Anomalous Head Posture Research Articles

Nystagmus in pediatric patients: interventions and patient-focused perspectives.

Nystagmus refers to involuntary, typically conjugate, often rhythmic oscillations of the eyes. The most common cause of nystagmus in children is infantile nystagmus syndrome (INS). INS presents within the first few months of life and is sometimes accompanied by an ocular condition associated with sensory impairment. Because this condition affects a person throughout life, it is important to understand the options available to manage it. This review focuses on the underlying nystagmus etiology, psychosocial and functional effects of nystagmus, as well as current principles of management, including optical, pharmacological, surgical, and rehabilitative options. Currently, the neural mechanisms underlying INS are not fully understood. Treatment options are designed to increase foveation duration or correct anomalous head postures; however, evidence is limited to mainly pre- and post-study designs with few objective comparisons of treatment strategies. Management of INS should be individualized. The decision on which treatment is best suited for a particular patient lies with the patient and his/her physician.

Respond to: Management of exotropic Duane retraction syndrome

Dear Editor, We thank the authors for showing interest in our article Sharma et al.[1] For clinical management purpose, we have followed Jampolsky's classification,[2] which would mostly comprise the Type II of Huber[3] and not Type III, herein considered as just exotropic Duane retraction syndrome (DRS). The esotropic DRS and Ortho DRS have to be tackled differently. One needs to clearly understand that the primary problem in all cases of DRS is paradoxic innervation of the lateral rectus (LR) during the adduction, and this cannot be eliminated by any procedure other than total extirpation or periosteal fixation of LR. In exotropic DRS, it is a good strategy to sacrifice the aberrant LR and convert the DRS into LR palsy and improve the abduction by transposition of the vertical recti. The adduction limitation in DRS is due to the co-contraction of LR and medial rectus (MR) in adduction and improves on periosteal fixation of LR. No surgery on the ipsilateral MR is usually required. The MR is usually normal and should be confirmed by passive ductions, which are of paramount importance and have been mentioned as the first step in operative procedure in the methodology. Adduction improvement was not full in Group B due to the resting tone of partial vertical rectus transpositioning (pVRT) providing some resistance. The improved abduction, however, is minimal and is mainly due to the relaxed MR action during abduction. Following pVRT, the vertical vectors start working in synergy to aid the abduction tone with the inhibition of the tone of MR helps in abduction. The case A6 shown in picture had no improvement in abduction as shown in Table 1. However, it has been highlighted by Jampolsky, one should not be mistaking the degree of exodeviation as good abduction in exotropic DRS. Fig. 2 clearly demonstrates improved abduction. Statistical tests have not been claimed by us due to small numbers in this pilot study. Use of VRT in esotropic DRS with abnormal LR innervation can worsen retraction and upshoots and downshoots in adduction. In exotropic DRS, rather than graded recession of the aberrant LR, pVRT done as an adjustable procedure, along with the periosteal fixation seems to be better alternative and will be published soon. This study clearly establishes the role of periosteal fixation of LR to solve the paradoxic innervation and of the partial VRT to correct the esotropia thus created and also improves abduction as shown by the two groups. This strategy corrects the anomalous head posture, the deviation in the primary position, the retraction, narrowing of palpebral aperture and upshoots or downshoots in adduction, as well as improves the adduction and convergence, a vital function, as also improves the fields of binocular single vision. The improvement in abduction is minimal but a good value addition.

Outcomes following surgery for anomalous head position with coexisting strabismus in children with infantile nystagmus syndrome

The modified Kestenbaum procedure is effective for surgical management of anomalous head position (AHP) associated with infantile nystagmus (INS). Some patients with AHP have coexisting strabismus which affects surgical planning. We report surgical design and outcomes for INS patients having coincident AHP and strabismus.

Visual function and compensatory mechanisms for hemianopia after hemispherectomy in children.

Little is known about the functional visual outcome of children after hemispherectomy. Several case reports have described an anomalous head posture (AHP) and exotropia (XT) contralateral to the side of early brain damage, as possible compensatory mechanisms (CMs) for homonymous hemianopia (HH). The aim of this study was to determine visual outcome and the prevalence of such CMs in hemispherectomized children. Patient files from all children who underwent hemispherectomy and had a postoperative ophthalmologic examination in the University Medical Center (UMC) Utrecht up to October 2012 were retrospectively reviewed. Preoperative and postoperative clinical information on visual fixation, visual acuity, visual fields, optic discs, head posturing, ocular alignment, and cognitive development was collected. Clinical characteristics were compared between children who developed CMs and those who did not. Forty-five children (21 male) underwent a hemispherectomy (22 right) at a median age of 2.1 years. Median ophthalmologic follow-up was 2.3 years. After hemispherectomy, visual fixation was present in all children, and 87% of the examined children had a normal visual acuity or a mild visual impairment. All children who underwent a visual field measurement had an HH. Anomalous head posturing and continuous or intermittent XT contralateral to the side of hemispherectomy were found in 53% and 38% of children, respectively. Children with CMs had more frequently right-sided surgery and earlier onset of epilepsy, and they tended to be younger when they underwent hemispherectomy than children without. Despite HH, the majority of children who undergo hemispherectomy have a good visual outcome. Furthermore, they frequently develop AHP and continuous or intermittent XT contralateral to the hemispherectomy as part of a coping strategy to optimize the functional visual field. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.

Anomalous head posture related to visual problems

The cause of the anomalous head posture (AHP) has been mainly assigned to ocular, orthopedic, and neurologic causes. The AHP can take the form of head tilt, face turn, chin up, chin down, or combined, depending on the specific etiology. However, there are many variations, and the type of the head posture cannot reliably predict the underlying cause. Ocular AHP is usually an attempt to improve visual acuity or binocularity. Since the etiology is not always obvious, we stress that these patients must be carefully evaluated by ophthalmologists. Our effort here is to offer the neurologist a thorough insight in the specific head posture pattern primarily related to visual disorders.

Bilateral Superior Oblique Palsy: Etiology and Therapeutic Options

To study the causes of bilateral superior oblique palsy (BSOP), treatment with botulinum toxin and/or surgery, and outcome of treatment. This was an 11-year retrospective study of patients with BSOP treated with injections of botulinum toxin (Botox), surgery, or both. Treatment was considered successful when anomalous head turn and diplopia in primary gaze position and downgaze resolved. Bilateral superior oblique palsy was diagnosed in 12 patients (8 male; mean age, 29.5 years). Palsy was secondary to a neoplasm in 3 cases and to head trauma in 2. In 2 cases, it was ischemic, in 2 it was congenital; the remaining cases were iatrogenic (hydrocephalus secondary to meningitis, 1), hemorrhagic (1), and idiopathic (1). The clinical manifestations recorded were diplopia (10), anomalous head posture (9), V pattern (12), subjective excyclotorsion (8), and objective excyclotorsion (6). Recovery was spontaneous in 1 case with neoplastic disease. Botox was injected in 8 cases (inferior oblique and/or inferior rectus muscles [successful in 2]), and subsequent surgery was required in 6. Two patients underwent surgery without prior injection of Botox. The most common surgical technique was recession of the inferior oblique muscle (6 patients), either as the only operation or associated with other procedures. The final result was good in 72.72% (8/11). Mean follow-up was 62.6 months (range 9-99 months). Causes of BSOP were varied (most frequently neoplastic). Botox was effective as the only treatment in 25% (2/8). Outcome was good in a high percentage of cases with Botox, surgery, or both.

Evaluation of periosteal fixation of lateral rectus and partial VRT for cases of exotropic Duane retraction syndrome

Purpose:The purpose of this study is to evaluate the lateral rectus periosteal fixation and partial vertical rectus transpositioning (VRT) as treatment modalities to correct exotropic Duane retraction syndrome (Exo-DRS).Materials and Methods:Prospective interventional case study of cases of Exo-DRS with limitation of adduction. A total of 13 patients were subdivided into two groups. Six patients underwent only lateral rectus periosteal fixation (group A) and seven patients also underwent partial VRT (group B). Assessment involved prism bar cover test, abduction and adduction range, extent of binocular single visual field and exophthalmometry. These tests were repeated at 1 week, 1 month and 3 months post-operatively and data analyzed.Results:The pre-operative mean values and ranges were 26.2Δ (22-35) exotropia for group A and −21.3Δ (14-30) exotropia for group B. The post-operative mean and range was +0.6Δ esotropia (+20 to −8) for group A and 8Δ (−2 to −20) exotropia for group B. Mean grade of limitation of abduction changed from −3.8 to −3.6 versus −3.6 to −2.8 and mean grade of limitation of adduction changed from −1.9 to −0.7 versus −1.5 to −0.5 in the groups A and B respectively. Mean binocular single visual field changed from 14.7° to 23.3° in group A and 11.8° to 26.4° in the group B respectively.Conclusion:Lateral rectus periosteal fixation is an effective surgery to correct the exodeviation, anomalous head posture and improving adduction in Exo-DRS and partial VRT in addition is effective in improving abduction and binocular single visual fields.

Clinical Analysis, Management and Outcome of Duane’s Retraction Syndrome at a Tertiary Eye Care Centre in South India

Aim: To study clinical features, management & outcome of Duane’s retraction syndrome (DRS). Materials & Methods: This prospective, interventional, non randomised study of 102 patients diagnosed with DRS was done from September 2009 to August 2011. Various characteristics studied included sex, age at presentation, laterality, manifest primary position horizontal deviation, upshoot and downshoot, amblyopia, and associated ocular and systemic abnormalities. Follow up for non surgical patients was for minimum period of 6 months. Surgery was advised in patients with anomalous head posture of more than 15 degrees, cosmetically unacceptable upshoot or downshoot, significant deviation in primary position and globe retraction. Post operative follow up of surgical cases was done at 1 month and were followed up for a minimum period of 6 months. Results: : Of the total 102 patients, 92 (90.2%) had unilateral and 10 (9.8%) had bilateral DRS. Type I (78.57 %) was the most common while Type II (2.68%) was the least common among unilateral as well as bilateral cases. Preponderance for the female gender was seen in unilateral and bilateral cases. Left eye preponderance was seen in unilateral cases. A significant association was found between type of unilateral DRS and type of ocular deviation in primary gaze, direction of face turn, presence of upshoot/downshoot. Exotropia (41.2%) was the most common ocular deviation in primary position in our study. Exotropia was more common in unilateral cases and esotropia more in bilateral cases.Surgical correction was done in 20 patients (22 eyes). Patients with both esotropia and exotropia who underwent surgical correction showed a significant improvement after surgery (p=0.0368 and 0.0011 respectively). There was a significant improvement of abnormal head posture (AHP) and globe retraction post operatively (p =0.014 and p =0.005 respectively). Conclusion: Unilateral types I, II, and III Duane syndrome and bilateral cases differs in the primary position horizontal deviation, upshoot and downshoot, and associated ocular abnormalities. Surgical intervention is recommended in patients with abnormal head posture (AHP), overshoot, globe retraction and significant deviation in primary position.

Surgical results of patients with unilateral superior oblique palsy presenting with large hypertropias.

Surgical management of superior oblique palsy (SOP) is challenging because of combined vertical, horizontal, and torsional misalignment. The authors report the surgical results of patients with large primary position hypertropias (> 20 prism diopters [PD]) due to unilateral SOP. Criteria for success included correction of the anomalous head posture, primary position alignment between orthotropia and 6 PD of undercorrection, and no reoperation required for residual deviations in any direction of gaze. Forty-five patients met inclusion criteria. Mean preoperative alignment in primary gaze was 26.5 ± 6.5 PD compared to 3.0 ± 4.4 PD postoperatively (P < .001). Twenty-three (51%) cases met the criteria for success with one operation. Of the patients who had single muscle surgery, 14% had a successful outcome, with a mean 67% (17.3 PD) reduction in hypertropia. Of patients who underwent simultaneous multiple muscle surgery, 58% met the criteria for a successful result, with a mean 92% (24.6 PD) reduction in primary gaze hypertropia. Success was the highest in patients who underwent ipsilateral inferior oblique combined with contralateral inferior rectus recessions with (60% success) or without (65% success) a Harada-Ito procedure. Undercorrections are frequent following surgery for unilateral SOP with preoperative deviations greater than 20 PD in primary position, especially after single-muscle surgery. Simultaneous multiple muscle surgery rarely results in overcorrection and is recommended in patients with SOP and more than 20 PD of hypertropia in primary position.

Inferior oblique underaction: a transient complication related to inferior orbital wall fracture in childhood

To evaluate inferior oblique (IO) underaction related to orbital floor fracture and its management. We retrospectively assessed 137 patients with orbital floor fractures who had undergone surgical repair between July 2003 and August 2009. Review of clinical data, which included photographs and radiologic findings, was performed. IO underaction was diagnosed based on anomalous head position and which was confirmed with the Hess test and limitation of duction and version in the nine diagnostic positions of gaze. Twelve patients demonstrated IO underaction pattern (8.8%); 3 patients presented preoperatively and 9 patients presented postoperatively. All the patients showed IO underaction pattern in the Hess test and head tilt position. The median age was 9.5 years (range, 6-24 years), and all the patients were male. Of 12 patients, 10 (85%) presented with nausea and vomiting symptoms, 2 (17%) infraorbital hypoesthesia, and 3 (25%) pupillary dilatation. On the basis of the CT scans, all patients had trap door fractures with soft tissue entrapment. The IO underaction recovered spontaneously within 2 months without any treatment. Head tilt towards the injured side can be a warning sign of IO underaction in orbital floor fracture, especially pre- or postoperatively in the paediatric population. Physicians managing paediatric orbital fracture should be aware of this transient complication.

Anomalous head posturing in essential infantile esotropia: a hypothesis

1This editorial presents our understanding of why a child with infantile esotropia syndrome may manifest a visually directed anomalous head posture (AHP). Many aspects of essential infantile esotropia (EIE) or infantile esotropia syn-drome remain controversial and ill-defined (1). The recent National Eye Institute, National Institutes of Health, USA–sponsored Classification of Eye Movement Abnormalities and Strabismus (CEMAS) workshop and document has at-tempted to consolidate its definition (2). Table I presents the CEMAS description of EIE.Torticollis in childhood is most commonly the result of ab-normalities of the central nervous system, the ocular motor system, or the neck musculature. Head posturing associ-ated with abnormalities of the ocular motor system is usu-ally compensatory. The visual purpose of head posturing serves to enhance bifoveal fixation, singular binocular vi-sion, or visual acuity. The ocular etiologies of anomalous head posture include strabismus, nystagmus, refractive errors, dissociated ocular deviations, and visual pathway obstruction (e.g., lid, corneal, lens, and vitreous anoma-lies). Although clinical differentiation of these disorders is accurately accomplished after a thorough history and ophthalmic examination, the etiology of anomalous head posture is often elusive in patients with a combination of strabismus and nystagmus. Children with nystagmus will have anomalous head posture due to multiple causes. A “gaze null” is a position of one or both eyes with respect to the orbit where the nystagmus intensity is the least and vision is the best and is usually associated with infantile nystagmus syndrome or more rarely, forms of acquired nystagmus with an eccentric gaze null position, e.g., gaze evoked, upbeat, and downbeat nystagmus. An “adduction null” is a position of a preferred eye in adduction charac-

Infrared Oculography as a Non Invasive Methods to Measure Visual Acuity before and after Surgery in Children with Congenital Nystagmus

Background: Congenital nystagmus is an involuntary, bilateral, conjugate oscillation of the eyes which is present at birth or develops within the first 6months of life. A specific surgical intervention may improve visual acuity in these subjects. However, measurement of visual acuity in children is difficult because of invasive procedures and scarce patient's collaboration. Infrared oculography (IROG) is a non invasive method validated to define time of foveation, an indirect measure of visual acuity.Aims: To evaluate the utility of IROG in children with congenital nystagmus treated by surgery. Methods. Prospective study enrolling children with congenital nystagmus and anomalous head position. All subjects underwent surgical intervention according to Anderson. Before and after surgical intervention visual acuity was measured by IROG.Results: We enrolled 18 subjects with congenital nystagmus (Age 12.1 ± 6.6 y, Male 11) and anomalous right (n=9) or left (n=9) head position. After surgery, head position was corrected in 9 out of 18 subjects. Visual acuity measured by IROG improves in all enrolled subjects after surgery (NAFP 0.68 ± 0.07 before vs 0.73±0.04 after surgery). IROG was well tolerated by all enrolled subjects.Conclusions: IROG is an useful non invasive tool to evaluate efficacy of surgery for congenital nystagmus in pediatric patients.

Design and efficacy of surgery for horizontal idiopathic nystagmus with abnormal head posture and strabismus

The design and efficacy of surgery for horizontal idiopathic nystagmus (HIN) with abnormal head posture and strabismus were investigated. Different surgical procedures were selected according to the angle of head turn in 44 cases of HIN with abnormal head posture and strabismus. For patients with a head turn of 15° or less, the Anderson procedure was used; the yoke muscles were recessed upon slow-phase. For patients with a head turn between 15° and 25°, the surgery was designed as a Kestenbaum 5-4-4-5 procedure. For patients with a head turn of 25° or more, the surgery was designed as a Parks 5-8-6-7 procedure. The surgery to correct the abnormal head posture was performed on the fixating eye while that to correct the deviation was then performed on the non-fixating eye at the same time. The amount of surgery of the horizontal rectus muscles on the non-fixating eye was sum of the angle of head turn and the degree of deviation, which was calculated as follows: recession/resection amount of medial and lateral rectis / 2×5 =angle of head turn ± degree of deviation. The results showed as follows: (1) Visual acuity: the visual acuity in the primary ocular position increased two lines or more in 35 patients, accounting for 79.55%. Nine patients had no or only one-line improvement, accounting for 20.45% of the entire study population; (2) The degree of deviation in the primary ocular position: 37 cases had a normal primary ocular position or the degree of deviation ≤ 8(δ) after surgery, accounting for 84.09%. Six patients had a residual degree of deviation of 8(δ)-15(δ), accounting for 13.64%. One patient had a residual degree of deviation >20(δ), accounting for 2.27% of the patients examined; (3) Abnormal head posture: 34 patients had a normal head posture or a head turn of less than 5°, accounting for 72.27%. Eight patients had a residual head turn of 5°-15°, accounting for 18.18%. Two patients had a head turn of 15°-25°, accounting for 4.55%. It was concluded that different surgical procedures based on the angle of head turn and the relationship between deviation and null zone can eliminate anomalous head posture, correct deviation, and improve vision acuity in the primary ocular position.

Clinical and Oculomotor Characteristics of Albinism Compared toFRMD7Associated Infantile Nystagmus

Previous studies have found no difference between nystagmus characteristics associated with idiopathic infantile nystagmus (IIN) and that associated with albinism. The present aim is to compare the oculomotor characteristics and other associated clinical features of albinism and a genetically homogenous group of IIN volunteers where the nystagmus is associated with FRMD7 mutations. Oculomotor characteristics and related clinical features between albinism (n = 52) and idiopathic nystagmus associated with FRMD7 mutations (FRMD7-IIN, n = 83) were compared. The nystagmus characteristics compared included amplitude, frequency, intensity of nystagmus, foveation characteristics, and waveform type. Other clinical features compared were strabismus, stereopsis and anomalous head posture. The FRMD7-IIN group contained a higher proportion of pendular waveform types compared with the albinism group (P < 0.0001). Nystagmus frequency was significantly lower in albinos (mean = 3.3 Hz, SD = 0.13 Hz) compared with the FRMD7-IIN group (mean = 4.3 Hz, SD = 0.18 Hz) (F = 14.5, P < 0.0001). Strabismus and anomalous head posture was seen in higher proportions in the albinism group, and stereopsis was worse compared with the FRMD7-IIN group (P ≪ 0.0001). Differences in nystagmus characteristics associated with albinism and those associated with FRMD7 mutations leading to IIN are described for the first-time. These findings may provide useful information in the future elucidation of mechanisms underlying the nystagmus associated with albinism and idiopathic infantile nystagmus.

Application of a Digital Head-Posture Measuring System in Children

To report the repeatability of a digital head-posture measuring system when used to record anomalous head postures in children. Prospective study and clinical laboratory investigation. Using a digital head-posture measuring system, we measured 36 different anomalous head postures in 27 children with infantile nystagmus syndrome. Repeatability values and 95% limits of repeatability of measurements were generated for anomalous head postures. Among the 27 children, 3 had 2 head postures (right and left head turns) in 2 different directions; 6 had 2-dimensional head postures that were considered 2 different head postures; and 18 had a 1-dimensional head posture. There were 5 chin-up or chin-down postures, 23 head-turn postures, and 8 head-tilt postures in a total of 36 anomalous head postures. The repeatability value for all anomalous head postures was less than 10 degrees. Ninety-five percent limits of repeatability yielded ranges of less than 10 degrees for all anomalous head postures. The digital head-posture measuring system is a valid and reliable device for measuring 3-dimensional head postures in children with nystagmus.

Acquired Monocular Nystagmus as the Initial Presenting Sign of a Chiasmal Glioma

A 15-month-old male presented with a one-day history of acute onset, continuous oscillating movement of his right eye. He had received his one-year immunizations four days prior and had a four-day history of a febrile viral respiratory tract infection. Pregnancy was unremarkable. He had severe iron deficiency anemia (MCV 66, Hb 65) and was developmentally delayed, as he was unable to stand independently and was non-verbal. His head circumference was 49 cm (95th percentile) and his weight was at the 25th percentile. On physical examination, continuous horizontal large amplitude pendular nystagmus of the right eye at a frequency of 3-4 Hz was observed. No nystagmus was observed in the left eye, even on funduscopic examination. The child could fixate targets in all four quadrants with both eyes independently, and could fixate and track small objects with both eyes independently suggesting no significant visual field defect or visual loss. Dilated funduscopic examination was normal, extra ocular movements were full, pupils were equal and reactive and there was no relative afferent pupillary defect. The remainder of the neurological examination was normal. There was no head bobbing or anomalous head position and no stigmata of neurofibromatosis type 1. Magnetic resonance imaging of the brain (Figure) demonstrated a 2 cm x 1.6 cm x 1.2 cm suprasellar enhancing mass involving the optic chiasm, hypothalamus, mamillary bodies and superior pituitary stalk. There was no extension into the pituitary fossa or the optic nerves and no ventricular enlargement. A biopsy of the mass was obtained and revealed histology consistent with a low-grade pilocystic astrocytoma (World Health Organization (WHO) grade 1), consistent with a diagnosis of chiasmal glioma.

Eye muscle surgery for infantile nystagmus syndrome in the first two years of life

To report visual and elctrophysioloigcal effects of eye muscle surgery in young patients with infantile nystagmus syndrome (INS). Prospective, interventional case cohort of 19 patients aged under 24 months who were operated on for combinations of strabismus, an anomalous head posture, and nystagmus. All patients were followed at least nine months. Outcome measures, part of an institutionally approved study, included Teller acuity, head position, strabismic deviation, and eye movement recordings, from which waveform types and a nystagmus optimal foveation fraction (NOFF). Computerized parametric and nonparametric statistical analysis of data were perfomed using standard software on both individual and group data. Age averaged 17.7 months (13.1-month follow-up). Thirteen (68%) patients had associated optic nerve or retinal disease. 42% had amblyopia, 68% had refractive errors. Group means in binocular Teller acuity (P < 0.05), strabismic deviation (P < 0.05), head posture (P < 0.001), and the NOFF measures (P < 0.01) from eye movement recordings improved in all patients. There was a change in null zone waveforms to more favorable jerk types. There were no reoperations or surgical complications. Surgery on the extraocular muscles in patients aged less than two years with INS results in improvements in multiple aspects of ocular motor and visual function.

Traumatic Brain Injury–Induced Anomalous Head Posture and Vertical Diplopia

Traumatic Brain Injury–Induced Anomalous Head Posture and Vertical Diplopia

Infantile Aperiodic Alternating Nystagmus

This study identifies the clinical and ocular motility characteristics of the periodic and aperiodic forms of infantile alternating nystagmus (IAPAN) and establishes the range of electrophysiological and clinical characteristics while providing clues to its presence and pathophysiology. Seventy-eight patients with ocular oscillations consistent with IAPAN were reported. Outcome variables were: age, follow-up in months, vision, strabismus, other eye and systemic abnormalities, head position, periodicity, cycle and null period duration, foveation time, waveforms, and cycle symmetry. Age range was 1 to 67 years, 50% had pure periodic and aperiodic forms, 46% had albinism, 26% had binocular acuity of 20/40 or greater, 72% had strabismus, 35% had amblyopia, 31% had other eye disease, 14% had systemic disease, 87% had an anomalous head posture, and 65% had binocular directional asymmetry. The periodic cycle averaged 224 seconds and the aperiodic cycle ranged from 2 to more than 300 seconds. One in three patients with strabismus and nystagmus periodicity had a static head posture. Fifteen percent of the infantile nystagmus syndrome population had either the periodic or aperiodic form. A changing null period is often clinically missed because of long or irregular cycles, decreased acuity, associated strabismus, and either a nonexistent or inconsistent head posture. The changing null period is easier to recognize using eye movement recordings or if the non-preferred eye is occluded and the preferred eye is examined with the head straight and gaze in primary position for at least 5 to 7 minutes. The recognition of this variant has profound treatment implications.

Kestenbaum procedure with posterior fixation suture for anomalous head posture in infantile nystagmus

BackgroundThe purpose of this study was to report the effect of combining the Kestenbaum procedure with posterior fixation suture for infantile horizontal nystagmus with anomalous head posture (AHP) in children.MethodsNine consecutive patients who underwent combined Kestenbaum procedure plus posterior fixation suture to the recessed muscles at the same time were retrospectively studied. All patients were orthotropic before surgery and were followed for at least 6 months. Pre- and postoperative AHP and binocular corrected visual acuity (BCVA), and ocular alignment were assessed.ResultsMean age at surgery was 4.8 ± 1.5 years. The average follow-up was 29.7 months. The average head turn preoperatively was 27.4° and postoperatively 7.2°. The average net change in AHP was 24.8° (P = 0.008). Seven of 9 patients (78%) achieved a residual head turn of 10° or less. The average Log Mar BCVA was 0.33 preoperatively and 0.31 postoperatively (P = 0.68). Only 1 patient needed additional surgery for residual horizontal AHP. No patient developed strabismus.ConclusionCombined Kestenbaum procedure with posterior fixation suture was an effective and stable procedure in reducing AHP of the range of 20° to 35° in children with infantile nystagmus.