nicu audit. preterm via normal spontaneous delivery 29 year old g1p1 (0101) 32 6/7 weeks aog, mt...
DESCRIPTION
1 st Trimester – threatened abortion Consult: Dihydrogesterone and Isoxuprine 3 rd Trimester- Upper respiratory tract infectiom Consult: Cefuroxime MATERNAL HISTORYTRANSCRIPT
NICU AUDIT
Preterm via normal spontaneous delivery29 year old G1P1 (0101)32 6/7 weeks AOG, MT 34 AGA Apgar 8, 9 (H 90’s)
E.Y.
• BW 1930 g• BL 44 cm• HC 30 cm• CC 27 1/2 cm• AC 23 cm
1st Trimester – threatened abortion Consult: Dihydrogesterone and Isoxuprine
3 rd Trimester- Upper respiratory tract infectiom Consult: Cefuroxime
MATERNAL HISTORY
Past Medical History: (+) Bronchial asthma since childhood, no maintenance
medications, last attack: 2010 Ganglion cyst, left hand; s/p excision in 2000
Family History: Diabetes, Hypertension, Asthma
Personal/Social History Unremarkable
OB History: G1 – present pregnancy
Feeding history Mixed feeding, expressed breastmilk+milk formula
1 cord coilFlat fontanelsNo molding, caputMicrotia, right earHematoma, upper lipNo cleft lip or palate(-) alar flaringGood air entry, no retractionsHR 90bpm to 110bpm, Good cardiac activity, Soft abdomenGrossly male genitalia with descending testesFull pulses
PHYSICAL FINDINGS
LIVE PRETERM BABY GIRLSEPSIS UNSPECIFIED
MICROTIA, RIGHT EAR
DIAGNOSIS
Diagnostics: CBC PC, Blood culture and sensitivity, HGT
Therapeutics: UV cannulation D10W 100ml to run at 6.4mk/hr (TFR 80mg/kg/day) Vitamin K 0.1mL/IM Erythromycin eye ointment Hepatitis B vaccine 0.5mL/IM IV antibiotics:
Amikacin 34mg q36hrs (17.6 mg/kg/day) Ampicillin 100mg q12hrs (103.6 mg/kg/day)
PLAN
CBC Hgb Hct WBC St Neu Lym Mon Eos PC12/30 150 45 11.9 58 30 08 04 309 MTG
Abdominal X-ray (12/30): umbilical catheter directed superoposteriorly with its tip at the level of T10. Ileus.
HGT= 95mg/dl
SUBJECTIVE• On NPO• (-)
desaturation• (-) cyanosis
OBJECTIVE• T 370C HR
121pm RR 40 cpm
• O2 sats 98%• Microtia,
right ear• Good air
entry
ASSESSMENT• Preterm
baby• Sepsis
unspecified• Microtia,
right ear
PLAN• UV
cannulation – D10 water 80ml/kg/day
• Ampicillin (103.6mkday) and Amikacin (17.6mkday)
9TH HOUR OF LIFE
SUBJECTIVE• On NPO• (-) desaturations• (-) cyanosis
OBJECTIVE• T 370C HR 121pm RR 21 cpm
• O2 sats 100%• (-) alar flaring• Microtia, right ear• Jaundice to chest• Good air entry
ASSESSMENT• Preterm baby• Sepsis unspecified
• Hyperbilirubinemia, unspecified
• Micotia, right ear
PLAN• Single phototherapy
• Start EBM 2ml every 3hrs
• Bilirubin levels and CBC at 6am
DAY 1 OF LIFE
Blood culture: No growth in 7 days
CBC Hgb Hct WBC St Neu Lym Mon Eos PC12/30/13
150 45 11.9 58 30 08 04 309 MTG
12/31/13 158 47 19.1 9 64 14 10 03 326 STG
TB DB IB Zone12/31 6am 31st HOL
10.82 0.41 10.6 Increased for Preterm at 1.2 DOL (NV<2mg/dl)
Abdominal X-ray (12/30): umbilical catheter directed superoposteriorly with its tip at the level of T10. Ileus.
33RD HOUR OF LIFE
SUBJECTIVE• Good cry and
activity• No cyanosis• No desaturation
OBJECTIVE• T 36.90C HR
129bpm RR 56 cpm
• O2 sats 100%• Microtia, right
ear• Jaundice to
abdomen• Good air entry• Full pulses
ASSESSMENT• Preterm baby• Sepsis
unspecified• Hyperbilirubine
mia unspecified• Microtia, right
ear
PLAN• Shift ampicillin
to cefotaxime• Increase
feedings
GOING DAY 4 OF LIFE
SUBJECTIVE• 10ml every 3hrs• Good suck,
good cry, active• No cyanosis• No jitteriness
OBJECTIVE• T 36.50C HR
118bpm RR 47cpm
• Decreased jaundice
• Microtia, right ear
• Good air entry• Full pulses
ASSESSMENT• Preterm baby• Sepsis
unspecified• Hyperbilirubine
mia unspecified• Microtia, right
ear
PLAN• Discontinue
phototherapy• Increase
feedings
GOING DAY 5 OF LIFE
SUBJECTIVE• 18ml every 3hrs• Good suck,
good cry, active• No cyanosis• No jitteriness
OBJECTIVE• T 36.90C HR
149bpm RR 51cpm
• Jaundice upto upper thigh
• Microtia, right ear
• Good air entry• Full pulses
ASSESSMENT• Preterm baby• Sepsis
unspecified• Hyperbilirubine
mia unspecified• Microtia, right
ear
PLAN• Reexposure to
single phototherapy
• Increase feedings
GOING DAY 6 OF LIFE
SUBJECTIVE• 30ml every 3hrs• Good suck,
good cry, active• No cyanosis• No jitteriness
OBJECTIVE• T 37.40C HR
145bpm RR 32cpm
• No jaundice• Microtia, right
ear• Good air entry• Full pulses
ASSESSMENT• Preterm baby• Sepsis
unspecified• Hyperbilirubine
mia unspecified, resolved
• Microtia, right ear
PLAN• Repeat CBC and
bilirubin levels• Insert peripheral
line and remove UVC
• Transfer to level 2
• Continue phototherapy and antibiotics
Blood culture: No growth in 7 days
CBC Hgb Hct WBC St Neu Lym Mon Eos PC12/30/13
150 45 11.9 58 30 08 04 309 MTG
12/31/13 158 47 19.1 9 64 14 10 03 326 STG
1/4/13 139 42 12.1 67 28 02 03 509
TB DB IB Zone12/31 6am 31st HOL
10.82 0.41 10.6 Increased for Preterm at 1.2 DOL (NV<2mg/dl)
1/4 10:15am
12.88 0.50 12.6 Increased for Preterm at >day 5 (NV<2mg/dl)
Abdominal X-ray (12/30): umbilical catheter directed superoposteriorly with its tip at the level of T10. Ileus.
GOING DAY 8 OF LIFE
SUBJECTIVE• 30ml every 3hrs• Good suck,
good cry, active• No cyanosis• No jitteriness
OBJECTIVE• T 37.40C HR
145bpm RR 32cpm
• No jaundice• Microtia, right
ear• Good air entry• Full pulses
ASSESSMENT• Preterm baby• Sepsis
unspecified• Hyperbilirubine
mia unspecified, resolved
• Microtia, right ear
PLAN• Repeat bilirubin
levels• Continue
phototherapy and antibiotics
Blood culture: No growth in 7 days
CBC Hgb Hct WBC St Neu Lym Mon Eos PC12/30/13
150 45 11.9 58 30 08 04 309 MTG
12/31/13 158 47 19.1 9 64 14 10 03 326 STG
1/4/13 139 42 12.1 67 28 02 03 509
TB DB IB Zone12/31 6am 31st HOL
10.82 0.41 10.6 Increased for Preterm at 1.2 DOL (NV<2mg/dl)
1/4 130th HOL
12.88 0.50 12.6 Increased for Preterm at >day 5 (NV<2mg/dl)
1/6 176th HOL
7.42 0.36 7.18 Increased for Preterm at >day 5 (NV<2mg/dl)
Abdominal X-ray (12/30): umbilical catheter directed superoposteriorly with its tip at the level of T10. Ileus.
GOING DAY 9 OF LIFE
SUBJECTIVE• 30-60ml every
3hrs• Good suck,
good cry, active• No cyanosis• No jitteriness
OBJECTIVE• T 36.50C HR
140bpm RR 32cpm
• Light jaundice chest
• Microtia, right ear
• Good air entry• Full pulses
ASSESSMENT• Preterm baby• Sepsis
unspecified• Hyperbilirubine
mia unspecified• Microtia, right
ear
PLAN• Repeat bilirubin
levels• Continue
phototherapy• Antibiotics
completed
Blood culture: No growth in 7 days
CBC Hgb Hct WBC St Neu Lym Mon Eos PC12/30/13
150 45 11.9 58 30 08 04 309 MTG
12/31/13 158 47 19.1 9 64 14 10 03 326 STG
1/4/13 139 42 12.1 67 28 02 03 509
TB DB IB Zone12/31 6am 31st HOL
10.82 0.41 10.6 Increased for Preterm at 1.2 DOL (NV<2mg/dl)
1/4 130th HOL
12.88 0.50 12.6 Increased for Preterm at >day 5 (NV<2mg/dl)
1/6 176th HOL
7.42 0.36 7.18 Increased for Preterm at >day 5 (NV<2mg/dl)
1/7 204th HOL
8.24 0.35 8.02 Increased for Preterm at >day 5 (NV<2mg/dl)
Abdominal X-ray (12/30): umbilical catheter directed superoposteriorly with its tip at the level of T10. Ileus.
Live preterm baby boy, delivered via normal spontaneous delivery at 32 6/7 weeks AOG, MT 34 weeks, AGA, AS 8,9
Sepsis unspecifiedHyperbilirubinemia unspecifiedMicrotia, right ear
WORKING DIAGNOSIS
MICROTIA
a congenital anomaly of the ear that ranges in severity from mild structural abnormalities to complete absence of the ear
can occur as an isolated birth defect or as part of a spectrum of anomalies or a syndrome
often associated with hearing loss hearing impairment and surgical ear
reconstruction
MICROTIA
Luquetti, D., Heike, C., Hing, A. , Cunningham, M., Cox, T. Microtia: Epidemiology & Genetics. American Journal of Medical Genetics. 2012 January ; 158A(1): 124–139.
prevalence varies among regions, from 0.83 to 17.4 per 10,000 births
considered to be higher in Hispanics, Asians, Native Americans
Males > Females: estimated 20-40% increased risk
Unilateral: 77–93% (right)
MICROTIA: PREVALENCE
Luquetti, D., Heike, C., Hing, A. , Cunningham, M., Cox, T. Microtia: Epidemiology & Genetics. American Journal of Medical Genetics. 2012 January ; 158A(1): 124–139.
low birth weighthigher maternal paritymaternal acute illness and use of medications
(specific acute maternal conditions or medications were not identified in these studies)
maternal diabetes mellitusMultiple births, advanced maternal age, low maternal
education and Hispanic ethnicitypericonceptional intake of folic-acid-containing
supplements has been associated with reduced risk of microtia among non-obese women [Ma et al., 2010]
MICROTIA: RISK FACTORS
Luquetti, D., Heike, C., Hing, A. , Cunningham, M., Cox, T. Microtia: Epidemiology & Genetics. American Journal of Medical Genetics. 2012 January ; 158A(1): 124–139.
General Male sex all authors First parity High parity Multiple births Maternal acute il lnesses Maternal insulin dependent
diabetes Maternal use of medications Advanced paternal age Advanced maternal age Low maternal education Maternal exposure to altitude Maternal residence in an urban
area
Maternal residence in a rural area
Maternal exposure to air pollution
Race/Ethnicity Native ethnicity Hispanic
ethnicity Ecuadorian Chilean Asian, Philippine, Pacific
Islander Teratogens Retinoic acid Thalidomide Alcohol Mycophenolate mofetil
MICROTIA: RISK FACTORS REPORTED IN LITERATURE
Luquetti, D., Heike, C., Hing, A. , Cunningham, M., Cox, T. Microtia: Epidemiology & Genetics. American Journal of Medical Genetics. 2012 January ; 158A(1): 124–139.
Microtia, First Degree Presence of all the normal ear components median longitudinal length > 2 SD below the mean
Microtia, Second Degree Median longitudinal length of the ear > 2 SD below the
mean in the presence of some, but not all, parts of the normal ear
Microtia, Third Degree Presence of some auricular structures, but none of these
structures conforms to recognized ear components.Anotia
Complete absence of the ear.
MICROTIA: CLASSIFICATION SYSTEM
Hunter A, Frias JL, Gillessen-Kaesbach G, Hughes H, Jones KL, Wilson L. Elements of morphology: standard terminology for the ear. Am J Med Genet A. 2009a; 149A(1):40–
60.
vertebral anomaliesmacrostomiaoral clefts facial asymmetryrenal abnormalitiescardiac defectsmicrophthalmiaholoprosencephaly, and polydactyly
MICROTIA: ASSOCIATED ANOMALIES
Luquetti, D., Heike, C., Hing, A. , Cunningham, M., Cox, T. Microtia: Epidemiology & Genetics. American Journal of Medical Genetics. 2012 January ; 158A(1): 124–139.
Approximately 20-60%: have associated anomalies or an identifiable syndrome
Craniofacial microsomiaTownes-Brocks syndromeMandibulofacial dysostoses
Treacher-Collins Nager syndrome
Oculo-auriculo-vertebral spectrum (OAVS): facial asymmetry, microtia, ear and facial tags, epibulbar dermoids, microphthalmia, and macrostomia. [Heike and Hing,2009] Craniofacial, or hemifacial, microsomia and Goldenhar syndrome Extracranial features include renal, cardiac and vertebral
anomalies. Most cases: sporadic, but may be genetic (autosomal>recessive)
MICROTIA: SYNDROMES
Luquetti, D., Heike, C., Hing, A. , Cunningham, M., Cox, T. Microtia: Epidemiology & Genetics. American Journal of Medical Genetics. 2012 January ; 158A(1): 124–139.
hemifacial microsomia, oculo-auriculo-vertebral spectrum, or first and second branchial arch syndrome
approximately 1 in 5500 l ive births sporadically acquired association of anomalies: defect in the 1 s t
and 2nd branchial arches (usually vascular insult) 85-90% unilateral (right) orbital distortion, mandibular hypoplasia ear anomalies (microtia, accessory preauricular tags and/or pits,
and middle ear defects with hearing impairment) facial nerve involvement (hypoplasia of the facial muscles) soft tissue deficiency, such as profound hypoplasia or absence of
the parotid gland and masticatory muscles (temporalis, masseter)
Surgical correction: Preauricular skin tags removed and correction of craniooral comissure
before 2yrs of age Cranial remodeling at 8-12mos
CRANIOFACIAL MICROSOMIA
Autosomal dominantTriad
Imperforate anus (82%) Dysplastic ears (88%)
(overfolded superior helices and preauricular tags) associated w/ sensorineural or conductive hearing impairment (65%)
Thumb malformations (89%) (triphalangeal thumbs, duplication, or rarely, thumb
hypoplasia)Renal impairment (27%),
including ESRD may occur w/ or w/o structural abnormalities (malrotation, ectopia, renal hypoplasia, etc)
Congenital heart disease (25%)
Foot (52%) and genitourinary malformations (36%)
Management: Surgical, hemodialysis
TOWNES-BROCKS SYNDROME
Kohlhase, J. 2012, May 3. Townes-Brocks syndrome. Gene Reviews (extracted from www.ncbi.nlm.nih.gov/books/NBK1445)
mandibulofacial dysostosis autosomal dominant disorder of
craniofacial development1 in 25,000 to 50,000 live birthsClinical features: malar hypoplasia and a cleft in the
zygoma, eyes with antimongoloid slant with colobomas (eyelid notch) along the lateral 1/3 of the lower lid; absent lashes from the medial two-thirds of the lower eyelid: face has a convex profile with a retrusive chin and jaw, associated with an overbite
External ear abnormalities Profound conductive hearing loss: common in severe cases
left lip and palate and choanal atresia
TREACHER COLLINS SYNDROME
Dixon J, Trainor P, Dixon MJ. Treacher Collins syndrome. Orthod Craniofac Res 2007; 10:88.
can result in airway narrowing and respiratory compromisewith airway and feeding difficulties: first couple
years of life tongue-lip adhesion (glossopexy), distraction
osteogenesis of the mandible, tracheostomy, correction of cleft lip and/or palate and choanal atresia, and gastrostomy tube placement
facial abnormalities: 7yrs-oldsurgical correction of the mandible: at 13 to 16
year-old, as the jaw reaches dental and skeletal maturity
TREACHER COLLINS SYNDROME: SURGICAL MANAGEMENT
Acrofacial dysostosis syndromerare disorder, sporadic> genetic similar to Treacher-Collins but w/o eyelid colobomassevere cleft palate (wide defect): always presenthypoplasia or agenesis of the radius and thumbshort staturenormal Intelligence; delays in speech and language
development may occur secondary to hearing impairment
frequently have respiratory and feeding problems: may require gavage feeding or gastrostomy tube placement
High perinatal mortality rate (approximately 11%): related to respiratory compromise
NAGER SYNDROME