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ARTICLE
Year : 1969  |  Volume : 17  |  Issue : 1  |  Page : 24-26

Luckenschadel (lacunar skull) and its effects on eyeballs


Ophthalmic Department of the Nil Ratan Sarcar Medical College, Cacutta-14, India

Date of Web Publication4-Jan-2008

Correspondence Address:
B M Chatterjee
Ophthalmic Department of the Nil Ratan Sarcar Medical College, Cacutta-14
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Chatterjee B M, Ghosh P K. Luckenschadel (lacunar skull) and its effects on eyeballs. Indian J Ophthalmol 1969;17:24-6

How to cite this URL:
Chatterjee B M, Ghosh P K. Luckenschadel (lacunar skull) and its effects on eyeballs. Indian J Ophthalmol [serial online] 1969 [cited 2020 Nov 28];17:24-6. Available from: https://www.ijo.in/text.asp?1969/17/1/24/37576

Luckenschadel, which literally means a break in the skull, is an extremely rare congenital deformity of the skull, usually detected by skiagram, which shows an extraordinary appearance of the cranium. WEST[6] in 1875, first described this condition, but not under this present name. It was ENGSTLER[2] who first in 1905, attributed the name Luckenschadel to this deformity which is also known as Lacunar skull.

The cranial deformity consists of a network of bony ridges delimiting bony defects. The areas from where bone appears to be missing may consist of a thin layer of bone only or there may not be any bone at all, the cranial wall consisting of a thick membrane (SHANKS AND KERLEY[5]) The changes are most marked in the parietal and frontal areas but the occipital bone may also be involved. Premature fusion of sutures in this condition may be associated with microcephaly, meningocoele and occasionally encephalocele and spina bifida (B RAILS FORD[1]). Associated with the deformity of the skull, there is malformation of the orbits also with the consequent effect on eyeballs. The majority with this defect have a short life and usually succumb in infancy.

Following is the report of a similar case which may be of ophthalmic interest.


  Case Report Top


A male baby, only 9 days old, was referred to the ophthalmic department of the N.R.S. Medical College on 14-1-68 for his obvious eye conditions. This child being the second issue of the mother was born in normal course on 5-1-68, being 5 lbs. and 13 ounces in weight. There was neither any history of consanguinity nor any serious illness of the mother during pregnancy. The first child of the mother - a boy 3 years old, was healthy and alive.


  Examination Top


The photograph of the baby as shown in [Figure - 1], proves how ghastly the appearance of the baby was. In addition to marked malnourishment, there were any amount of malformations throughout the body. The head was small in size with a globular swelling occupying the centre of the vertex of the skull. On palpation, the outer wall of the cranium appeared to have soft and cystic areas in between moderately hard bony ridges: The central swelling was tensely cystic to the feel, with pulsations.

The two orbits were very shallow and malformed, so that the contents of the orbit including the eyeballs which were of normal size, were virtually extra orbital. The two eyeballs were exposed much beyond the equator with marked limitation of movements. The only movement which could be immediately detected was gentle forward and backward movements of the eyeballs with expiration and inspiration. Moreover the eyeballs pulsated slightly particularly during crying. The cornea in each eye was completely ulcerated with sloughing of the cornea in the left eye, followed by exposure of the uveal tissue. Marked congestion and chemosis of the bulbar- conjunctiva on each side were other conspicuous features. The eyelids were rudimentary and rested behind the equator of the eyeballs with no attempt to cover the eyes. In addition, a deep furrow in the skin of the left cheek extending from the inner side of the inferior orbital margin, downwards and outwards towards the left ear, was also visible.

Other deformities -

There was syndacty in both the hands affecting the index, middle and ring fingers. Talipes equinovarus type of deformity was visible in the right foot and talipes equinovalgus in the left foot. The spinal column was normal.

Skiagram of the skull and orbit -

The X'ray pictures of the skull and orbit as shown in [Figure - 2] and [Figure - 3], showed multiple bone defects in the cranial wall with irregular ridges. The central globular swelling might be a meningocoele. In the lateral view of the skull [Figure - 3], a vertical line drawn through the superior orbital margin, passed behind the eye-ball, showing marked shallowness and malformation of the orbit.

Appendix - The child expired within a week from the first date of examination.


  Discussion Top


In addition to microcephaly, there were defects in the cranial wall in the form of areas of thinning separated by ridges. These appearances did not resemble the typical "silver-beaten" appearance of an oxycephalic skull, because the areas of thinning could not possibly conform to the positions of convolution markings of cerebral hemispheres. So undoubtedly the skiagram proved the case to be a Lacunar skull. In all probability the central globular swelling was a meningocoele.

The most conspicuous feature of the deformity of the skull was the extreme shallowness of the orbits, so as to make the eyeballs completely extra orbital. The uncovered cornea in each eye was easily ulcerated due to exposure. As stated by IDA MANN (1957), the orbit must be considered as forming a part of the whole skull and its deformities are linked with the deformities of the skull rather than the defects of the eye. Consequently in cases of anophthalmos and microphthalmos although the, eyeballs are extremely defective, orbits attain normal development. In the reported case, inspite of the normal shape and size of the eyeballs, the orbits failed to develop because of the deformity of the skull. As suggested by PENA and SANCHEZ-SALORIA (1961) the term exorbita' would be more appropriate than exophthalmos to denote the proptosis of the orbital contents in similar cases.

The etiology of this type of deformity is still obscure. As stated by SHANKS AND KERLEY (1950) some observers believe that it is due to a disturbance of the development of the membrane bones of the skull, while others maintain that it is due to increased intracranial pressure during intrauterine life. That it is due to a simple increase of intracranial pressure seems to be unlikely, for it does not occur in hydrocephalus. It may well be due to a combination of primary bone defect and an imbalance of intracranial and amniotic pressure in the uterus.


  Acknowledgement Top


We are grateful to the Professor of Radiology, Nilratan Sircar Medical College for the X'ray diagnosis

 
  References Top

1.
BRAILSFORD, J. F.: The radiology of bones and joints, 4th Ed., J. and A. Churchill Ltd., London, p. 473, 1948.  Back to cited text no. 1
    
2.
ENGSTER, G.: On Liickensschadel of the new-born and its association with (in German) Spina Bifida, Arch. f. Kinderh, 40: 322, 1904-5.  Back to cited text no. 2
    
3.
MANN, IDA: Developmental anomalies of the Eye, 2nd Ed., B.M.A. Association, London, p. 42, 1957.  Back to cited text no. 3
    
4.
PENA, J. AND SANCHEZ-SALORIO, M.: Ocular problems in craneo-synostosis (in Spanish) Arch. Soc. Oftal. Hispano-Amer, 21: 1851-1869, 1961. Quoted by Jones, Ira S: Annual review -. The orbit, Arch of ophth, 70: 407, 1963.  Back to cited text no. 4
    
5.
SHANKS, S. C. AND KERLEY, P.: H. K. Lewis and Co. London, p. 106, A text-book of X'ray diagnosis, 2nd Ed., 1950.  Back to cited text no. 5
    
6.
WEST, J. F.: Case of meningocele Associated with cervical spina Bifida, cured by Aspiration, Lancet, 2, 552, 1875.  Back to cited text no. 6
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]



 

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Case Report
Discussion
Acknowledgement
Examination
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