J Neurosurg 77:949-951, 1992
Bilateral morning glory syndrome associated with sphenoid encephalocele Case report T o n u ITAKURA, M.D., KAZUKI MIYAMOIO, M.D., YUJI UEMATSU, M.D., SEI31 HAYAStn, M.D., AND NommKO KOMAI, M.D.
Departmenl ofNeurological Surgery. Waka.vama Medical College, Wakayama City', Japan ~" Morning glory, syndrome is a congenital anomaly of the optic disc in which the disc is enlarged and excavated, with white glial tissue in the center. A case is presented of morning glory syndrome associated with sphenoid encephaloeele, median cleft lip, and agenesis of the corpus callosum. A 22-day-old boy was referred to the Wakayama Medical College Hospital for management of dyspnea due to a soft-tissue mass in the oral cavity. Magnetic resonance imaging revealed a mass extending through a bone defect in the sphenoid region and into the oral cavity. Surgical repair was attempted through a biffontal craniotomy. A bone defect was identified in the sphenoid plate, through which the arachnoid membrane was connected to the oral cavity. Both optic nerves were elongated and adhered to the encephalocele. The wall of the meningocele was compressed digitally through the oral cavity and sutured to the dura mater of the bone defect. The operative findings suggest that a basal encephalocele protruding from a bone defect in the sphenoid plate may disturb the normal development of the optic nerve.
KEY WO•DS
morningglory syndrome
ORNING glory syndrome is a congenital anomaly of the optic disc, first described by Kindler in 1970. 4 The disc is usually enlarged and excavated, with an elevated peripheral ring. White tissue in the center of the optic disc obscures retinal blood vessels. This anomaly was termed "morning glory syndrome" since it resembles the flower of that name. 2'47 Encephalocele is an uncommon, but not rare, brain anomaly and usually involves the occipital and parietal regions) However, basal encephaloceles in the spbenoid or ethmoid regions have rarely been reported? 6 In this report, we describe an unusual case of morning glory syndrome associated with sphenoid encephalocele.
M
Case Report This 22-day-old baby boy was referred to the Department of Neurological Surgery at Wakayama Medical College for evaluation of a large head size and dyspnea. He weighed 3180 gm at birth, after an uncomplicated 40 weeks' gestation and normal delivery. Examination. Median cleft lip and palate were detected at birth, and a soft-tissue mass was observed in the oral cavity, causing breathing difficulty. Both optic discs were enlarged and excavated, with white glial
J. Neurosurg. / Volume 77/December, 1992
encephalocele
optic nerve
tissue in the disc center (Fig. 1). A diagnosis of morning glory syndrome was made. The patient did not follow light with his eyes, but his spontaneous eye movement was normal. Skull roentgenograms revealed a bone defect in the sphenoid region. Computerized tomography and magnetic resonance (MR) imaging showed ventricular enlargement, agenesis of the corpus callosum, and a sphenoid meningocele. A mass with low intensity on T,-weighted and high intensity on T2weighted MR imaging extended through the bone defect in the sphenoid region and into the retropharyngeal region and posterior oral cavity (Fig. 2 left). Operation. Surgical repair of the sphenoid encephalocele to treat the dyspnea was attempted when the patient was 50 days of age. A bifrontal craniotomy was performed, and the sphenoid region was explored through a subdural approach. A bone defect was identified in the median part of the sphenoid plate, through which the arachnoid membrane was continuous with the oral cavity, creating a basal encephalocele. Both elongated optic nerves ran anterior to the bone defect after leaving the optic canal and formed the optic chiasm in the normal position. Since it was impossible to explore the entire bone defect and divide the stalk of 949
T. Itakura, et al.
FIG. 2. Left: Preoperative magnetic resonance (MR) T~weighted image demonstrating a mass with low intensity extending into the retropharyngeal region and posterior oral cavity (arrou9 and agenesis of the corpus callosum. Right: Postoperative MR image showing that the meningocele protruding into the oral cavity had decreased in size.
FIG. 1. Ophthalmoscopic view of the left eye showing an enlarged disc surrounded by glial tissue.
the encephalocele, the wall of the encephatocele was compressed digitally through the oral cavity and was sutured to the dura mater of the bone defect. Postoperative Course. The dyspnea improved postoperatively and no cerebrospinal fluid leakage was observed. Postoperative MR imaging showed that the encephalocele protruding into the oral cavity had decreased in size (Fig. 2 right). At follow-up study when the patient was 3 years 9 months of age, he was completely blind. The pupils were equal but neither eye showed reaction to light. Extraocular movement was normal. Discussion
Five cases, including the present case, of morning glory syndrome associated with brain anomalies have been reported (Table l). ~'5"6"~Four patients were male and one was female, with ages ranging from 22 days to 7 years. All five cases were associated with basal encephalocele; two had a cleft lip and agenesis of the corpus callosum. The combination of morning glory syndrome with basal encephalocele suggests a mechanism for the
pathogenesis of morning glory syndrome. The hard palate is derived embryologically from the maxillary process arising from the first branchial arch. The palatine process originating from the maxillary process fuses completely with the nasal septum, derived from the frontal prominence, at about 60 days" gestation. This produces the hard palate. Failure of the two palatine processes and nasal septum to fuse completely at 6 to 9 weeks' gestation results in cleft lip and palate. At this developmental stage, neither the internal layer of the retina (ganglion cell layer) nor the optic nerves are well differentiated. During the 7th week of gestation, the axons of the retinal ganglion cells start to form the optic nerve, which is completely developed at 27 weeks' gestation2 If a sphenoid encephalocele prevents complete fusion of the palates, which precedes the formation of the optic nerve, then development of the optic nerve may be abnormal. In the present case, traction of the optic nerves drew them into the sphenoid bone defect. The fact that this anomaly occurs in combination with ethmoid or sphenoid encephalocele suggests the following. During development, a bone defect secondary to a basal encephalocele develops in the sphenoid or ethmoid bone. The optic nerves attached to the encephalocele become elongated and are drawn into the encephalocele. This results in the abnormal development of the optic nerves and the formation of white glial tissue in the center of the optic nerves.
TABLE 1 Summary offive reported cases of morning glory syndrome associated with brain anomalj., Authors & Year Pollock, et al., 1968 Caprioli & Lesser, 1983 Nawratzki, et al., 1985 Takezawa, el a]., 1987 ltakura, et aL, ]992 950
Patient Characteristics Sex, Age M, 7yrs F, 17 mos M, l0 mos M, 58 mos M, 22 days
Brain Anomaly basal encephalocele sphenoethmoid encephalocele,cleft lip and palate basal encephalocele,agenesisof corpus callosum, autism sphenoid encephalocele,pituitary dwarfism sphenoid encephalocele,cleft lip & palate, agenesis of corpus callosum
AffectedEye fight left both left both
3. Neurosurg. / Volume 77/December, 1992
Morning glory syndrome with encephalocele References 1. Caprioli J, Lesser RL: Basal cncephalocele and morning gloD, syndrome. Br J Ophthalmol 67:349-351, 1983 2. Giuffrb G: Morning glory syndrome: clinical and electrofunctional study of three cases. Br J Ophthalmol 70: 229-236, 1986 3. Ingraham FD, Swan H: Spina bifida and cranium bifidum: a survey of 546 cases. N Engl J Med 228:559-563, 1943 4. Kindler P: Morning glory syndrome: unusual congenital optic disk anomaly. Am J Ophthalmol 69:376-384, 1970 5. Nawratzki 1, Schwartzenberg T, Zaubermann H, et al: Bilateral morning gloD' syndrome with midline brain lesion in an autistic child. Metab Pediatr Syst Ophthalmol 8:35-36, 1985 6. Pollock JA, Newton TH, Hoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and
J. Neurosurg. / Volume 77/December, 1992
roentgen features in 8 cases. Radiology 90:442-453, 1968 7. Steinkuller PG: The morning glory disk anomaly: case report and literature review. J Pediatr Ophthalmol Strabismus 17:8[-87, 1980 8. Takezawa N. Sato M, Yanagisawa T, et al: [Pituitary dwarfism associated with morning glory syndrome and transsphenoidal encephalocele: a case report.] No To ttattatsu 19:492-496, 1987 (Jpn) 9. Tuchmann-Duplessis H, Auroux M, Haegel P: Illustrated Haman Embryology. New York: Springer-Verlag, 1982
Manuscript received December 3, 1991. Accepted in final form June 19, 1992. Address reprint requests to: Toru Itakura, M.D., Department of Neurological Surgery, Wakayama Medical College, 7-27 Wakayama City, Japan.
951
Bilateral morning glory syndrome associated with sphenoid encephalocele Case report T o n u ITAKURA, M.D., KAZUKI MIYAMOIO, M.D., YUJI UEMATSU, M.D., SEI31 HAYAStn, M.D., AND NommKO KOMAI, M.D.
Departmenl ofNeurological Surgery. Waka.vama Medical College, Wakayama City', Japan ~" Morning glory, syndrome is a congenital anomaly of the optic disc in which the disc is enlarged and excavated, with white glial tissue in the center. A case is presented of morning glory syndrome associated with sphenoid encephaloeele, median cleft lip, and agenesis of the corpus callosum. A 22-day-old boy was referred to the Wakayama Medical College Hospital for management of dyspnea due to a soft-tissue mass in the oral cavity. Magnetic resonance imaging revealed a mass extending through a bone defect in the sphenoid region and into the oral cavity. Surgical repair was attempted through a biffontal craniotomy. A bone defect was identified in the sphenoid plate, through which the arachnoid membrane was connected to the oral cavity. Both optic nerves were elongated and adhered to the encephalocele. The wall of the meningocele was compressed digitally through the oral cavity and sutured to the dura mater of the bone defect. The operative findings suggest that a basal encephalocele protruding from a bone defect in the sphenoid plate may disturb the normal development of the optic nerve.
KEY WO•DS
morningglory syndrome
ORNING glory syndrome is a congenital anomaly of the optic disc, first described by Kindler in 1970. 4 The disc is usually enlarged and excavated, with an elevated peripheral ring. White tissue in the center of the optic disc obscures retinal blood vessels. This anomaly was termed "morning glory syndrome" since it resembles the flower of that name. 2'47 Encephalocele is an uncommon, but not rare, brain anomaly and usually involves the occipital and parietal regions) However, basal encephaloceles in the spbenoid or ethmoid regions have rarely been reported? 6 In this report, we describe an unusual case of morning glory syndrome associated with sphenoid encephalocele.
M
Case Report This 22-day-old baby boy was referred to the Department of Neurological Surgery at Wakayama Medical College for evaluation of a large head size and dyspnea. He weighed 3180 gm at birth, after an uncomplicated 40 weeks' gestation and normal delivery. Examination. Median cleft lip and palate were detected at birth, and a soft-tissue mass was observed in the oral cavity, causing breathing difficulty. Both optic discs were enlarged and excavated, with white glial
J. Neurosurg. / Volume 77/December, 1992
encephalocele
optic nerve
tissue in the disc center (Fig. 1). A diagnosis of morning glory syndrome was made. The patient did not follow light with his eyes, but his spontaneous eye movement was normal. Skull roentgenograms revealed a bone defect in the sphenoid region. Computerized tomography and magnetic resonance (MR) imaging showed ventricular enlargement, agenesis of the corpus callosum, and a sphenoid meningocele. A mass with low intensity on T,-weighted and high intensity on T2weighted MR imaging extended through the bone defect in the sphenoid region and into the retropharyngeal region and posterior oral cavity (Fig. 2 left). Operation. Surgical repair of the sphenoid encephalocele to treat the dyspnea was attempted when the patient was 50 days of age. A bifrontal craniotomy was performed, and the sphenoid region was explored through a subdural approach. A bone defect was identified in the median part of the sphenoid plate, through which the arachnoid membrane was continuous with the oral cavity, creating a basal encephalocele. Both elongated optic nerves ran anterior to the bone defect after leaving the optic canal and formed the optic chiasm in the normal position. Since it was impossible to explore the entire bone defect and divide the stalk of 949
T. Itakura, et al.
FIG. 2. Left: Preoperative magnetic resonance (MR) T~weighted image demonstrating a mass with low intensity extending into the retropharyngeal region and posterior oral cavity (arrou9 and agenesis of the corpus callosum. Right: Postoperative MR image showing that the meningocele protruding into the oral cavity had decreased in size.
FIG. 1. Ophthalmoscopic view of the left eye showing an enlarged disc surrounded by glial tissue.
the encephalocele, the wall of the encephatocele was compressed digitally through the oral cavity and was sutured to the dura mater of the bone defect. Postoperative Course. The dyspnea improved postoperatively and no cerebrospinal fluid leakage was observed. Postoperative MR imaging showed that the encephalocele protruding into the oral cavity had decreased in size (Fig. 2 right). At follow-up study when the patient was 3 years 9 months of age, he was completely blind. The pupils were equal but neither eye showed reaction to light. Extraocular movement was normal. Discussion
Five cases, including the present case, of morning glory syndrome associated with brain anomalies have been reported (Table l). ~'5"6"~Four patients were male and one was female, with ages ranging from 22 days to 7 years. All five cases were associated with basal encephalocele; two had a cleft lip and agenesis of the corpus callosum. The combination of morning glory syndrome with basal encephalocele suggests a mechanism for the
pathogenesis of morning glory syndrome. The hard palate is derived embryologically from the maxillary process arising from the first branchial arch. The palatine process originating from the maxillary process fuses completely with the nasal septum, derived from the frontal prominence, at about 60 days" gestation. This produces the hard palate. Failure of the two palatine processes and nasal septum to fuse completely at 6 to 9 weeks' gestation results in cleft lip and palate. At this developmental stage, neither the internal layer of the retina (ganglion cell layer) nor the optic nerves are well differentiated. During the 7th week of gestation, the axons of the retinal ganglion cells start to form the optic nerve, which is completely developed at 27 weeks' gestation2 If a sphenoid encephalocele prevents complete fusion of the palates, which precedes the formation of the optic nerve, then development of the optic nerve may be abnormal. In the present case, traction of the optic nerves drew them into the sphenoid bone defect. The fact that this anomaly occurs in combination with ethmoid or sphenoid encephalocele suggests the following. During development, a bone defect secondary to a basal encephalocele develops in the sphenoid or ethmoid bone. The optic nerves attached to the encephalocele become elongated and are drawn into the encephalocele. This results in the abnormal development of the optic nerves and the formation of white glial tissue in the center of the optic nerves.
TABLE 1 Summary offive reported cases of morning glory syndrome associated with brain anomalj., Authors & Year Pollock, et al., 1968 Caprioli & Lesser, 1983 Nawratzki, et al., 1985 Takezawa, el a]., 1987 ltakura, et aL, ]992 950
Patient Characteristics Sex, Age M, 7yrs F, 17 mos M, l0 mos M, 58 mos M, 22 days
Brain Anomaly basal encephalocele sphenoethmoid encephalocele,cleft lip and palate basal encephalocele,agenesisof corpus callosum, autism sphenoid encephalocele,pituitary dwarfism sphenoid encephalocele,cleft lip & palate, agenesis of corpus callosum
AffectedEye fight left both left both
3. Neurosurg. / Volume 77/December, 1992
Morning glory syndrome with encephalocele References 1. Caprioli J, Lesser RL: Basal cncephalocele and morning gloD, syndrome. Br J Ophthalmol 67:349-351, 1983 2. Giuffrb G: Morning glory syndrome: clinical and electrofunctional study of three cases. Br J Ophthalmol 70: 229-236, 1986 3. Ingraham FD, Swan H: Spina bifida and cranium bifidum: a survey of 546 cases. N Engl J Med 228:559-563, 1943 4. Kindler P: Morning glory syndrome: unusual congenital optic disk anomaly. Am J Ophthalmol 69:376-384, 1970 5. Nawratzki 1, Schwartzenberg T, Zaubermann H, et al: Bilateral morning gloD' syndrome with midline brain lesion in an autistic child. Metab Pediatr Syst Ophthalmol 8:35-36, 1985 6. Pollock JA, Newton TH, Hoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and
J. Neurosurg. / Volume 77/December, 1992
roentgen features in 8 cases. Radiology 90:442-453, 1968 7. Steinkuller PG: The morning glory disk anomaly: case report and literature review. J Pediatr Ophthalmol Strabismus 17:8[-87, 1980 8. Takezawa N. Sato M, Yanagisawa T, et al: [Pituitary dwarfism associated with morning glory syndrome and transsphenoidal encephalocele: a case report.] No To ttattatsu 19:492-496, 1987 (Jpn) 9. Tuchmann-Duplessis H, Auroux M, Haegel P: Illustrated Haman Embryology. New York: Springer-Verlag, 1982
Manuscript received December 3, 1991. Accepted in final form June 19, 1992. Address reprint requests to: Toru Itakura, M.D., Department of Neurological Surgery, Wakayama Medical College, 7-27 Wakayama City, Japan.
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