Arachnoid cyst resulting in sixth nerve palsy in a child

A 2 year old presented with incomitant esotropia and abduction deficit consistent with sixth nerve palsy. Neuroimaging revealed an arachnoid cyst on the left. Neurosurgical shunting followed by strabismus surgery relieved the abduction deficit and esotropia. An arachnoid cyst may be a rare cause of acquired sixth nerve palsy and strabismus in children.     

Residual incomitant DVD following large bilateral superior rectus recession

A recent survey of 25 experienced strabismus surgeons indicated that a very large superior rectus recession is the procedure of choice for DVD. We report a series of seven patients who exhibited a persistent DVD following large superior rectus recession. Furthermore, the DVD was markedly incomitant, much greater in adduction than in abduction. This residual DVD would mimic inferior oblique overaction on version testing, although proving to be purely DVD on critical observation. This "overaction" is apparently due to the marked weakening of the superior rectus and was not always predictive of the location and amount of persistent DVD. This previously unreported complication of large superior rectus recession is important to recognize so that alternative surgical approaches may be contemplated. It is hoped that more accurate observations and measurements both preoperatively and postoperatively will lead to a more orderly approach to DVD surgery.     

Improvement of horizontal excursion and abduction by vertical muscle transposition in patients with Duane's retraction syndrome type I

PURPOSE: To measure the change in horizontal excursion and improvement of abduction in Duane's retraction syndrome type I after transposition of both vertical rectus muscles and recession of the medial rectus muscle in the affected eye. PATIENTS AND METHODS: This was a retrospective study of patients undergoing surgery for Duane's retraction syndrome type I. Thirty-six patients were treated by transposition of both vertical rectus muscles in combination with medial rectus recession of the affected eye. Head posture, binocular vision, abduction and adduction of the affected eye, and angle of strabismus were measured before and after surgery. RESULTS: After surgery, abduction improved by 15.9 degrees +/- 8.1 degrees (mean +/- standard deviation) and adduction decreased by 5.9 degrees +/- 7.2 degrees. Horizontal excursion improved from 43.1 degrees +/- 8.8 degrees to 53.1 degrees +/- 11.8 degrees. One patient had signs of anterior segment ischemia (ie, enlarged, fixed oval pupil and cells in the anterior chamber), which disappeared after local steroid eye drops were administered. CONCLUSIONS: Surgery enlarges the range of horizontal excursion of the affected eye and causes only a limited decrease in adduction. One patient developed transient anterior segment ischemia. Vertical muscle transposition combined with medial rectus recession is an effective procedure to improve horizontal excursion and abduction in patients with Duane's retraction syndrome type I.     

M?bius and M?bius-like syndromes (TTV-OFM, OMLH)

There exists a spectrum of syndromes characterized by cranial nerve palsies, limb anomalies, and craniofacial malformations. Criteria based on type and severity of limb anomaly or presence or absence of cranial nerve palsy may alter the syndrome nomenclature due to a selection bias but do not appear to benefit syndrome delineation to any extent. Patients with seventh nerve palsy and abduction weakness are usually diagnosed as having M?bius syndrome by ophthalmologists. The observed ocular motility findings range from primarily abduction deficiencies to patients who have a Duane or gaze palsy pattern of horizontal movements. Some cases previously described as "sixth nerve and partial third" may better fall into one of the latter groups, since an isolated adduction deficit as a manifestation of third nerve involvement is rare. The presence of clinical appearance of Duane syndrome in these M?bius-type patients raises the possibility of abnormal innervation of the lateral rectus as an explanation of some patterns of motility observed.     

Occult Duane syndrome: co-contraction revealed following strabismus surgery.

Three patients presented in infancy with large angle esotropia and limited abduction. Narrowing of the lid fissures with globe retraction on horizontal gaze became apparent only after recession of the medial recti. Abduction remained limited. Two of the three exhibited dissociated vertical deviations, and seemed to have both congenital esotropia and Duane syndrome. The third had an unusual pattern of apparent co-contraction in which the eye attempting abduction actually adducted. Although rare, Duane syndrome may occur in children with large angle esotropia, and its presence may not be revealed until after strabismus surgery.     

Synergistic divergence: saccadic velocity analysis and surgical results.

The clinical findings are described in four patients with synergistic divergence (SD), an ocular motility disorder which is considered to be a variant of Duane retraction syndrome (DRS). Saccadic velocity studies in three patients demonstrated preserved abducting saccades in the affected eye with equally reduced amplitudes on both attempted abduction and adduction. The preserved saccades on attempted abduction of the affected eye, coupled with electromyographic evidence that shows maintenance of normal reciprocal innervation with respect to gaze on attempted abduction as dictated by Sherrington's law, strongly suggest that the abducens nerve is not necessarily absent or severely hypoplastic in this condition. One patient showed typical DRS in the contralateral eye and had a son with bilateral DRS, attesting to a possible genetic and etiologic relationship between SD and DRS. Three patients underwent strabismus surgery. Review of our surgical results and an analysis of the literature indicate that a crippling procedure to the ipsilateral lateral rectus muscle, such as extirpation, may be necessary to eliminate simultaneous abduction. Overcorrection did not occur following lateral rectus extirpation.     

Combined eyelid and strabismus surgery: examining conventional surgical wisdom

PURPOSE: To determine whether satisfactory results of both eyelid surgery and strabismus surgery can be obtained when these procedures are performed in the same setting in selected patients. METHODS: Nine patients (16 eyelids) who had undergone surgery in the same setting for eyelid malposition and strabismus of the horizontal rectus muscles, with or without concurrent vertical rectus muscle surgery, were retrospectively reviewed. Eyelid malposition surgery success was defined as a postoperative margin reflex distance (MRD1) within 1 mm of the target. Strabismus surgery success was defined as a postoperative deviation within 8 prism diopters of orthophoria or a decrease in binocular diplopia if the patient had a less than 8 prism diopters deviation in primary gaze preoperatively. RESULTS: Eyelid retraction repair was performed on 13 upper eyelids, levator advancement was performed on 2 eyelids, and frontalis suspension was performed on 1 eyelid. Four patients underwent bilateral medial rectus recession and the remaining 5 patients received varying strabismus surgery. Four patients received adjustable sutures. Mean follow-up was 11 months. Postoperatively, 13 of 16 eyelids (81%) achieved an MRD1 within 1 mm of the target. Eight of 9 patients (89%) demonstrated satisfactory strabismus correction. CONCLUSION: Combined eyelid malposition and strabismus surgery can be successfully performed in selected cases, particularly when the strabismus surgery involves the horizontal rectus muscles. Combined surgery is not recommended in cases that do not involve the horizontal rectus muscles.     

Long-term results: botulinum for sixth nerve palsy

Eight patients with intracranial malignancies or vascular lesions and sixth nerve palsies were treated with botulinum toxin chemodenervation of the antagonist medial rectus muscle. Primary deviation ranged from 20 to 75 prism diopters (pd) of esotropia. Six were treated acutely (within 3 months of onset) and two, which demonstrated partial recovery of lateral rectus function but with residual esotropia and diplopia, were treated after 6 months. After a mean follow-up of 20.6 months, seven were diplopia-free with excellent rotations. Five had complete resolution of the esotropia and diplopia, with near complete recovery of abduction. One had 6 pd residual esotropia, while another, whose sixth nerve had been resected, required a modified Jensen procedure, resulting in full rotations. The single case of bilateral sixth nerve palsy had a functional improvement but was lost to follow-up. One patient had a vertical strabismus induced with the injection and had a gradual return of the esotropia.     

Unilateral anterior transposition and resection of the inferior oblique muscle for the treatment of hypertropia

BACKGROUND: Anterior transposition of the inferior oblique muscle generally is restricted to bilateral cases because of reports of postoperative ipsilateral hypotropia or significant limitation of elevation when performed unilaterally. We performed unilateral anterior transposition of the inferior oblique muscle in patients with vertical and horizontal strabismus who were at risk of anterior segment ischemia. PATIENTS AND METHODS: Six patients underwent unilateral anterior transposition of the inferior oblique muscle in combination with a resection of the inferior oblique muscle. Two patients had lost an inferior rectus muscle in a previous procedure, and four patients had coexistent horizontal and vertical strabismus of various etiologies as well as poor unilateral vision. RESULTS: All six patients achieved vertical alignment within 10 prism diopters. CONCLUSIONS: Unilateral anterior transposition of the inferior oblique muscle appears to be a useful procedure in selected patients with vertical strabismus.     

Facial nerve surgery in children

We report results of surgery of the facial nerve in nine cases of CSOM with cholesteatoma in children. Of these cases, seven had facial palsy following mastoid surgery and they needed re-exploration surgery with facial nerve decompression or nerve grafting depending on the extent and degree of trauma to the nerve. The other two cases had facial palsy due to cholesteatoma itself and they were subjected to facial nerve surgery along with mastoid surgery. Results in these nine cases were very encouraging.     

Strabismus in patients over the age of 60 years

We studied all cases of strabismus surgery performed at the Manhattan Eye, Ear and Throat Hospital between January 1, 1981 and June 30, 1986. Of the procedures, 106 (5.4%) were performed on patients over the age of 60 years. Strabismus in patients over age 60 represents a category of diseases which are different from those of childhood. Horizontal deviations were seen in 74% of the patients equally divided between esotropia and exotropia. Vertical strabismus was seen in 17%, and a combined vertical horizontal strabismus was seen in 9% of patients. In children, the overwhelming majority of ocular deviations are horizontal, with esotropias outnumbering exotropias. The strabismus was of adult onset in 71% of cases and of childhood onset in 29% of the patients. The etiology in the adult onset strabismus group included neuroparalytic, restrictive, sensory, and post-cataract surgery strabismus, as well as decompensated exophoria/intermittent exotropia; this was in contrast to children where the most common etiology of strabismus is innervational disturbances in fusional vergences. The major indications for surgery were diplopia and aesthenopia in 71% of cases. Diplopia is uncommon in childhood. Corrective surgery for the relief of diplopia may be enhanced with the use of adjustable sutures and postoperative prism therapy. Precise realignment is the goal of strabismus surgery in all age groups, however, the role of adjustable sutures and prisms is more important in the treatment of the functional complaints of older patients. Exploration and lysis of adhesions and scar tissue are major components of surgery on patients with restrictive strabismus.(ABSTRACT TRUNCATED AT 250 WORDS)     

Augmented Hummelsheim procedure for paralytic strabismus

PURPOSE: To report a modification of the Hummelsheim procedure for use in the management of paralytic strabismus. METHODS: Eight patients with paralytic strabismus secondary to third nerve palsy (n=1), sixth nerve palsy (n=3), combined cranial nerve palsy (n=1), or extraocular muscle damage (n=3) were treated using a modification of the Hummelsheim transposition procedure. The procedure involves half-tendon transpositions of the adjacent rectus muscles to the insertion of the paralyzed muscle, coupled with resection of the transposed halves. Further augmentation was achieved by surgical or pharmacologic weakening of the ipsilateral (n=6) or contralateral (n=1) antagonist. One patient underwent the procedure bilaterally. All patients underwent at least 6 weeks of follow-up. RESULTS: The mean preoperative primary position deviation in the seven unilateral cases was 54 prism diopters (delta) (range: 25-85 delta). Preoperative forced ductions were positive in four cases. Resections varied from 4-8 mm. Ipsilateral antagonist recession varied from 0-14 mm. The mean change was 52 delta (range: 25-85 delta). Five cases were aligned within 15 delta of orthotropia at 6 weeks. No cases of anterior segment ischemia or induced vertical deviation were noted. CONCLUSION: The modified Hummelsheim procedure appears capable of correcting large angles of strabismus associated with muscle palsy of various etiologies. It is safe, amenable to adjustable sutures, and relatively tissue- and vessel-sparing. Additional study is required to understand more fully the procedure's component effects and its interaction with ocular rotation.     

Restrictive strabismus in Parry-Romberg syndrome

Forced duction and generation testing of a patient with Parry-Romberg syndrome (progressive hemifacial atrophy) confirmed that his incomitant hypotropia and esotropia were restrictive and not due to nerve paresis. This suggests that an orbital inflammatory process (causing extraocular muscle fibrosis) is part of this rare and poorly understood syndrome.     

Recurrent facial nerve palsy in paediatric patients

The aim of the study was to investigate the clinical presentation and prognosis of recurrent facial nerve palsy (RFNP) in children. The files of 182 patients referred to the Schneider Children's Medical Centre of Israel for neurological evaluation of isolated peripheral facial nerve palsy between October 1992 and December 1998 were reviewed. RFNP was found in 11 patients (9 females, 2 males), with an incidence of 6%. In two males, the aetiology was traced to Melkersson-Rosenthal syndrome and these patients were separated from the rest of the group. Three children had two episodes of facial nerve paresis which completely resolved clinically within several weeks. Six other children underwent electrophysiological studies. Two of the latter with residual neurological damage, and one child with abnormal blink reflex only, showed decreased facial nerve conduction velocity and abnormal blink reflex. Three children with complete recovery had disturbed blink reflex only with normal nerve conduction. Brain imaging studies as well as laboratory work-up were non-contributory in all cases. CONCLUSION: The frequency of recurrent facial nerve palsy in children was similar to that in adults. The most significant factors in the evaluation of recurrent facial nerve palsy are medical history and physical findings at diagnosis and after short follow-up. In our patients, electrophysiological studies did not have either clinical or prognostic significance. The rate of full clinical recovery is about 70%, lower than in Bell palsy.     

Facial nerve palsy associated with Rickettsia conorii infection.

Facial nerve palsy has been occasionally attributed to infectious agents, but Rickettsiae species have not been documented as causative agents. We report two adolescent girls with facial nerve palsy and serological evidence of R conorii infection. These cases indicate that rickettsioses should be included among the causes of facial nerve palsy, particularly in endemic areas.     

Sensory strabismus--eso or exo?

PURPOSE: The type of horizontal strabismus from loss or impairment of vision is thought to depend on patient age at the time of vision loss. Association between the age at onset of vision loss and development of esotropia vs exotropia will be determined. METHODS: Patients with a diagnosis of sensory strabismus and visual acuity of 20/40 or poorer were reviewed as well as patients with diagnoses consistent with the development of sensory strabismus. Parameters considered were age at onset of vision loss and type of strabismus. Patients were excluded if the age at onset was not clear. RESULTS: Of 123 patients with sensory strabismus reviewed: 82 (67%) had unilateral vision loss; 41 (33%) had bilateral vision loss; 75 (61%) had congenital vision loss; 50 (67%) developed esotropia; 25 (33%) developed exotropia; 48 (39%) had acquired vision loss; 5 (10%) developed esotropia; and 43 (90%) developed exotropia. A significant difference was noted between age at onset and type of horizontal strabismus (X2= 37.44; P <.0001). CONCLUSION: Of patients with congenital vision loss, 67% developed sensory esotropia and 33% developed sensory exotropia. Of those with acquired vision loss, 10% developed sensory esotropia and 90% developed sensory exotropia. Patients with congenital vision loss are significantly more likely to develop esotropia, P <.005, and those with acquired vision loss are significantly more likely to develop exotropia, P <.001.     

ɫ��ʧ��֢15���ٴ��о�

目的总结色素失禁症患儿临床特征,特别是皮肤、神经系统、眼部、基因改变的特点,提高对该病的认识。方法对1994~2004年首都儿科研究所附属儿童医院收治的色素失禁症患儿15例进行临床多方面检查分析,并进行追踪随访,其中7例患儿接受基因检测。结果15例患儿均有皮肤受累;神经系统受累10例:10例中发生癫5例,其中合并脑积水2例,均死亡,表现有智力发育落后6例;眼部受累6例:视神经萎缩2例,先天白内障1例,视网膜发育不良1例,斜视2例;接受基因检测的7例中5例的NEMO基因的共有序列NEMOΔ4~10缺失。结论色素失禁症患儿皮肤改变显著,眼部及神经系统病变严重,应得到早期诊断治疗。     

Lyme borreliosis--the most frequent cause of acute peripheral facial paralysis in childhood

A prospective hospital-based multicentre study in Lower Saxony allowed to investigate the frequency of acute peripheral facial palsy due to Lyme borreliosis and its clinical and laboratory characteristics. Diagnosis of Lyme Borreliosis was based on detection of IgM antibodies against Borrelia burgdorferi in serum and CSF as well, using an IgM capture ELISA. Between June 1986 and October 1987 27 consecutive cases with acute peripheral facial palsy were studied. Lyme borreliosis is the main cause of peripheral facial palsy in childhood. It was verified serologically in two thirds of the cases. All cases with a positive history for a tick bite and/or an erythema migrans in the head-neck region showed ipsilateral neurological affection suggesting a direct invasion via the affected nerve by Borrelia burgdorferi. Peripheral facial palsy due to Lyme borreliosis represents a monosymptomatic meningoradiculitis. All children with Lyme borreliosis revealed a lymphocytic CSF pleocytosis, whereas in cases of unknown etiology CSF findings usually were normal. Therefore, in any case of facial palsy with an inflammatory CSF syndrome Lyme borreliosis has to be suspected unless proven otherwise.     

Poland-M?bius syndrome. Apropos of 2 cases

The authors report 2 sporadic cases of the Poland-M?bius syndrome in neonates. Both cases presented with unilateral facial palsy, bilateral loss of abduction of the eye, palatal palsy and tongue hypoplasia. The Poland anomaly was total in the first case (absence of pectoralis major, hypoplasia of the right superior limb, syndactyly and brachydactyly) and only partial in the second (absence of pectoralis major).