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CASE REPORT
Year : 2009  |  Volume : 53  |  Issue : 4  |  Page : 501-503 Table of Contents     

Peters' Anomaly-Anaesthetic Management


1 Senior Resident, Department of Anaesthesiology & Intensive Care Unit, All Inda Institute of Medical Sciences, New Delhi, India
2 Professor, Department of Anaesthesiology & Intensive Care Unit, All Inda Institute of Medical Sciences, New Delhi, India

Date of Web Publication3-Mar-2010

Correspondence Address:
M Senthilkumar
Room No. 506, Resident Doctor Hostel, Masjid Moth, New Delhi 110049
India
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Source of Support: None, Conflict of Interest: None


PMID: 20640218

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Peters' anomaly occurs as an isolated ocular abnormality, in association with other systemic abnormalityor one component of a number of well-defined syndromes. We review our experience of anaesthetic management and systemic association of peters' anomaly. To the best of our knowledge there are no reports in the literature of Peters' anomaly with relevant to anaesthesia.

Keywords: Peters′ anomaly, Systemic malformation, Anaesthetic management


How to cite this article:
Senthilkumar M, Darlong V, Punj J, Pandey R. Peters' Anomaly-Anaesthetic Management. Indian J Anaesth 2009;53:501-3

How to cite this URL:
Senthilkumar M, Darlong V, Punj J, Pandey R. Peters' Anomaly-Anaesthetic Management. Indian J Anaesth [serial online] 2009 [cited 2020 Oct 20];53:501-3. Available from: https://www.ijaweb.org/text.asp?2009/53/4/501/60327


   Introduction Top


Peters' anomaly was first described in 1906 by a German Ophthalmologist, Dr. Alfred Peters. It is char­acterized by a central corneal opacity due to defect in the corneal endothelium, Descemet's membrane, and posterior stroma. Systemic associations with Peters' anomaly include trisomy 13-15, partial deletion of chromosome arm 1 lq, and Norrie disease [1] . Major ophthalmologic complications in patients with Peters' anomaly include glaucoma and deprivation amblyopia. Despite aggressive surgical and post operative care, almost 50% ofpatient lose all light perception, mainly because of glaucoma.


   Case Series Top


Nine patients with Peters' anomaly, admitted for various eye procedures in our ophthalmic institute. Di­agnosis was made by the ophthalmologist and paediatrician. Patient's characteristics are described in [Table 1]. All children underwent preoperative examina­tions and major congenital malformations were re­corded. Investigations were done as per the require­ment. Children age less than three years and more than 6 months were premedicated with promethazine syrup 2 mg.kg -1 . Anaesthetic technique was planned accord­ingto their clinical involvement.

Five patients admitted for examination under ana­esthesia(EUA). Inthe operatingtable, ECGynon inva­sive blood pressure and oxygen saturation were estab­lished (Datex Ohmeda). General anaesthesia was in­duced with O 2 :N 2 0 and sevoflurane 5-8 %. Intrave­nous (IV) access was secured after induction. Anaes­thesia was maintained with using O 2 :N 2 0: isoflurane mixture. In all children spontaneous respiration was maintained with a face mask attached to T - piece. Laryngeal mask airway (LMA) was inserted in two patients because ofdifficulty in holding mask due to mandibular hypoplasia. The procedures were com­pleted without any complications.

Three patients were operated for optical iridectomy and one for keratoplasty. Anaesthesia was induced with inhalational agent, using 0 2 :N 2 0 and sevoflurane 5 - 8 %. IV line was secured after induction and fentanyl was given 1-2 mcg.kg -1 for analgesia. LMA was inserted when an adequate depth of anaesthesia was achieved (MAC- LMAinsertion for sevofluranre 2.0-2.5%). Ana esthesiawas maintained with O 2 :N 2 0: isoflurane mix­ture and pressure- controlled ventilation was used intra­operatively by adjusting the pressure to maintain normocarbia. Muscle relaxation was achieved using atracurium (0.1mg. kg -1 ) and rectal paracetamol (40mg.kg -1 ) was inserted in allthe patients for postop­erative analgesia. We encountered difficulty in intubating the trachea of 1 1/2 month old baby who was operated for uidectomy.Tracheal intubation was planned but couldn't be intubated in the fast attempt due to man­dibular hypoplasia and anterior larynx. Finally intubation was done after fifth attempt with stylet and BURP ma­neuver.

Intraoperatively ECG, non-invasive blood pres­sure, end-tidal carbon dioxide and oxygen saturation were monitored. At the end of surgery, neuromuscular blockade was reversed with neostigmine (50-70mcg.kg -1 ) and glycopynolate (10mcg.kg -1 ). Extubation was per­formed with the child fully awake. All patients were shifted to post anaesthesia recovery room for monitor­ing and oxygen was administered by face mask till they were fit to be discharged or shifted to ward.


   Discussion Top


Anaesthetic management of Peters' anomaly varied dependingon the clinical manifestations and as­sociated other anomalies. Anaesthetic consideration includes possibility of difficult airway; presence of con­genital heart disease and other systemic anomalies. Systemic associations are described in the [Table 2]. Midline body structures seem to be involved. All associated systemic anomalies appeared to arise from maldevelopment of the neural crest cells [2] .

Most common heart disease is acyanotic heart disease with leftto right shunt. In our case series, four patients had acyanotic heart disease with left to right shunt(VSD, PDA, ASD). Inhalational induction was done in all children using sevoflurane as sevoflurane has been shown to preserve myocardial function better in patients with congenital heart disease (CHD)as com­pared with halothane [3] . In our case series there were no significant hemodynamic changes after induction.

In Peters' anomaly airway management may be difficult in patients with significant dysmorphic facial features, especially with micrognathia and cleft lip/pal­ate. The anterior mandibular space is reduced due to micrognathia, thus makungtracheal intubation more dif­ficult in Peters' anomaly. In our case series, five pa­tients had anticipated difficult airway problems (micro­gnathia, cleftlip/palate, smallmouth opening, anterior larynx). We used LMA in these patients without any difficulty. The otheradded advantages of using LMAin ophthalmic procedure are: (i) intraocularpressure and coughing are lower with the LMAthan the tracheal tube (TT) (ii) Anaesthetic depth requirements are lower for the LMAthanthe TT [4] . Fibreoptic intubation should be considered in patient with limited mouth opening and cervical vertebral anomaly. Although not a significant feature of this syndrome, patients with hyperor hypo mobility joint disease may require careful positioning and padding.

Mental retardation is common in majority of these patients and seizures have been less reported. In our case series none of the children had mental retardation and seizures. Mental retardation may limit patient co­operation, therefore sedative and/or anxiolytic premedi­tation maybe helpful. In 1984, van Schooneveld et al first proposed theterm Peters'-plus syndrome, com­prising Peters anomaly, face anomalies, clefting, short limb dwarfism, and retarted development [5] . In our case series, some patients presented with some of these find­ings. However, no cases completely fulfilled the criteria for this syndrome. One important differential diagnosis is anterior chamber cleavage disorder (Jung wolff back stahl syndrome) which includes cerebellar hypoplasia, hypothyroidism, andtracheal stenosis [6] . Peters' anomaly with history of airway obstruction should always be screened fortracheal stenosis and hypothyroidism.

Preoperative echocardiography, neumimaging studies and other diagnostic studies should be done whenever indicated. The ability to detect associated systemic anomalies inthe early stage is important, be­cause early treatment for those anomalies is essential for nonnal development. We have been referring such patients for examination to a paediatrician on their first visit. We feel that patients with Peters' anomaly be screened for systemic malformations and anaesthetic implication is based mainly on the presence of systemic anomalies and difficult airway.



 
   References Top

1.Elias I. Traboulsi, MD, Irene H. Maumenee, MD. Peters' anomaly and associated congenital malformations. Arch Ophthalmol 1992;110:1739-1742.  Back to cited text no. 1      
2.Ozeki H, Shirai S, Nozaki M, Sakurai E, Mizuno S, Ashikari M, Matsunaga N, Ogura V Ocular and systemic features of Peters' anomaly. Graefe's Arch Clin Exp Ophthalmo1200023 8:833-839.  Back to cited text no. 2      
3.Rivenes SM, Lewin MB, Stayer SA, Bent ST, Schoenig HM, McKenzie ED, Fraser CD, Andropoulos DB. Car­diovascular effects of sevoflurane, isoflurane, halothane, and fentanyl-m idazolam in children with congenital heart disease: an echocardiographic study of myocardial con­tractility and hemodynamics. Anesthesiology 2001; 94:223-9.  Back to cited text no. 3      
4.Akhtar Tlvl, McMurray P, Kerr WJ, Kenny GN. A com­parison of laryngeal mask airway with tracheal tube for intra-ocular ophthalmic surgery. Anaesthesia 1992; 47:668-71.  Back to cited text no. 4      
5.Van SchooneveldMJ, Delleman JW, BeemerFA Bleeker­Wagemakers EM. Peters'-plus: a new syndrome. Oph­thalmic Paediatr Genet 1984;4 :141-5.  Back to cited text no. 5      
6.Jung C. Wolff G, Back E, et al. Two unrelated children with developmental delay, short stature and anterior chamber cleavage disorder. Cerebellar hypoplasia, endocrine disturbances and tracheal stenosis: A new en­tit1/? ClinDysmorphol 1995;4:44-51.  Back to cited text no. 6      



 
 
    Tables

  [Table 1], [Table 2]



 

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