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Syringobulbia: a Surgical Appraisal 113212ournal of Neurology, Neurosurgery, and Psychiatry 1992;55: 1132-1141 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.12.1132 on 1 December 1992. Downloaded from Syringobulbia: a surgical appraisal David Morgan, Bernard Williams Abstract Material and methods Syringobulbia is a term which has been Fifty four patients were selected because - of clinically applied to brain stem symptoms hindbrain problems. Tumour cases have been or signs in patients with syringomyelia. excluded. All cases had clinical involvement of Syringobulbia clefts are found on inves- the brain stem and had craniovertebral decom- tigation or at necropsy caused by cutting pression (CVD) between 1962-88. Hindbrain outwards of the CSF under pressure from hernia headache has not been used on its own the fourth ventricle into the medulla. as a selection criterion neither has syncope nor These should be differentiated from the nystagmus. There were 34 females and 20 ascending syringobulbia which may occur males. Forty four patients were available for from upward impulsive fluid movements review. Of the remainder, two had died of in a previously established syringomyelia. unrelated causes and 8 were untraceable or Clinical analysis of 54 patients suggests lived an excessive distance away. The age at that bulbar features are most often found diagnosis for females was 10-58, a mean of with neither of the above mechanisms but 37-3, and males 18-61, a mean of 44. The are due to the effects of pressure differ- symptoms often started many years before ences acting downward upon the hind- diagnosis. The mean follow up period from the brain with consequent distortion of the original operation was 14-2 years, range 3-39. cerebellum and brainstem, traction on Thirty four patients had either radiological or cranial nerves or indentation ofthe brain- clinical evidence ofsyringomyelia, 20 cases had stem by vascular loops. The commonest hindbrain features without syringomnyelia. symptoms in the 54 patients were head- Skull radiographs show basilar impression in ache (35), vertigo (27), dysphonia or dys- 26 cases and CT scanning and myelography or arthria (21), trigeminal paraesthesiae MRI showed that 46 had tonsillar descent, 28 (27), dysphagia (24), diplopia (16), tinni- had a demonstrable syrinx in the spine. Two tus (11), palatal palsy (11) and hypo- did not undergo CT scanning, myelography or glossal involvement (11). Careful MRI. attention to hydrocephalus is advisable All patients had CVD. The technique before craniovertebral surgery, but the included a small craniectomy decompressing decompression of the hindbrain and the the tonsils usually by removing the arachnoid correction of dis- and often by sucking away part of the tissue of craniospinal pressure http://jnnp.bmj.com/ sociation remains the mainstay ofsurgical the tonsils, and always stitching the dura back treatment. The results of careful surgery and leaving it widely open. Five patients had are good, 45 of the 54 cases reported ventriculo-atrial shunting (VEA) following improvement. Most of the reported dete- postoperative deterioration, one of these had a rioration occurred in a few patients who syringopleural shunt at the time of the CVD did conspicuously badly. and two had late syringopleural shunting. At review each patient completed a ques- (3 Neurol Neurosurg Psychiatry 1992;55:1132-1141) tionnaire and had a neurological and otolar- on September 25, 2021 by guest. Protected copyright. yngological (ENT) examination. It is not uncommon for patients with syr- CLINICAL FEATURES ingomyelia or hindbrain herniation, to have Occipital headaches dysfunction of the lower cranial nerve and Occipital headache was the commonest pre- Department of Otorhinolaryngology, brainstem. Such symptoms are often ascribed senting symptom and occurred in 35 patients. East Birmingham to "syringobulbia". Syringobulbia, however, is In seven patients the headache persisted post- Hospital, an uncommon lesion of the central nervous operatively, but was less. The headache was Birmingham, UK system; it may be defined as a pathological most characteristically a pounding pain usually D Morgan and Midland Centre for cavitation in the brain stem. There are several posterior bilateral, sometimes ascending to Neurosurgery and possibilities for the pathology and little agree- the vertex or present mostly unilaterally or in Neurology, Holly Lane, ment about what syringobulbia is. the neck. Typically this beat in time with the Smethwick, West We pulse, coming on two or three seconds after a Midlands B67 7JX, UK have reviewed patients with bulbar B Williams symptoms encountered in a neurosurgical strain, rising to a crescendo after a few more Correspondence to: practice expressing an interest in hindbrain seconds and then dying away within half a Mr Bernard Williams herniation. The patients with adequate follow minute. This is the "hindbrain hernia head- Received 2 January 1991 up have been selected from a database of ache" stressed byWilliams.`3 It may be tested and in final revised form 13 January 1992 patients with syringomyelia and related dis- for in the clinic by asking the patient to blow Accepted 17 January 1992 orders. into a mouthpiece attached to a sphygmoman- Syringobulbia: a surgical appraisal 1133 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.12.1132 on 1 December 1992. Downloaded from ometer to a pressure of 50 mm Hg or so for five Nystagmus seconds and then suddenly relaxing. Observa- Twenty six patients were noted to have nys- tion of the patient usually makes the diagnosis tagmus pre-operatively. Thirteen had rotatory but the patient may be asked to draw a graph of nystagmus, which improved after CVD in the pain. nine, with marked amelioration of ocillopsia or dizziness in those who had symptoms. In only Vertigo one case did severe rotatory nystagmus resolve Twenty seven subjects complained of inter- postoperatively. Four patients developed nys- mittent vertigo. Ten patients were symptom tagmus postoperatively, three of whom com- free postoperatively, while the vertigo persisted plained of ocillopsia, possibly contributing to in 9. In 2 cases, 1 and 2, persistent vertigo difficulty in walking. Ocillopsia improved after occurred postoperatively which was relieved VEA shunting in two of these and in none did following ventriculo-atrial shunting. it remain prominent. Voice disturbance Ptosis Twenty one patients had voice disturbance, Of 11 patients with ptosis in 5 it appeared to be either dysphonia due to cord palsy or in 5 of due to a partial unilateral Horner's syndrome them, slurring of speech. Ten patients had and in one a bilateral Horner's syndrome. In 2 confirmed vocal cord palsies. 1 of which was a cases unilateral Horner's syndrome persisted bilateral abductor palsy and presented with as did one with unilateral ptosis. Complete stridor. Four unilateral and 1 bilateral recov- recovery occurred in 8 cases. A postoperative ered following operation. Only in 7 cases did unilateral Horner's syndrome developed in one the voice disturbance persist following CVD. which resolved following VEA. However, 2 patients developed a cord palsy immediately postoperatively. Following ven- Diplopia triculo-atrial shunting the cord palsy com- Sixteen patients complained of diplopia appar- pletely recovered in one and in the other the ently due to abducent nerve palsies. 4 persisted symptoms resolved, although the cord postoperatively. In one of these, case 2, the remained immobile. Another patient devel- paralysis was considerably worse after CVD oped a vocal cord palsy some years after and subsequent hydrocephalus but improved operation. after VEA, although it remained a subjective problem. In 1 patient Myodil (Pantopaque) Paraesthesiae which had been used in the myelogram could Of 27 patients with disordered trigeminal be seen in the cavity of the syrinx in the sensation, 1 of which was bilateral, 18 showed immediate the post-myelogram period. Later a complete resolution. In 8 the numbness radiographs showed that some of the contrast persisted at least partially and in only 1 ofthese material was in the pons. This indicated that was there deterioration in symptoms. Only 1 fluid had ascended from the cervical syrinx and case had trigeminal pain associated with para- the sixth nerve palsy may have been due to esthesiae, this was the presenting symptom and nuclear involvement. was severe. This woman was completely cured by CVD, at which a loop of artery was found Facial Nerve firmly pressed into the side of the medulla and Five patients had a pre-operative lower motor http://jnnp.bmj.com/ was lifted out. neuron (LMN) palsy of the facial muscles and 1 patient an upper motor neuron palsy. One of Dysphagia the patients with LMN features had a history Twenty four patients had pre-operative dys- of 6 episodes of facial palsy before the diag- phagia, some had palatal regurgitation. In 18 nosis was made. All but 1 LMN palsy recov- cases the dysphagia resolved postoperatively. ered postoperatively. One patient had an acute In most of the others there was some improve- herpes zoster of the left facial nerve a few days ment but in one there was no change. Four after discharge from hospital with temporary on September 25, 2021 by guest. Protected copyright. patients developed postoperative dysphagia, 1 complete paralysis. recovered following ventriculo-atrial shunting. One patient had cricopharyngeal myotomy Palatal palsy with improvement. Eleven patients had a unilateral and 2 a bilateral palatal palsy associated with an absent Tinnitus gag reflex and analgesia of the palate. Six Eleven patients complained of tinnitus, 5 completely recovered including one with bilat- bilateral and 6 unilateral. Two patients with eral palsy and in the other bilateral case there unilateral tinnitus became worse postoper- was improvement in one side only, including atively. Out of the patients with bilateral restoration of the gag reflex. tinnitus, 4 showed complete resolution while One patient developed persistent palatal one persisted. Only one patient complained of analgesia and an absent gag reflex immediately persistent postoperative tinnitus, others said postoperatively, which resolved after VEA.
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