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Case Reports
. 2022 May 24;2022(5):rjac187.
doi: 10.1093/jscr/rjac187. eCollection 2022 May.

Cutaneous fistula on the cheek associated with oculo-auriculo-vertebral spectrum

Soh Nishimoto  1 , Kenichiro Kawai  1 , Toshihiro Fujiwara  1 , Hisako Ishise  1 , Masao Kakibuchi  1
Affiliations
Case Reports

Cutaneous fistula on the cheek associated with oculo-auriculo-vertebral spectrum

Soh Nishimoto et al. J Surg Case Rep. .

Abstract

A Japanese boy, presented with epibulbar dermoid and ipsilateral preauricular appendages, had a pit on his cheek of the same side. An atrial septal defect and vertebral fusions were also identified. He was diagnosed with a mild type of oculo-auriculo-vertebral spectrum (OAVS). At the age of 18 months, his cheek was swollen with a slight fever. An infected cyst and cutaneous fistula enveloped by the risorius muscle were extracted. It was assumed to be a remnant of the fissure between the maxillary and mandibular prominences. This was the first case of cutaneous fistula confirmed histologically with OAVS, although there seem to be more cases. The possibility of the mechanism of smiling cheek dimple is also discussed.

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Figures

Figure 1
Figure 1
(A) A 2-month-old baby presented a small pit outside of his right oral commissure. The difference between the left and right cheeks was not remarkable. (B) There presented preauricular appendages on his right side. (C) There presented an epibulbar dermoid inside of his right external eye angle.
Figure 2
Figure 2
At the age of one and a half, his right cheek was swollen with a slight fever. No discharge from the pit was observed.
Figure 3
Figure 3
(A) A transverse sT2W mDixon MRI image. A cystic lesion beneath the superficial musculo-aponeurotic system is identified. (B) A coronal section of MRI.
Figure 4
Figure 4
Christal violet ink was injected from the cheek pit. The ink came out from the perforated hole. An elongated retro-mandibular incision was designed.
Figure 5
Figure 5
The yellow arrow points one of the buccal branches of facial nerve. The white arrow points Steensen’s duct. C: cyst, F: fistula, canulated with a plastic outer tube of an intravenous catheter.
Figure 6
Figure 6
Extracted fistula and cyst. A, B, C indicate cross-sections for histology.
Figure 7
Figure 7
(A) Section at A in Fig. 6. ×2 objective lens. The fistula wall consists of squamous cells. The lumen is filled with cornified material. (B-a) Section at B in Fig. 6. ×2 objective lens. Many hair follicles can be seen. Adjacent to the fistula, chronic inflammation is observed. (B-b) Section at B in Fig. 6. ×10 objective lens. The fistula is surrounded by skeletal muscle. (C-a) Section at C in Fig. 6. ×2 objective lens. At this section level, the fistula is not accompanied by muscles. (C-b) Section at C in Fig. 6. ×10 objective lens. Long hair follicles and glands can be observed.
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