Peripartum Anesthetic Management of Patient with Osteogenesis Imperfecta: Case Report and Literature Review

Keywords

Osteogenesis imperfect; Brittle bones disease; Mutations of collagen; Anesthesia; Regional anesthesia; Combined spinal epidural anaesthesia; CSE; Difficult airways; Obstetrics; Caesarean section

Introduction

Osteogenesis Imperfect (OI) (Brittle Bones Disease) is the most common hereditary connective tissue disease. The main clinical forms of OI are quantitative and qualitative anomalies of type I collagen, the most common protein in bones [8].

In modern literature, cases of successful delivery of patients with imperfect osteogenesis are described, however this disease is considered a contraindication to pregnancy, and when it is advised, an artificial interruption is recommended. Such tactic is associated with the occurrence of a significant number of complications, during pregnancy and delivery, sometimes fatal for both the mother and the fetus [8].

Caesarean section is conducted more often in such patients due to maternal pelvic deformity, cephalopelvic disproportion, abnormal fetal position variants [1,8]. In literature, there are few cases of an anesthetic aid for Cesarean Section (CS) in patients with OI (types 3-4).

The clinical observation that is presented, describes features of labor management of a patient with OI type IV.

Case Report

Patient B. 35 years old was admitted to the National Medical Research Center of Obstetrics, Gynecology and Perinatology named after Academician V.I. Kulakov with a diagnosis of pregnancy 31-32 week. Head previa. OI type 4. Condition of multiple fractures from the age of 2.

Pregnancy first. Has come spontaneously. In the I trimester – mild pregnancy anemia. In the II trimester, correction of anemia of the pregnant with iron preparations (Maltofer). At 24 weeks inpatient treatment of the threat of preterm birth was carried out at the place of residency.01/09/2017 An attempt to abort pregnancy. There were 3 unsuccessful attempts to incubate the trachea using endoscopic equipment. Further attempts of abortion were declined due to the fear of the patient for her life. III trimester - threatening premature birth.

In the aftermath, she was repeatedly consulted by the Center’s specialists through telemedicine communications.

For delivery, the patient was admitted to the National Medical Research Center of Obstetrics, Gynecology and Perinatology named after Academician V. I. Kulakov on 09.11.2017. Objectively: the condition was satisfactory, no complaints. Disproportional short stature (height - 92 cm, weight - 23.5 kg). Cannot move independently. Sharply intensified thoracic kyphosis and lumbar lordosis. The general muscular tonus is lowered. Limitation of movement in all limbs. Triangular shaped skull, lower jaw is underdeveloped. Sclera of a blue hue. Airway assessment revealed bad mouth opening (Mallampati class 4) and bad dentition.

There are no respiratory and hemodynamic disorders. In the clinical analysis of blood mild anemia was observed: (hemoglobin 109g/l) other indicators within the limits of the norm. In the biochemical analysis of the blood, moderate hypoproteinemia was observed (Protein 54.9g/L), urine analysis, an ECG - without pathology.

Genetic consultation: the form of the disease is hereditary (Mother – OI type 4).

An ultrasound of the fetus revealed no abnormalities. The fetus corresponds to the 31st week of gestation. Pelvic presentation, transverse position of the fetus. Normal fetoplacental and uteroplacental blood flow.

The indication for operative labor was the increase of dyspnoea at night of the 35-year-old primipara with severe extragenital pathology: OI type IV and transverse position of the fetus. Considering the severe extragenital pathology and the wishes of the woman, a ligation of the fallopian tubes is shown. It was decided to carry out the delivery of the patient by a caesarean section operation in a planned manner.

21/11/2017 a Pfannenstiel incision was conducted, a caesarean section in the lower uterine segment.

Taking into account 3 unsuccessful intubation attempts in the past, it was decided to conduct a cesarean section with a provision of a combined spinal epidural anesthesia, in the presence of endoscopic service.

The initial hemodynamic parameters of the patient: BP 140/78 mm Hg. Heart rate at 104 beats/minute.

Premedication - Dexamethasone 2 mg.

With the patient on the left side, under local anesthesia Sol. Lidocaini 2% - 3 ml, an 18-gauge Tuohy needle was inserted at L2-3 and, using the loss of resistance to saline, the epidural space was identified at 3.0 cm. A 27-gauge pencil-point spinal needle was inserted through the Tuohy needle and after clear cerebrospinal fluid was observed, hyperbaric bupivacaine 0.5% 4 mg was injected. A catheter was then threaded 2 cm into the epidural space. After 7 min sensory block had reached T11 - T12.

Medication: 250 mg Tranexamic acid for bleeding prophylaxis, uterotonic medication - Oxytocin 4 IU, infusion therapy - 380 ml saline, constant norepinephrine infusion at a dosage of 0.01 to 0.08 μg/kg/min was used to maintain hemodynamics.

Surgery was allowed to be proceeded and a female infant weighing 1800 g was delivered with Apgar scores of 7 at 1, 7 at 5 min.

The total duration of the operation was 30 minutes.

Postoperative pain was managed initially with epidural boluses of Ropivacaini 0.2% 2-3 ml and with regular i/v injections of tramadol and dexketoprophen.

The antibacterial prophylaxis was conducted intraoperatively after clamping the umbilical cord -Amoxiclav1.2 g.

Mother and baby were discharged home after seven days.

The child was advised by a geneticist - a blood test was conducted to find mutations in the genes COL1A1, COL1A2, 27.11.17 - no mutations were detected. Blood was taken for genetic research (targeted sequencing) - the result was negative.

Discussion

The frequency of OI is approximately 1 per 20,000 newborns. People with OI caused by mutations of collagen have a 50% risk of transmitting the disease to the child. The proportion of cases caused by the de novo mutation varies depending on the severity of the disease [1,2].

Osteogenesis imperfect is divided into 8 types, the most common are 1-, 2-, 3- and 4-th. The first type of OI is the lightest and most common form of the disease, after which by severity of complications follow 2-, 3- and 4-th types. Recently, types 5, 6, 7 and 8 have been classified, which have the same clinical features as the 4th, but each has unique histological and genetic data [3,4,5].

Along with a high frequency of bone fracture osteogenesis imperfecta is also characteristic of otherclinical manifestations: short stature, scoliosis, a triangular face configuration (large arch of the palate, small lower jaw), deformation of the skull, hearing loss, blue sclera due to reduced collagen content, resulting in a visible pigmentation of the choroid, incomplete tooth formation, loss of connective tissue of ligament apparatus and the heart valves [1].

We found 13 cases of an anesthetic aid for CS in patients with OI (types 3-4) from which: 4 - general anesthesia; 2 combined spinal epidural anesthesia; 2 - epidural anesthesia; 3 - spinal anesthesia (Table 1).

  


    
Year

    
Author

    
OI type

    
Age(years)

    
Weight(kg)

    
Height (sm)

    
Gestation (weeks)

    
Airway Examination

    
Anesthetic technique

    
Anesthetic Details

  

  


    
1977


      [7]

    
Bullard

    
No data

  

  


    
1984


      [9]

    
Cunningham

    
No data

    
27

    
52

    
154

    
38

    
No data

    
Epidural

    
L3-L4.12 ml 0.5% Bupivacaine

  

  


    
1986


      [10]

    
Allanson

    
No data

    
GA

    
No data

  

  


    
1992


      [11]

    
Cho

    
No data

    
20

    
No data

    
No data

    
38

    
No data

    
No data

    
No data

  

  


    
2002


      [12]

    
Vogel

    
4

    
30

    
No data

    
91

    
24

    
MP I, no neck flexion or


      extension, poor dentition

    
Epidural

    
Grade II view on DL; LOR


      at 3 cm; 2% lidocaine 13 mL


      for T4 block

  

  


    
2002


      [12]

    
Vogel

    
3

    
35

    
No data

    
86

    
32

    
MP II, cervical immobility,


      TMD 4 cm, poor dentition

    
GA

    
Grade III-IV view on DL;


      awake nasal fiberoptic


      intubation conducted

  

  


    
2006


      [13]

    
Rudlof

    
3

    
21

    
No data

    
SA

    
No data

  

  


    
2009


      [14]

    
Hwang

    
4

    
28

    
41

    
116

    
36

    
MP II, normal neck


      extension, TMD 3 finger


      breadths, poor dentition

    
SA

    
Bupivacaine 8 mg produced


      T4 block in 5 min

  

  


    
2010


      [15]

    
Murray

    
3

    
24

    
25

    
91

    
35

    
MP I, normal mouth


      opening, normal dentition

    
CSE

    
L3-4, LOR 3.5 cm,


      hyperbaric bupivacaine 4 mg


      with fentanyl 5 μg


      produced T12 block after


      15 min; 0.5% bupivacaine


      5 mL produced T4 block

  

  


    
2010


      [16]

    
Lyra

    
Mild Form

    
23

    
No data

    
No data

    
38

    
No data

    
SA

    
L3-L4 0,5%hyperbaric bupivacaine 10 mg + Morphini    60μg

  

  


    
2011


      [17]

    
Fiegel

    
3

    
27

    
36

    
130

    
31

    
MP 2, limited neck mobility, normal    bite , poor dentition.

    
GA

    
RSI, LOR.

  

  


    
2015


      [18]

    
Dinges

    
3

    
37

    
36

    
111

    
32

    
MP 4,mouth


      opening3 sm, limited neck    mobility,protruding mandibula .

    
GA

    
Video laryngoscopy with    difficulties


      Induction: remifentanil,    propofol, rocuronium 35 mg. maintenance of anesthesia :nitrous oxide+sevoflurane .

  

  


    
2015


      [18]

    
Dinges

    
3

    
37

    
42

    
92

    
28

    
MP3, Mouth opening 4 sm,normal neck


      extension

    
CSE

    
L3-L4 with ultrasound navigation. hyperbaric bupivacaine 5 mg + Fentanil 10 mcg; 2 Lidocaini 2% bolus 3 ml + bicarbonate 0.2 mEq/ml +


  Epinephrine 5 μg/ml – produed T4 block.

  

Table 1: