Paediatric Anaesthesia 1999 9: 359–361

Case reportUnexpected difficult intubation in the patientwith Morning Glory syndrome

YURI SHEVCHENKO MD, MOHAMED REHMAN MD,

ALFRED T. DORSEY MD, ROY E. SCHWARTZ MD AND

RALPH MOSELEY SRNA

Department of Anaesthesia and Critical Care, St. Christopher’s Hospital for Children,Philadelphia, Pennsylvania, USA

SummaryMorning Glory syndrome is an uncommon congenital optic disc

anomaly with occasional systemic associations. A case of

unsuspected difficult intubation in a three-year old patient is

described in this case report.

Keywords: Morning Glory syndrome; difficult intubation

Introduction Case report

Morning Glory syndrome is a congenital optic disc A three-year-old white female with Morning Gloryanomaly characterized by a funnel-shaped, excavated syndrome, right-sided facial nerve palsy andoptic disc surrounded by chorioretinal pigmentary exotropia presented for an elective right lateral rectusdisturbance. Occasionally seen are high myopia, muscle resection.exotropia, and retinal vascular abnormalities. The child was born at full-term and had anGenerally, it is an isolated ocular abnormality, uncomplicated postnatal course. Antenatal historyhowever, systemic associations reported with this was remarkable for maternal substance abuse. Thesyndrome are of importance to the anaesthesiologist. child was adopted and the family history was notBasal myelomeningoceles, midline facial defects such available. The patient’s past medical history wasas hypertelorism, cleft lip and palate, agenesis of otherwise unremarkable. The patient had twothe corpus callosum, renal abnormalities and the surgical procedures for esotropia correction on herCHARGE association have been described with this right eye in the past, performed under generalsyndrome (1–13). There are no reports in the literature anaesthesia.about anaesthetic implications concerning manage- The first surgery was done about a year prior toment of patients with Morning Glory syndrome. this admission at another hospital. Information about

We report a case of a difficult intubation in a the anaesthetic was gathered from personalpatient with Morning Glory syndrome. communication with the attending anaesthesiologist.

Inhalational induction of general anaesthesia was

uneventful and mask ventilation was easy. Several

intubation attempts were performed by an

experienced anaesthesiologist, who was not able toCorrespondence to: M. Rehman, Department of Anaesthesia and

visualize any laryngeal structures. Several attemptsCritical Care, St. Christopher’s Hospital for Children, Erie Avenue

at Front Street, Philadelphia, PA 19134, USA. at ventilation through laryngeal mask airways of

359 1999 Blackwell Science Ltd

360 Y. SHEVCHENKO ET AL.

different sizes were made, but ventilation was cords were visualized significantly displaced to the

right. An uncuffed unstyletted oral RAE no. 4.5possible only with a LMA size 1. The second

anaesthetic was performed one month prior to this tracheal tube was inserted without difficulty.

Anaesthesia was maintained with sevoflurane,admission at our institution. After inhalational

induction and easy mask ventilation, three attempts nitrous oxide and oxygen. Surgery was performed

without complications. Residual neuromuscularat intubation were made by an experienced

anaesthesiologist. With the help of a Wis-Hipple 1.5 blockade was reversed with neostigmine

0.07 mg·kg−1 and atropine 0.02 mg·kg−1 IV. at the endblade (straight blade with a large flange and slightly

curved flat tip) and significant cricoid pressure, of the case. The patient’s trachea was extubated in

the operating room without difficulty. Her recoveryarytenoid cartilages were visualized on the third

attempt and an oral styletted uncuffed RAE 4.5 was uneventful and she was discharged to home the

same day.tracheal tube was inserted aimed anterior to the

arytenoid cartilages. Interestingly, there was a record

of perceived misplacement of the laryngeal structures Discussionto the right.

On the day of this admission, physical examination Morning Glory syndrome is an uncommon

congenital optic disc anomaly, which was describedrevealed a three-year-old female in no acute distress.

Her weight was 12 kg, pulse 108, BP 68/50 and as early as 1908 by Reis and in 1929 by Handmann.

In 1970 Kindler (14) described ten patients withtemperature 37°C. Head and neck examination was

remarkable for esotropia in the right eye, slight right similar ophthalmological findings and first coined

the term Morning Glory syndrome because theear deformity and features consistent with facial

nerve palsy on the right, evident only with facial ophthalmological appearance of this anomaly closely

resembles that of the morning glory flower. Morningmovement. Morphological appearance of the face

was normal. Airway exam revealed full range of Glory syndrome is characterized by a funnel-shaped,

excavated optic disc surrounded by chorioretinalcervical spine motion, adequate thyromandibular

distance and normal mouth opening. The uvula was pigmentary disturbance. Occasionally seen are

collections of glial tissue overlying the centre of theonly partially visualized due to the lack of patient

cooperation. The patient was premedicated with 6 mg disc, retinal vascular abnormalities, high myopia,

exotropia and retinal detachments. The majority ofof midazolam orally and was separated from her

mother 30 min later without difficulty. cases are unilateral, although several bilateral cases

were reported. Females are affected almost twice asIn the operating room, standard noninvasive

monitors were applied. Fifty per cent oxygen and frequently as males and the right side is involved

more frequently than the left (60%). Generally,nitrous oxide were administered through a face mask,

and a 22 G intravenous catheter was placed on the Morning Glory syndrome is an isolated ocular

abnormality; however, there is evidence in the recentdorsum of the left hand. Atropine 0.02 mg·kg−1

intravenously was administered and inhalational literature of associated systemic abnormalities (1–13).

The most frequently reported systemic associationsinduction of general anaesthesia was performed with

a mixture of sevoflurane, nitrous oxide and oxygen. are basal myelomeningoceles, encephaloceles,

agenesis of the corpus callosum and defects in theAfter the ability to ventilate the patient by mask was

confirmed, neuromuscular blockade was achieved floor of the sella turcica. Midline craniofacial

deformities (hypertelorism, cleft lip and palate) andwith rocuronium 1 mg·kg−1 IV. General anaesthesia

was maintained with sevoflurane and oxygen. Direct dwarfism have been reported. Infrequently, renal

abnormalities and the CHARGE associationlaryngoscopy was performed with the patient’s head

in the sniffing’ position with midline stabilization. (coloboma, heart disease, atresia choanae, retarded

growth and development, genital anomalies, andNo cricoid pressure was applied at that time. Upon

insertion of the Wis-Hipple blade no. 1.5, a long ear anomalies) are encountered (3). An association

between Morning Glory syndrome and Polandfloppy epiglottis was immediately visualized. After

it was elevated up with some difficulty, the arytenoid syndrome (absence of left pectoralis muscle,

hypoplasia of left arm, synbrachydactyly) has beencartilages and the posterior one-third of the vocal

1999 Blackwell Science Ltd, Paediatric Anaesthesia, 9, 359–361

INTUBATION IN MORNING GLORY SYNDROME 361

reported (2). Ten patients with Morning Glory detailed review of all systems. Unexpected difficult

syndrome and cardiac abnormalities have been intubation may be encountered in children with

reported. Cardiac abnormalities in these cases grossly normal morphological appearance.

included ventricular septal defect, atrial septal

defect, patent ductus arteriosus, patent foramenReferencesovale, endocardial cushion defect, total anomalous

pulmonary venous return, tetralogy of Fallot, and 1 DeLaey JJ, Ryckaert S, Leys A. The ‘morning glory’ syndrome.

Ophthal Paediat Genet 1985; 5 (1–2): 117–124.bicuspid aortic valve (13). Of interest to the2 Pisteljic DT, Vranjesevic D, Apostolski S et al. Poland syndromeanaesthesiologist, two of these patients had

associated with ‘morning glory’ syndrome (coloboma of thegastrointestinal abnormalities consisting of abnormal optic disc). J Med Genet 1986; 4): 364–366.

3 Risse JF, Guillaume JB, Boissonnot M et al. An unusualtongue, pharyngeal incoordination or paralysispolymalformation syndrome: CHARGE association’ withrequiring gastrostomy, and two patients hadunilateral morning glory syndrome. Ophtalmologie 1989; 3:

musculoskeletal abnormalities of proximal196–198.

musculature. Six of these patients had facial nerve 4 Kobayashi S, Miyazaki M, Miyagi O et al. A case of

transsphenoidal meningoencephalocele. No Shinkei Geka 1990;palsy on the side of an ear malformation.11: 1065–1070.The presence of slight ear deformity in our patient

5 Wilson DC, Cunningham MJ, Reid MM et al. Meningitis andcould have been a mild variant of Goldenhar midline facial deformity. Acta Paediat 1992; 1: 84–85.

6 Itakura T, Miyamoto K, Uematsu Y et al. Bilateral morningsyndrome. However, Goldenhar syndromeglory syndrome associated with sphenoid encephalocele. Case(hemifacial microsomia, facio-auriculo-vertebralreport. J Neurosurg 1992; 6): 949–951.

spectrum) is usually associated with other 7 Eustis HS, Sanders MR, Zimmerman T. Morning glorydysmorphic features such as hypoplasia of malar, syndrome in children. Association with endocrine and central

nervous system anomalies. Arch Ophthalmol 1992; 2: 204–207.maxillary, and/or mandibular region, microsomia,8 Morioka M, Marubayashi T, Masumitsu T et al. Basal

hypoplasia of facial musculature, abnormalities inencephaloceles with morning glory syndrome, and progressive

function or structure of the tongue and unilateral hormonal and visual disturbances: case report and review of

the literature. Brain Dev 1995; 3: 196–201.vertebral abnormalities. On occasion, cardiac (VSD,9 Merlob P, Horev G, Kremer I et al. Morning glory fundusPDA, TOF), genitourinary (renal agenesis, reflux) or

anomaly, coloboma of the optic nerve, porencephaly andCNS (hydrocephalus, encephaloceles) abnormalities hydronephrosis in a newborn infant. MCPH Entity Clinare encountered (15, 16). Facial nerve palsy has not Dysmorphol 1995; 4: 313–318.

10 Nawratzki I, Schwartzenberg T, Zaubermann H et al. Bilateralbeen described as part of the syndrome.morning glory syndrome with midline brain lesion in an

The need for a thorough physical examination ofautistic child. Metab Pediatr Syst Ophthalmol 1985; 2–3): 35–36.

the patient with primary ophthalmological diagnosis 11 Bron AJ, Burgess SE, Awdry PN et al. Papillo–renal syndrome.

An inherited association of optic disc dysplasia and renalof Morning Glory syndrome is very important.disease. Report and review of the literature. Opthalmic PaediatrAttempts at nasal intubation in a patient withGenet 1989; 3): 185–198.

undiagnosed basal meningomyelocele may 12 Pollock JA, Newton TH, Hoyt WF. Transsphenoidal andpotentially be disastrous. The abnormal position of transethmoidal encephaloceles. Radiology 1968; 90: 442–453.

13 Hittner HM, Hirsch WJ, Kreh GM et al. Colobomatousthe larynx in our case may explain prior inability tomicrophthalmia, heart disease, hearing loss, and mental

ventilate through an LMA of appropriate size for theretardation––a syndrome. J Pediat Ophthalmol 1979; 16: 122–128.

patient’s age. The use of a lighted stylet for blind 14 Kindler P. Morning glory syndrome: unusual congenital optic

disc anomaly. Am J Ophthalmol 1970; 69: 376–384.tracheal intubation may be difficult in a patient with15 Jones KL. In: Smith’s Recognizable Patterns of Humanthe laryngeal inlet displaced off the midline. For

Malformation, Philadelphia, PA: WB Saunders: 5th edn, 1997:the patients with suspected difficult airway, ENT 642.consultation and (indirect) laryngoscopy may be 16 Katz J, Steward DJ. Anesthesia and Uncommon Pediatric Diseases,

2nd edn. Philadelphia, PA: WB Saunders, 1993: 341warranted.

In summary, the anaesthesiologists managing

patients with Morning Glory syndrome need a Accepted 23 June 1998

1999 Blackwell Science Ltd, Paediatric Anaesthesia, 9, 359–361