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…..
1 day old neonate Antenatal History of anterior neck swelling Confirmed postnatally
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
Clinical assessments normal Fetus has large neck mass on USG in 37th week Mild Polyhydramnios Antenatal diagnosis was cystic hygroma
11.01.2011Fetus with vertical lieCephalic presentationNo abnormality detected
8.03.2011Well defined hypo echoic massDimensions 5.5 X 6 cmlocated in cervical regionLateral to neck vessels
Guarded prognosis was explained Continuation of pregnancy was advised Mode of delivery was to be decided upon obstetric indications Antenatal procedures not feasible
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
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 clothings Oro-nasal suctioning were performed Distress was alleviated in right lateral position
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
› Euthermic› No other dysmorphic features› CRT < 2 sec› Heart rate 122/min› Respiratory Rate 40/min› BP 58/32 (40) mm Hg› Anthropometry
› 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
› Solitary neck mass› Large –10x8x6 cms› Midline, encroaching towards right› Non pulsatile› Skin - normal
Palpation confirmed the findings Temp – normal Tenderness – absent firm to hard mass Not compressible Well encapsulated
Mobile in all the directions Not adherent to underlying structures Not adherent to skin Trans illumination- absent Not pulsatile No thrills or hum
Thermo neutral environment with servo control Airways management and positioning Fluid and electrolyte Nutrition Respiratory and Hemodynamic monitoring
Localisation of mass Characterisation of nature of lesion Airway column assesment Relationship with major neck vessels
3 mm section thickness plain and post contrast Base of skull to diaphragm
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
Serum alpha feto-protein on day 2- 83,000 ng/ml Normal range = 100000 to 125 ng/ml from neonatal to infancy
Specimen of 6.5x6x3.5 cm received. O/S- nodular with retracted capsule.
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.
• 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)
0 Mature solid teratomaI 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
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
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
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 mothers age No greater odds of occurance in males versus females No racial or ethnic preference.
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
Novel karyotypic changes on comparative genomic hybridization › 1p21.1 amplification › 9p22 deletion › 17q21.33 1-copy gain
Rare › Imperforate anus › Chondrodystrophia fetalis, › Hypoplastic left ventricle with pulmonary hypoplasia, › Cystic fibrosis, › Absence of corpus callosum, › Arachanoid cyst
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
Ultrasound – best modality Asymmetric, well-defined masses Large and bulky. Calcifications Polyhydramnios in 20 to 40 percent cases Other fetal abnormalities +
Shows mediastinal involvement position of the airway. Partial / total Compression
Ex utero intrapartum treatment (EXIT) procedure / OOPS procedure Specifically designed to preserve uteroplacental gas exchange to provide time to secure the airway
provides time for: › Neck dissection › Clip removal › Bronchoscopy › Endotracheal intubation › Surfactant administration › Placement of umbilical arterial and venous catheters
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
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
Primary surgical excision If malignancy proved then › Chemotherapy › Radiotherapy › Combination therapy
Risk for serious thyroid conditions › Hypoparathyroidisim › Hypothyroidism Developmental delay and mental retardation Malignant transformation
Recurrence Metastasize to regional lymph nodes Occurring among siblings (only one case reported)
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.
Airway obstruction at birth Degree of maturity of tissues Completeness of resection Associated anomalies Mortality is high in untreated infants & low if treated surgically