhere in the body
Most common location – sacral region
Rarer in adults since most are detected in childhood.
Neonatal period are uncommon and virtually always benign
56. Rare congenital tumours of neck
Challenging in the neonatal period
Present as massive neck swelling with airway
compression
High perinatal mortality and morbidity rates.
Predominantly of the mature variety
57.
58. Constitute 1.6 to 9.3% of pediatric teratomas, 1per
40,000 births
Global scenario - Over 150 cases reported so far
Indian scenario - 4 cases ,1stiiborn, 1 died soon after
birth, 2 surviving
No apparent relationship to the mother's age
No greater odds of occurance in males versus females
No racial or ethnic preference.
59. Exact cause still unknown
Inability of totipotent cells to differentiate into
a complete body or organ
Abnormal development of a conjoined twin
Arises from stem cells within the thyroid gland
60. Novel karyotypic changes on comparative
genomic hybridization
› 1p21.1 amplification
› 9p22 deletion
› 17q21.33 1-copy gain
61. Rare
› Imperforate anus
› Chondrodystrophia fetalis,
› Hypoplastic left ventricle with pulmonary
hypoplasia,
› Cystic fibrosis,
› Absence of corpus callosum,
› Arachanoid cyst
62. Based on birth status, age at diagnosis, and the
presence or absence of respiratory distress.
› Group I--stillborn and moribund live newborns
› Group II--newborn with respiratory distress
› Group III--newborn without respiratory distress
› Group IV--children age 1 month to 18 years
› Group V--adults
72. Ultrasound – best modality
Asymmetric, well-defined
masses
Large and bulky.
Calcifications
Polyhydramnios in 20 to 40
percent cases
Other fetal abnormalities +
73. Shows mediastinal
involvement
position of the
airway.
Partial / total
Compression
74. Ex utero intrapartum
treatment (EXIT)
procedure / OOPS
procedure
Specifically designed to
preserve uteroplacental
gas exchange to provide
time to secure the airway
75. provides time for:
› Neck dissection
› Clip removal
› Bronchoscopy
› Endotracheal intubation
› Surfactant administration
› Placement of umbilical arterial and venous
catheters
76. Frequent ANCs
Frequent ultrasound exams recommended
to monitor
› amniotic fluid volume,
› tumor size,
› growth and the general health of the fetus
Institutional delivery encouraged
Elective cesarean preferred
Team approach for ex utero management
77. Baseline hemogram and blood biochemistry
USG
CT scan/MRI
FNAC and Biopsy
Thyroid and parathyroid function test
Serum alpha fetoprotien and beta HCG
Transcription factors GATA-4 and GATA-6
Genetic studies
82. Recommendations
› AFP levels be obtained
at birth
at 1month,
three-month intervals in infancy and
yearly thereafter, upto 3 years of life
› MRI scanning twice a year for the first three
years of life.
83. Airway obstruction at birth
Degree of maturity of tissues
Completeness of resection
Associated anomalies
Mortality is high in untreated infants & low if
treated surgically
Notes de l'éditeur
Will be presenting a case report of b/o rekhajain ……….A day old neonate with antenatal history of anteriorly placed tumoral mass in neck… The finding later cofirmedpostnatlly .
Let us see the antenatal details…..Mother was 26 years old healthy women….. the Edd was 28.03.11. she was gravida two with one alive child. …..Except for LSCS which was done in last pregnancy no significant medical and surgical history including exposure to radiation and teratogenic drugs were present which could have imapct on this pregnancy. Mother had regular follow up in antenatal period including serial ultrasound examinations.
Clinicalassessments done throughout pregnancy till this time were normal ….A routine ultrasound done 3 weeks prior to delivery revealed a fetus with large neck mass along with mild polyhydramnios. The proposed antenatal diagnosis was cystic hygroma,
..lets see the antenatal ultrsounds….This slide shows an ultrasound aprox 10 weeksproir to delivery…….. Its shows a single alive fetus in uterine cavity in vertical lie with cephalic presentation……. Surprisingly no abnormal findings were reported. This usg was done by outside radiologist.
This is another usg which was done As a routine follow up in antenatal period and as a part of fetomaternal survillence….. the USG was reported at 37 th weeks of gestation with similar findings of presentation and lie. The significant finding reported in this usg was the presence of well defined hypoechoic mass with dimensions of 5.5 x 6 cms. Located in cervical region anteriorly .
Based on the findings of antenatal usg the parents were explained about the possible nature of disease and post delivery compliaction including need for immediate intubation and ventilation along with available modalities of treatment at our institute.…………In absence of any severe cardiac renal and cerebral malformation and overall fetal compromise continuation of pregnancy was advisable but with frequent antenatal assessments……mode of delivery again was to be decided by obstetrician….wide variety of antenatal procedure are advocated including most novel EXIT but were not feasible at our institute…….
The baby delivered electively by LSCS taking into consideration the previous LSCS and anticipation of difficulty to deliver vaginally due to large mass…………important finding are displayed on the screen…..head to toe examination revealed no fascial or craniospinal dimorphism except for this mass which was placed anteriorly in the neck….baby cried immediately had a good reflex activity, normal tone in both extremities with appropriately developed sucking and rooting reflex And needed no active resuscitation at birth ……another significant finding was the presence of respiratory distress in supine position…. This was obviously related to upper airway due to compression from the mass laying over the airways.
With the antecipation of high risk nb instruments for securing airway were kept ready. As many as 40 to 50 % of these nb with large cervical mass may need intubation as birth but fortunately in this case it was not required………initial stabilisation was in form of drying the baby, providing thermoneutral environment, and clearing airways by oronasal suctioning. The destress present in supine position was alleviated by placing the baby in right lateral position.
While the initial stabilisation in LR was ongoing the NICU staff and pediatric surgeon were informed……………necessary eqipments were kept ready in NICU in case baby develops airway compramise………………….the baby then transported with warm clothing while airways position assured during transportation…….
At about 15 min after birth the baby had a normal body temperature……..Finding of anthropometry including weight, length and head circumference were under normal norms for gestational age and days of life…..
CVS and GI system examination yield no abnormal findings……..cns examination revealed well developed sucking rooting reflexes with symmetrical moro..normal tone and good reflex activity…….gentalias were that of female and were normal..In respiratory system b/l equal air entry was present, few conducted sounds, Adequate chest expansion symmetrical on both sides…inspiratorystridor best heard in supine and least in right lateral position….
GOALS of management were to keep the airways patent and to provide essential nb care with emphasis on nutrition……body temperature was kept normal by providing thermo neutral environment with help of servo control open care system…….baby kept in right lateral postion to decrease the airway compression with slightly raised head end…….normal body fliud and electrolytes balance was maintained………..due importence was given to nutritional needs of baby….on day one itself the baby started with expressed breast milk in form of small tube feeds which were5 gradually increased to avoid intolerance…..at about 16-20 hrs after birth the baby was fed by mother….the feeding was supervised and the mother taught about correct positioning while feeding…respiratory and vascular dynamics was closely observed throughout the stay…..