The relationship between external bony defects and widened lateral interorbital distance in frontoethmoidal encephalomeningocele

PurposeThe aim of this study was to identify the prevalence of the abnormal lateral interorbital distance (LIOD), micropthalmia or anopthalmia and incidence of hydrocephalus in frontoethmoidal encephalomeningocele (FEEM), and to identify the correlation between the external bony defects and a widened lateral orbital wall.Materials and methodsFEEM patients who were treated in the Faculty of Medicine, Chiang Mai University between January 1, 2006, and December 31, 2016, were retrospectively reviewed from the medical records. We included the patients who had undergone head computed tomography (CT) of the facial bones. Exclusion criteria were any patients who had undergone prior facial surgery or had maxillofacial bone trauma before they underwent a CT scan, craniofacial disease such as facial clefts, frontonasal dysplasia, craniosynostosis, and inter-frontal encephalocele. Measurements were taken from the CT images to establish the diameter of the external bony defect, medial interorbital distance and lateral interorbital distance. The relationships between the external bony defect and widened LIOD were analyzed. Patients were divided into two groups: those with normal LIOD and those with wide LIOD.ResultsA total of 94 FEEM patients were evaluated. Thirteen patients (13.83%) had a wider than normal LIOD. The incidence of micropthalmia or anopthalmia was 9.57% and hydrocephalus was 45.74%. From a univariable model, there were no statistically significant differences in terms of patient characteristics between the LIOD groups, except for sex. The proportion of wide LIOD was higher in male patients (male vs female; 20.34% vs 2.86%, p = 0.027). The proportion between the external bony defect and the LIOD (EL ratio) associated with a wide LIOD was shown in a cutoff point of 23% (AuROC = 73.27%, 95%CI = 59.87%–86.66%). The multivariable analysis indicated factors associated with abnormal LIOD were an EL ratio of 23% (adjusted RR = 1.98, 95% CI = 1.58–2.51) and male sex (adjusted RR = 6.85, 95% CI = 2.00–23.55).ConclusionThere is an association between the EL ratio and widening of the LIOD. An EL ratio greater than 23% could be used to moderately predict a wide LIOD. A large external bony defect may be associated with lateral displacement of the orbit causing true hypertelorism. Therefore, orbital translocation must be considered in patients with a wide LIOD.     

Complete Recovery Following Electroacupuncture Therapy in Refractory Unilateral Sensorineural Hearing Loss

Role of electroacupuncture (EA) in refractory unilateral sensorineural hearing loss (SNHL) remains unclear but might be promising for the Meniere's disease. Two cases of unilateral SNHL who were unresponsive to conventional treatment of sudden SNHL showed complete recovery after receiving EA therapy. The first case was a 46-year-old woman who received EA in the seventh month after the acute onset of sudden right hearing loss and tinnitus. She had mild-to-moderate degree of SNHL at high frequencies in the right ear with episodic vertigo. The second case was a 55-year-old woman who received EA in the sixth year after developing sudden SNHL in the right ear. Before the EA began, her pure tone average of the affected ear was 45 dB and the phonetically balanced score was 88%. The regimen for both patients included 12 sessions of EA over four weeks at the main acupoints (Tinggong (SI 19), Ermen (TE 21), Qimai (TE 18) and Yifeng (TE 17) on the affected ear and the adjuvant acupoints (Zhongzhu (TE 3), Hegu (LI 4), Qihai (CV 6), Guanyuan (CV 4), Taixi (KI 3), and Taichong (LIV 3)). Both patients regained their normal hearing thresholds three weeks after the first EA. No adverse events were observed. Hence, EA may be a useful additional therapy in unilateral SNHL, even at the late phase when other treatments have failed because the possibility of Meniere's disease cannot be excluded.     

Split hand-foot malformation and a novel WNT10B mutation

We report an Indian girl with split-hand/foot malformation (SHFM), sparse hair, and interrupted eyebrows, who carries a novel homozygous deletion c.695_697delACA in WNT10B. The variant is deduced to cause an in-frame deletion of Asn residue 232 (p.Asn232del). According to the protein model, this single amino acid deletion at the critical position in the protein structure is likely to severely affect the protein structure and function. This deletion is likely to lead decreased lifetime and make it unable to bind to its receptors and other ligands. The patient and all family members had normal bone density and they were not obese like some of the patients with WNT10B variants. Here we report a patient with SHFM6 who carried a novel WNT10B mutation. Sparse hair and interrupted eyebrows may be associated findings of SHFM6.     

How to Reconstruct Middle Hepatic Vein Branches With Explanted Portal Vein and Inferior Mesenteric Vein Graft: A Case Report

Objective

The middle hepatic vein reconstruction is one of the crucial parts in adult living donor liver transplantation. Numerous techniques had been reported by using cadaveric iliac vessel or synthetic graft. The limitations of reported techniques are availability of the vessel and complication of synthetic graft. We report the technique of using explanted portal vein and inferior mesenteric vein graft in sequential fashion.