Right Microtia

 

Sex: F, Age: 1 month old

Preterm 35 weeks – AGA

C. Section - Breech

Oligohydramnios

Rt. Microtia

Small PFO

 

The neonate was born after 35 weeks +3/7 of pregnancy, weight 2340 gr., to a 39 years old mother, third delivery, fourth pregnancy. Mother is generally healthy, blood type A+.

Pregnancy – Spontaneous. Amniocentesis: normal chromosomes. Ultrasound scans: Organ Scan at 15 weeks – normal , besides 2 echogenic foci in the heart (golf balls). Fetal echocardiography – normal.

Oligohydramnios.

Rupture of membranes at delivery – clear fluid.

Delivery – C. Section due to breech presentation and Oligohydramnios.

Was born in a good general condition. APGAR score 9-1’ , 10-5’.

On Admission – In a good general state. Skin color – Pink. Vivacious, normal perfusion, saturation – 100%. Body temp 36.2 C°, BP - 93/42, HR – 149 ,breathing rate – 25/m.

Phyisical examination:

Head: symmetric, head circumference - 32.5 cm, length - 46 cm.

Auricle of right ear is not developed.

Lungs: Breathing rate of 25, no retractions. Normal ventilation of both lungs.

Heart: normal heart sounds. Femoral pulse, bilateral – normal.

Abdomen examination: normal.

Neurological examination: Alert, reflexes are normal.

Genitalia – female, normal.

Limbs – wide spread, feet - normal.

Neurologically: normal. Normal examination on dismissal.

Head Ultrasound : normal on ---.

Respiratory: No respiratory disease. Discharged with normal room air saturation.

Cardiovascular: Thermodynamically stable throughout hospitalisation.

Echocardiography have demonstrated: small PFO.

Metabolically: Feeding from a bottle was initiated at day 1, based on a formula and mother’s milk. Showed good absorption and weight gain.

Renal: normal renal function during hospital stay. Renal function: Creatinine 0.88, Urea 12mg/ml. Renal ultrasound - normal

Jaundice: Bilirubin levels peaked to 11.7 mg% on the eighth day of life. Mother’s blood type A+, neonate A+, Coombs – negative.

Genetics, on examination by a genetic clinician : “Microtia of right ear, left ear is normal, with no other dysmorphism. It seems like a single finding and not part of a syndrome. Echocardiography and renal ultrasound are normal. Eye examination is normal”.

ENT, on examination by a ENT physician : “Rt. Microtia. It is recommended to complete hearing test, and if normal – postpone imaging to a later stage in life”.

Liver function tests: normal

Haematology: complete blood count : Wbc-10,650, Plt – 433,000, Hct-63%.

Thyroid: tests were taken. By the time this report is written, no results yet.

Eyes: normal eyes test.

Hearing: Screening test was performed – passed in left ear. BERA – normal

 

The newborn is discharged at day 8. (36 weeks), in a good general condition. Normal physical examination except Lt. Microtia.

Weight on discharge – 2188 gr., head circumference 34 cm. , length 46.7 , Hct – 54%.

Among routine recommendations on discharge the newborn was scheduled for: ENT clinic (at one month), Cardiology clinic (at 4 months), Plastic surgery clinic (at 2 months), Child development clinic, ABR at Audiology clinic, Genetic clinic (at 3 months),Eye clinic (at 2 months).

 

Photos are attached to this report.

 

 

Microtia is a genetic malformation of the auricle which effects 1 in every 8,000 infants in the general population occurring more often in right ears as is the case with this patient. The cause of microtia remains unknown or multifactoral in most cases. In less than 15% a family history of similar lesions is reported. Most cases are isolated and unilateral. However, microtia may be associated with other congenital malformations (question #2). 30% of associated malformations involve facial clefts and cardiac defects. It seems that the patient does not have a facial cleft and she will be further investigated in the cardiology clinic. 14% may have anopthalmia or microthalmia. I noticed an appointment for the eye clinic for the patient. 11% may have limb reduction or severe renal malformations. Rarely, it might be associated with holoprosencephaly and various chromosomal aberrations.      

 

The diagnostic procedures performed and ordered by the patient's physicians appear appropriate (question #1). Consulting ENT, cardiology, plastic surgery, eye clinic, genetics, audiology and child development are all in order. It is important to emphasize the assessment of hearing in the left ear. Screening test was normal, which is encouraging, but as recommended by your ENT specialist a complete hearing test is recommended because treatment schedule differs in case hearing loss is bilateral or unilateral. 

 

Ultrasound of the kidneys appears normal but I would recommend to follow the patient in Nephrology clinic to exclude the possibility of a malfunction which was not detected by ultrasound.

 

Finally, genetics will assess a possible association with a defined syndrome of chromosomal abnormality which seems unlikely in the patient's case.

 

(Question #3) the technique of choice for the reconstruction of the microtia ear is the use of autogenous rib cartilage. Rib cartilage is sufficient for harvesting only after the age of 6 years old. Also, by that age the auricle has reached 95% of its adult size and the psychological impact does not become evident until the child reaches 7-10 years old.

 

It is therefore recommended to start these multistage surgeries at ages 6-10 years, most prefer 7- 8 years of age.

 

Other reconstructive techniques can start earlier because they depend on prosthetic materials. However, they are preferred by only 11% of patients because of aesthetics, the need for frequent replacement, high rate of failure and the fact that autogenous reconstruction is precluded if a prosthetic ear has been tried before. 

 

(Question #4) Vast experience as well as good artistic abilities in reconstruction is very important in achieving a good final result.