Indian Journal of Anaesthesia  
About us | Editorial board | Search | Ahead of print | Current Issue | Past Issues | Instructions
Home | Login  | Users Online: 521  Print this pageEmail this pageSmall font sizeDefault font sizeIncrease font size    




 
CASE REPORT
Year : 2008  |  Volume : 52  |  Issue : 6  |  Page : 844 Table of Contents     

Anaesthetic Management of Caesarean Section in an Achondroplastic Dwarf


1 Professor, Department of Anaesthesiology, Maulana Azad Medical College & Associated Lok Nayak Hospital, New Delhi, India
2 Senior resident, Department of Anaesthesiology, Maulana Azad Medical College & Associated Lok Nayak Hospital, New Delhi, India
3 Assistant Professor, Department of Anaesthesiology, Maulana Azad Medical College & Associated Lok Nayak Hospital, New Delhi, India
4 Associate Professor, Department of Orthopaedics, Maulana Azad Medical College & Associated Lok Nayak Hospital, New Delhi, India
5 P.G.Student, Department of Anaesthesiology, Maulana Azad Medical College & Associated Lok Nayak Hospital, New Delhi, India

Date of Acceptance29-Sep-2008
Date of Web Publication19-Mar-2010

Correspondence Address:
Kirti N Saxena
B-302 Gitanjali Apartments, Vikas Marg Extension, New Delhi-110092
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions
 

Achondroplasia is the commonest form of dwarfism which results from abnormal cartilage formation at epiphyseal growth plates .It is also known as short-limbed dwarfism. Anaesthetic management of caesarean section in these patients requires a deep understanding of this condition.
A twenty year old parturient with short stature presented to the hospital in early labour. An elective lower segment caesarean section(LSCS) was planned in view of cephalopelvic disproportion. She had papers which suggested that she had been diagnosed as a case of achondroplasia though details were not available. Combined spinal epidural(CSE) anaesthesia was planned in the patient in view of the death of her first baby following caesarean section under general anaesthesia. Repeatedly dry taps were achieved on attempting dural puncture. Dural puncture was abandoned and an 18 G epidural catheter was threaded via the Tuohy needle. Sensory block till T 6 was achieved with 6ml of local anaesthetic solution. The patient was stable during the intraoperative and postoperative period.

Keywords: Achondroplasia, Dwarf, Epidural anaesthesia, Caesarean section


How to cite this article:
Saxena KN, Kapoor D, Taneja B, Maini L, Hora S. Anaesthetic Management of Caesarean Section in an Achondroplastic Dwarf. Indian J Anaesth 2008;52:844

How to cite this URL:
Saxena KN, Kapoor D, Taneja B, Maini L, Hora S. Anaesthetic Management of Caesarean Section in an Achondroplastic Dwarf. Indian J Anaesth [serial online] 2008 [cited 2019 Nov 22];52:844. Available from: http://www.ijaweb.org/text.asp?2008/52/6/844/60699


   Introduction Top


Achondroplasia is the commonest form of dwarfism which results from abnormal cartilage forma­tion at epiphyseal growth plates [1] . It is also known as short-limbed dwarfism. Anaesthetic management of caesarean section in these patients requires a deep un­derstanding of this condition.


   Case report Top


A twenty year old parturient with short stature presented to the hospital in early labour. She had a term pregnancy and her antenatal course had been unevent­ful. An elective lower segment caesarean section (LSCS) was planned in view of cephalopelvic dispro­portion. On preanaesthetic assessment there was no significant past history except that she had undergone a previous LSCS under general anaesthesia two years back and the baby died after birth. However no records were available. The family history revealed that her father and both siblings were short statured. She had papers which suggested that she had been diagnosed as a case of achondroplasia though details were not available.

On examination she was seen to be short statured with a height of 115cm and her weight was 45 kg. She had a large head with frontal bossing, a saddle nose[Figure 1], short neck and short limbs[Figure 2]. Her air­way examination placed her in Mallampatti grade II and she had full range of neck movement. Examination of her spine showed mild kyphosis in the lumbar area.

The patient was administered ranitidine (50mg) and metoclopramide (10mg) for aspiration prophylaxis. Her informed consent in view of short stature with ky­phoscoliosis was taken. Combined spinal epidural (CSE) anaesthesia was planned in the patient in view of the death of her first baby following caesarean sec­tion under general anaesthesia . Equipment for man­agement of difficult intubation was kept to take care of any complications of the procedure. E.C.G , non inva­sive blood pressure and pulse oximeter monitoring were instituted and she was preloaded with 500 ml of Ringer's lactate solution.

The patient was put in the sitting position for CSE .Using a 17G Tuohy needle, epidural space was iden­tified at L 3 - L 4 ( 3 cm from skin ). A 27 G Whitacre needle was introduced via the Tuohy needle to per­form dural puncture however repeatedly dry taps were achieved. After this another attempt at subarachnoid block was made in the L4-L5 space with another 25 G spinal needle keeping the Tuohy needle in place. Again a dry tap was obtained. Dural puncture was aban­doned and an 18 G epidural catheter was threaded via the Tuohy needle and fixed at a depth of 7 cm. 2 ml of 2% lidocaine with 1: 200000 adrenaline was given as test dose.The patient was placed supine with a slight head up tilt and a wedge was placed under the right hip.

After waiting for 5 minutes to confirm proper placement of epidural catheter , 4 ml of 2 % lidocaine with 1:200000 adrenaline was given via epidural cath­eter in graded doses of 2ml. Sensory block till T 6 was achieved. Surgery was commenced and a male infant weighing 2.67kg was delivered after 10 min. The APGAR score was 9 at 1 and 5 minutes and there was no feature suggestive of achondroplasia in the baby.

The patient was stable during the intraoperative period. Post operative analgesia was given by tramadol 25 mg in 6 ml normal saline via the epidural catheter and intramuscular diclofenac 50 mg. The post opera­tive period was uneventful and the mother and baby were discharged after 7 days.

During the postoperative period we decided to investigate the patient. The patient was assessed by an orthopaedic surgeon. who advised certain X-rays to clinch the diagnosis. These revealed an enlarged calva­rium with shortening of skull base.The distal end of ulna appeared medially directed (reverse madelung deformity).There was deepened intercondylar notch in distal femur epiphysis and the joint space appeared reduced .There was mild reduction of AP diameter of vertebrae with posterior scalloping and progressive reduction in interpedicular distance along L 1 to L 5 ver­tebrae. In addition to these there was squaring of iliac wings (tombstone appearance), narrowing of the pel­vic cavity, reduced sacrosciatic notch, flattened ac­etabular roof with shortening of femoral necks. All these features were found to be characteristic of achondro­plasia [2] .


   Discussion Top


There are greater than hundred different types of dwarfism, many of which have specific implications for the anaesthesiologist. Although each particular disor­der is relatively rare, the large number of dwarfs en­sures that any practicing anaesthesiologist is likely to encounter these patients [3] . People with severe short stat­ures can be divided into two categories:­

  1. Midgets ,who have proportionate growth and nor­mal ratio of trunk to limb length.
  2. Dwarfs, having short stature and disprortionate de­velopment characterised either by short limbs, or short trunks.


Achondroplasia (abnormal endochondral bone formation) is the most common type of disproportion­ate short stature. It is an autosomal dominant genetic condition but 80% of cases result from a spontaneous mutation [4] . Females are affected more frequently than males. Anaesthesia, whether general or regional poses many potential problems in these patients specially dur­ing the later stages of pregnancy. Achondroplastic dwarfs characteristically have low fertility rate and those who do conceive ,often require caesarean section be­cause the normal sized fetal head and smaller than the normal maternal pelvic diameter results in cephalopel­vic disproportion during the later stages of pregnancy [5] . Additionally the fetus may have a narrow foramen magnum and upper cervical spine , creating the poten­tial for spinal cord compression during neck manipula­tion [1] .

These patients have several derangements which have potential for complications during administration of anaesthesia. Narrow nasal passages and nasophar­ynx, large tongue and mandible, short neck, craniofacial abnormalities such as macrocephaly, frontal bossing, depressed nasal bridge, maxillary hypoplasia and occipitalisation of the first cervical vertebra can all lead to difficulty in mask ventilation, glottic opening visualisation and intubation [6] . Tracheomalacia may also be present. Review of all previous anaesthetic records regarding airway management should be done. Preop­erative evaluation of the airway by various radiological investigations like CT scan and MRI is indicated. Awake fiberoptic intubation has been recommended [1] .

Restrictive lung disease is common in these pa­tients due to thoracic dysplasia(rib hypoplasia), tho­racic lordosis and thoracic kyphoscoliosis. There is ventilation perfusion mismatching due to decreased FRC and increased closing volume promoting atelecta­sis which require thorough evaluation of pulmonary function. In addition to this there is a tendency for sleep apnea which may be central or obstructive in nature due to craniofacial abnormalities and hypotonia of up­per airway muscles [3] . These can lead to development of pulmonary hypertension. Compression of spinal nerve roots can lead to development of neuromuscular weakness. Because of all these problems there may be difficulty in maintaining oxygenation during general anaesthesia and post operative mechanical ventilation may be required .

Spinal cord and nerve root compression syndrome due to prolapse of intervertebral discs into the stenosed spinal canal can lead to slowly progressing parapare­sis, quadriparesis, sensory deficits, sphincter deficits, autonomic hyperreflexia [3] . Suxamethonium should be avoided as it can lead to life threatening hyperkalemia in presence of peripheral denervation There is constriction of the spinal canal resulting in narrowing of sub­arachnoid and epidural spaces which may cause diffi­culty in establishment of central neuraxial blockade.

Pregnancy in achondroplastic dwarfs leads to fur­ther problems as the uterus remains an entirely intraabdominal organ because there is failure of normal sized fetal head to engage in narrow pelvic inlet [5] . Thus, diaphragmatic splinting occurs causing further reduc­tion in FRC and there is severe aortocaval compres­sion.

There are several reports of parturients with achondroplasia undergoing caesarean section success­fully under both general anaesthesia [7],[8],[9] as well as under central neuraxial blockade however difficult intubation has been encountered [10],[11] This decision may be guided by various factors such as expected difficulty in airway management, whether caesarean section is emergency or elective in nature, history of previous anaesthesia, history of prior laminectomy and individual variations in the cases. The possibility of atlanto-axial instability, restrictive lung disease and neuromuscular weakness would influence the decision to avoid general anaes­thesia. In our patient we avoided giving general anaes­thesia because we suspected that the death of her baby could have occurred during the previous caesarean sec­tion under general anaesthesia due to presence of pul­monary hypertension in the mother leading to hypoxaemia in the baby. Secondly, the author has suc­cessful experience of giving CSE to several short statured parturients with kyphoscoliosis for caesarean section.

Caution has been advised against giving regional anaesthesia as neurological anomalies caused by spinal anomalies may be attributed to it [12] . Problems may be encountered while giving central neuraxial blockade because of difficulty in identifying the interspinous spaces due to lumbar hyperlordosis, reduced interpedencular distance, osteophytes formations and malformed verterbrae. There may be spinal stenosis making it diffi­cult to perform spinal anaesthesia [13] . In our patient, re­peatedly dry tap was obtained on attempting lumbar puncture. It has been advocated that single shot sub­arachnoid block should be avoided due to unpred­ictability of spread [14] as the spinal cord may end at a lower level [7] . However spinal anaesthesia has been given successfully without any sequelae in such a patient. Sensory blockade tillT3 was obtained with 1.3 ml of 0.5% hyperbaric bupivacaine given in the sitting posi­tion [15] .

Epidural anaesthesia has been given for caesar­ean section in several parturients with achondroplasia by several authors, all of whom used an epidural cath­eter to give graded doses of local anaesthetic [4],[12],[13] . A large variation in the heights of the patients and indi­vidual variations would have an effect on the levels achieved. Different volumes of test doses have also been used keeping in mind the short stature of these patients as accidental subarachnoid injection could have lead to a high level of blockade as happened in one of these patients [13] .Keeping these factors in mind we gave a test dose of 2ml of 2% lidocaine with 1:2,00,000 adrena­line followed by 4ml of the same solution to achieve a level of T6 in our patient.There might still have been need for emergency tracheal intubation in the patient in case of any complication therefore equipment for tack­ling a difficult airway was kept. No such need has been reported in any of the above mentioned reports and the postoperative period was eneventful. CSE (titrable) is a good option for urgent caesarean section despite the small amount of added time required for this technique. It has been used for myomectomy in such a patient [6] .

The risk of general anaesthesia versus regional anaesthesia must be considered for each patient as se­verity of spinal abnormality can vary considerably.The chances of successful epidural anaesthesia in achon­droplastic patient will be maximized by careful patient selection and preparation, with discussion of risks and possibility of technical complications, optimal positioning and cautious dose titration.

 
   References Top

1.Tetzlaff JE. Skin and bone disorders. In:Anaesthesia and uncommon diseases.Published by Saunders.5th Edition 2006:327-29.  Back to cited text no. 1      
2.Adam Greenspan.Scoliosis and anomalies with general effect on the skeleton. In:Orthopaedic imaging .A prac­tical approach.Published by Lippincott Williams and Wilkins.4 th Edition 2004:943.  Back to cited text no. 2      
3.Berkowitz ID, Raja N,Bender KS, Kopits SE.Dwarfs: Pathophysiology and anaesthetic implications. Anes­thesiology 1990;73:739-759.  Back to cited text no. 3      
4.Carstoniu J,Yee I,Halpern S.Epidural anaesthesia for caesarean section in an achondroplasic dwarf.Can J Anaesth 1992;39:708-11.  Back to cited text no. 4  [PUBMED]    
5.Allanson JE, Hall JG. Obstetric and gynaecologic prob­lems in women with chondrodystrophies.Obstet Gynecol 1986;67:74-8.  Back to cited text no. 5  [PUBMED]    
6.Gupta S,Meena R,Nagarwal PR. Combined spinal epi­dural anaesthesia for myomectomy in an acondroplastic dwarf. Indian J Anaesth 2005;49:430-431.  Back to cited text no. 6      
7.Bancroft GH,Lauria JI .Ketamine induction for caesar­ean section in a patient with acute intermittent porphy­ria and achondroplastic dwarfism.Anesthesiology1983;59:143-144.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Mc Arthur RDA. Obstetric anaesthesia in an achondroplasic dwarf at a regional hospital. Anaesthe­sia and Intensive Care 1992;20:376-378.  Back to cited text no. 8      
9.Kalla,Fenning E, Obiaya.Anaesthetic management of achondroplasia. Br J Anaesth 1986;58:117-119.  Back to cited text no. 9      
10.Walts LF, Finerman G, Wyatt GM.Anaesthesia for dwarfs and other patients of short stature.Can Anaesth Soc J 1975;22:703-709.  Back to cited text no. 10  [PUBMED]    
11.Mather JS. Impossible direct laryngoscopy in achon­droplasia .Anaesthesia 1966;21:244-248.  Back to cited text no. 11  [PUBMED]    
12.Morrow MJ, Black IH. Epidural anaesthesia for caesar­ean section in an achondroplastic dwarf.Br J Anaesth 1998;81:619-621.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]  
13.Wardall GJ, Frame WT.Extradural anaesthesia for cae­sarean section in achondroplasia.Br J Anaesth 1990;64:367-370.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]  
14.Loughrey JPR,Genc MR.Orthopedic problems and ma­ternal trauma. In:Anaesthetic and obstetric management of high risk pregnancies. Published by Springer 3 rd Edi­tion 2004;236.  Back to cited text no. 14      
15.Ravenscroft A, GovenderT, Rout C.Spinal anaesthesia for emergency caesarean section in an achondroplastic dwarf.Anaesthesia 1998;53:1236-7.  Back to cited text no. 15      


    Figures

  [Figure 1], [Figure 2]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
    Introduction
    Case report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed3518    
    Printed85    
    Emailed0    
    PDF Downloaded508    
    Comments [Add]    

Recommend this journal