Age: 38.0
Sex: F
Vaccine: VARCEL
Symptoms: Injection site hypersensitivity
Clinical narrative: Pt recieved vax on 12/10/99. She reported to physician on 12/14/99 that she was pregnant. After vax, the pt experienced a few small red bumps at the injection site. An aminocentesis was performed due to advanced maternal age and exposure to varicella virus vaccine live. The results indicated a normal female karyotype.  Received on 09/21/2000: "Information has been received through the pregnancy registry from a physician concerning a 38 year old caucasian female who on 12/10/1999 was vaccinated with varicella virus vaccine live.  She reported to him on 12/14/1999 she was pregnant.  Concomitant vaccines included influenza vaccine.  The patient was approximately six weeks pregnant at the time of vaccination.  Follow-up was received from the consumer which indicated LMP was approximately 11/05/1999.  Expected date of delivery is 08/12/2000.  This was confirmed by ultrasound on 12/27/1999.  After vaccination, the patient experienced a few small red bumps at the injection site which resolved.  On 11/17/1999 the patient had been vaccinated with influenza vaccine.  Starting on 11/22/1999, the patient took a seven day course of cephalexin monohydrate (keflex) for a breast infection.  She also took prenatal vitamin.  The patient's obsterical history was unremarkable with one prior pregnancy which resulted in a full-term healthy daughter.  Follow up was received from the patient.  An amniocentesis was performed on 02/24/2000 due to advanced maternal age and exposure to varicella virus vaccine live.  The results indicated a normal female karyotype.  She also had two ultrasounds which found no abnormalities.  Follow up was received from a physician.  The infant was born 08/02/2000.  The infant had cardiac birth defects, spina bifida, and oral facial defects.  The infant died.  Follow up was received from the mother.  The anomalies included a "fused gumline so that there was no opening for her to take in amniotic fluid.  They did a tracheotomy at birth so that she could  breathe".  Other anomalies included "a deformed heart with one chamber, underdeveloped lungs, and problems with her vertebrae."  An autopsy was being performed.  The mother was scheduled to meet with her infants physicians in four weeks to obtain the results.  It was noted "the anomalies were not being attributed to anything at this point and there may never be a definitive answer."  No further information is available.  Recieved 01/23/2001: "Follow-up was received from a physician.  The infant was born on 08/02/2000 at 38 weeks from LMP.  She was born via normal spontaneous vaginal delivery at 3:50am.  The delivery was precipitous.  Birth weight was 7lb, 11oz.  She was pale pink, very active, and fighting.  She experienced respiratory distress.  The temperature was 97 degrees F, heart rate 126, and blood pressure 75/46.  Meconium was noted at the delivery, advanced live support nurse was present and attempted to intubate the infact but the mouth was fused except for a very small spot on the left side and would not open.  The infant was nares patent.  She had bilateral pulmonary hypoplasia.  The infant had Shone's variant with mild hypoplasia of the left-sided heart structures (small mitral to aortic valves).  She also had spina bifida.  She had dysmorphic facies with stenotic/atretic choanae (left and right, respectively), micrognathia with right syngnathia, left cleft palate, right chosnal atresia, low set ears, right hypoplastic tongue, fusion of maxilla and mandible on the right side, and single palmar transverse creases bilaterally.  The infant was hand bagged.  ALS was placed over the nose.  An anesthesiologist was able to pry a small part of the left jaw open to put an oral airway in the place and then ventilate via a bag and mask.  The infant was transferred to the NICU.  A O Shiley trache was in place.  She had a right subclavian artery catheter, a right subclavian artery catheter, and an umbilical artery catheter.  The patient was on a ventilator with lung hypoplasia and resolved right pneumothorax with two chest tubes in place and small left pneumothorax still present.  The heart beat was regular, and perfusion was brisk.  She had normal female genitalia.  Abdomen was soft.  there was a three vessel cord.  The extremities appeared somewhat shorter.  Multiple spiral anomalies on chest x-ray were noted with fused ribs and abnormal vertebral bodies.  Apgars were 1 at one minute and 5 at five minutes, and 7 at ten minutes.  At 20-30 minutes of age, an umbilical artery catheter and an umbilical venous catheter were inserted.  UVC was in the liver and removed.  The first arterial blood gas being hand bagged with the oral airway in place showed pff of 6.8, carbon dioxide pressure 148, oxygen pressure 19, and base deficit -24.  35cc normal saline, 35cc albumin and 10 mEq of sodium bicarbonate were given via the UAC.  An umbilical artery catheter and an umbilical venous catheter were inserted but the latter was removed after it was noted to be positioned in the liver.  A chest x-ray was obtained wich showed right pneumothorax, large hypoplastic lungs, and multiple spinal anomalies with fused ribs and abnormal vertebral bodies.  A physician inserted an 8 French Argyle chest tube without difficulty in the right side and attempted to performa  trache with a 14 gauge angio cath without success.  A chest x-ray also showed multiple vertebral anomalies.  An attempt was made to intubate the infant through a small opening in the jaw without success.  During this time, the infant's hear rate was noted at 0530 and was noted to be down in the 40s.  The patient received epinephrine 0.3cc and 0.7cc with good recovery to greater than 110.  Two physicians performed a trache at 05?42am.  Saturations increased to 91%.  The tube was changed to a O Shiley trachotomy tube from an endotracheal tube when it arrived and this was secured.  An ABG prior to the tracheotomy showed a carbon dioxide pressure of 144, oxygen pressure 20, and base deficit of =16.  Ten mEg of bicarbonate and 35cc normal saline were given again.  A repeat chest x-ray showed a trache in the right main stem and an increased right pneumo with hypoplastic appearing lungs.  Arterial blood gas at 6am after the trache had a pH of 6.93, carbon dioxide pressure 126, oxygen pressure 67, and base deficit -12.  21,000 hematocrit 42, and platelets 229.  Another 8 Frech Argyl chest tube was inserted on the right side with total evauation of the pneumothorax on the chest x-ray.  The impression was of a term AGA female; fused jaw, status post tracheotomy, two right chest tubes secondary to pneumothorax; rule out congenital heart defect; spinal anomalies; and rule out dysmorphic syndrome.  The plan included a trach, transfer to another hospital, dysmorphism consult, and heart echo.  It was noted the mother was O-positive, rubella immune, HPR nonreactive, hepatitis b negative, HIV negative, chlamydia negative, and GC nonreactive.  Follow up was received from the mother.  The anomalies included "a fused gumline so that there was no opening for her to take in amniotic fluid.  They did a tracheotomy at birth so that she could breathe."  Other anomalies included "a deformed heard with one chamger, underdeveloped lungs, and problems with her vertebrae."  An autopsy was being performed.  The mother was scheduled to meet with her infant's physicians in four weeks to obtain the results.  It was noted "the anomalies were not being attributed to anything at this point and there may never ben a definitive answer."  Additional follow up was received.  The infanct was subsequently transferred to another hospital.  An echocardiogram demonstrated a Shone's variant with small left atrium and abnormal nitral valve, and a small left ventricle with an abnormal aortic valve.  In addition, normal atrial anatomy with a fenestrated patent foramen ovale with left-to-right flow was seen.  No mitral regurgitation or tricuspid regurgitationw as identified.  A single perimembranous VSD with left-to-right flow was seen.  The infants prognosis was discussed with the family who elected to discontinue fesuscitative effots.  The infant was pronouned dead on 08/02/2000 at 2052 hours.  Skin was taken from the baby for a fibroblast culture.  An autopsy revealed left hypomandibular craniofacial dysostosis.  left choanal stenosis, right choanal stresia, micrognathia, lowset ears, right tongue hypoplasia, right mandibulomaxillary fusion, bilateral single palmar transverse creases, and mild cerecellar dentate dysplasia.  The infant had a hypoplastic left hear with mitral and aortic valve and severe left subaortic, small atrial septal defect (prinum type), small atrial septal defect (secundum type), bilateral distal coronary artery ostial origin, and vertebral anomalies.  The anatomic diagnosis of pulmonary hypoplasia (combined lung weight 38.3g; expected 52g), acute bronchopneumonia, especially right lung (multifocal), diffuse alvelolar damage with right lung hyaline membrane formation, diffuse severe pulmonary congestion, and bilateral mutifocal pulmonary hemorrahage.  Hepatobiliary system revealed hepatic sinusoidal erythropoiesis.  Lyumphorecticular system revealed acute diffuse thymic involution, and thymus and spleen vascular congestion.  The ventricular septum was unremarkable except for a 0.3cm subaortic membranous defect situated approximately 0.2cm beneath the aortic valve and beneath the anterior tricuspid leaflets.  Muscular defects were not identified.  The cordae tendinease and papillary muscles of the mitral valve were normal except for being small.  The posterior basal margin of the left ventricle was rounded, indicated hypertrophy. The right and left coronary artery ostia were of normal dimension but arose approximately 0.1 to 0.2 cm distal to the sinus of Valsalva.  Coronary artery thrombosis and ansurysus were not seen.  In summary, the heart displayed mitral and aortic valve and severe left ventricular hypoplasis, aortic septal defect, small secundum type atrial septal defect, and bilateral distal coronary artery ostial placement.  The mid and lower thoracic spine demonstrated multiple bony segmentation anomalies.  The abnormal vertebral bodies had associated rib anomalies.  The bone marrow within the ribs was red.  The lungs displayed diffuse severe vascular congestion in associaten with multifocal pleural and interlobular septal hemmorhage.  Focal mild intra-alveolar hemorrhage was also seen, lower lobes while they were extremely sparse and mild in the sections from the left lung.  The right lung sections also showed focal epithelial necrosis and hyaline membrane formation in the respiratory bronchioles and ducts.  Histologic sections of the diaphragm showed skeletal muscle bundles separated by fibroconnective tissue with focal interstitial hemmorhage.  Portions of hyocardium sampled from both atria dn both ventricles demonstrated diffuse hemorrhage and congestion.  Sections from the gastroesophageal junction showed an unremarkable nonkeratinized stratified demonstrated focal erythropiesis within sinusoids.  Portal tracts demonstrated focal vascular congestion.  The spleen displayed diffuse vascular congestions but was otherwise unremarkalbe.  The thyroid was within normal limits.  Sections of the adrenal glands demonstrated cortical and medullary congestion with focal hemorrhage.  The scalp and gales were mildly edematous.  Histologic sections of the frontal, temporal, and occipital lobes showed perinuclear vacuolization and vascular congestion.  Histological sections of the thalamus, hippocampus and basal ganglia demonstrated prominent perinuclear vacuolization and mild vasular congestion.  Follow up was received which indicated autopsy speciments will be submitted for PCR analysis.    No further information is available.Additional follow up wa received which indicated the autospy  specimena were tissue in paraffin and the cord in fromalin. The paraffin block was betaglobin positive and VZV negative, therefore the final result was that the specimen was negative by PCR for VZV. The cord in formalin was botaglobin negative and the specimen was therefore inadequate. No further information is available.