Development of acquired non-accommodative esotropia in a patient without a trochlear nerve

高居翰教授曾在美國(guó)華盛頓弗利爾美術(shù)館服務(wù)近十年，并擔(dān)任該館的中國(guó)藝術(shù)部主任。他也曾擔(dān)任已故瑞典藝術(shù)史學(xué)者喜龍仁的助理，協(xié)助其完成了七卷本《中國(guó)繪畫》(Chinese Painting:Leading Masters and Principles)的撰寫計(jì)劃，自1965年起，他開始任教于伯格利分校加州大學(xué)的藝術(shù)史系，負(fù)責(zé)中國(guó)藝術(shù)史的課程，1997年獲得學(xué)院頒發(fā)的終生杰出成就獎(jiǎng)，美國(guó)時(shí)間2014年2月14日，高居翰病逝于加州。

Congenital cranial dysinnervation disorders (CCDD)are neurodevelopmental diseases of the brainstem and cranial nerves

. Congenital superior oblique palsy (SOP) is one of the most representative CCDDs because approximately three quarters of patients show trochlear nerve agenesis and superior oblique (SO) hypoplasia

. Vertical diplopia associated with congenital SOP could emerge during the later life due to acute decompensation of vertical fusion

. Meanwhile,acquired non-accommodative esotropia (ANAET) is one of the common causes of horizontal diplopia

. Neuroradiological investigation has revealed Chiari malformation or intracranial masses in patients with ANAET

. However, the association of trochlear nerve aplasia and ANAET has never been reported.Herein, we found a patient with ANAET in whom trochlear nerve aplasia was confirmed with high-resolution thin-section magnetic resonance imaging (MRI).

A 10-year-old boy presented with intermittent esotropia which developed one month prior. He was born full term with no significant perinatal history. He denied any history of previous head trauma or family history of strabismus. His parents denied any history of longstanding or intermittent head tilt which was also not found in old photographs.

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On ophthalmologic examination, he showed no head tilt or facial asymmetry. His visual acuities were 20/25 OU. Prism and alternate cover test revealed constant esotropia of 45 prism diopters (PD) at distance and at near in the primary position.He showed additional right hypertropia of 4 PD only with the head tilted to the right. Ductions and versions showed a mild limitation of depression on adduction OD (Figure 1A). The pupils were round and isocoric in both eyes. Dilated fundus examination was unremarkable in both eyes. Cycloplegic refraction showed +1.25 D OD and +1.50 D OS.

The typical characteristics of congenital SOP patients with trochlear nerve aplasia include an early onset of severe head tilt before 1 year of age, and apparent facial asymmetry

.However, our patient did not show any of the characteristics of trochlear nerve aplasia, thus congenital SOP could not be expected. Dosunmu

found that manifestation of symptoms in presumed congenital SOP patients peaked in the fourth decade, therefore there is a chance that our patient may develop vertical diplopia later in his life.

Regarding the horizontal deviation associated with SOP,horizontal strabismus was present in 42% and exotropia was eight times more prevalent than esotropia in Asian patients without the trochlear nerve

. Helveston

also reported that 75% were exotropes in unilateral SOP patients who received surgical treatment. In contrast, Hata

reported that esotropia was more common than exotropia in isolated decompensated SOP which was contrary to the previous reports. Therefore, further studies would be necessary to elucidate the characteristics of horizontal deviation associated with congenital SOP.

德氏乳桿菌（Lactobacillus delbrueckii）亞種保加利亞乳桿菌是使用D-乳酸脫氫酶（DLDH）將丙酮酸鹽主要轉(zhuǎn)化為D-乳酸的非均勻乳酸菌，其功能特性仍然不佳，進(jìn)一步的研究表明，乳酸脫氫酶活性受氧下調(diào)，因此在厭氧條件下達(dá)到最高的D-乳酸滴度為1.94 g/L[29]。D-乳酸是德氏乳桿菌的主要乳酸產(chǎn)物，但不能被人體腸道代謝，Zhang J等[30]進(jìn)一步研究了氨基酸水平上D-LDH和L-LDH基因的進(jìn)化，發(fā)現(xiàn)德氏乳桿菌D-LDH基因是正向選擇的，可能是長(zhǎng)期馴化的結(jié)果。Viana R[31]發(fā)現(xiàn)干酪乳桿菌有兩種特征基因編碼乳酸脫氫酶活性，它特別催化了L-乳酸的形成。

Using a 3-tesla MRI system (Ingenia CX, Philips, Best, the Netherlands), T2-weighted coronal imaging of the orbit and high-resolution transverse imaging of the cranial nerves were performed. Mild hypoplasia of the right SO muscle was observed (Figure 1B), and the right trochlear nerve was absent(Figure 1C-1E).

In this report, a patient with ANAET was found to have trochlear nerve aplasia which was unexpected because he showed no vertical diplopia and vertical deviation in the primary position, nor any head tilt and facial asymmetry.The only evidence of trochlear nerve aplasia was a right hypertropia of 4 PD only with the head tilted to the right and a mild limitation of depression on adduction. Therefore, it is hard to believe that ANAET developed associated with decompensated congenital SOP.

In conclusion, trochlear nerve aplasia could be found in a patient with acquired, comitant non-accommodative esotropia.

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