NICU AUDIT

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)


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


118bpm RR 47cpm

• Decreased jaundice

• Microtia, right ear

• Good air entry• Full pulses




ear

PLAN• Discontinue

phototherapy• Increase

feedings

GOING DAY 5 OF LIFE




149bpm RR 51cpm

• Jaundice upto upper thigh






ear

PLAN• Reexposure to

single phototherapy

• Increase feedings

GOING DAY 6 OF LIFE




145bpm RR 32cpm

• No jaundice• Microtia, right

ear• Good air entry• Full pulses



mia unspecified, resolved


PLAN• Repeat CBC and

bilirubin levels• Insert peripheral

line and remove UVC

• Transfer to level 2

• Continue phototherapy and antibiotics



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



1/4 10:15am

12.88 0.50 12.6 Increased for Preterm at >day 5 (NV<2mg/dl)


GOING DAY 8 OF LIFE




145bpm RR 32cpm

• No jaundice• Microtia, right

ear• Good air entry• Full pulses



mia unspecified, resolved


PLAN• Repeat bilirubin

levels• Continue

phototherapy and antibiotics



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



1/4 130th HOL


1/6 176th HOL



GOING DAY 9 OF LIFE

SUBJECTIVE• 30-60ml every

3hrs• Good suck,



140bpm RR 32cpm

• Light jaundice chest






ear

PLAN• Repeat bilirubin

levels• Continue

phototherapy• Antibiotics

completed



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



1/4 130th HOL


1/6 176th HOL


1/7 204th HOL



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


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


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


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


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


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

NICU AUDIT

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