Surgical Management of Vascular Malformations of the Upper Extremity: A 12-Year Retrospective Cohort Study.

INTRODUCTION: Vascular malformations (VMs) typically appear at birth and grow commensurately with patients. They can vary broadly in vessel type and tissue involvement, and upper extremity (UE) VMs can pose unique functional and aesthetic challenges in children. Given the advent of operative and nonoperative technologies like sclerotherapy and medications, a contemporary review of the surgical management of UE VMs is warranted. METHODS: We performed a retrospective review of all patients who had surgical management of VMs from 2010 to 2021 at The Children's Hospital of Philadelphia. Demographics, lesion characteristics, treatment (including preceding nonsurgical therapies), complications, and final outcomes were recorded. Operative notes were reviewed for date of operation, depth of excision, type of closure, and current procedural terminology code. RESULTS: Sixty-seven patients with 88 procedures were studied. Average patient age was 5.8 years, with 64% White and 67% male. Venous (34%) and lymphatic (19%) malformations were most common, and anatomic locations were most frequently on the hand (33%) and forearm (25%). The average lesion diameter was 4.2 cm, although this varied by location (eg, 2.9 cm, hand; 11.1 cm, chest wall). Fifty-eight patients (87%) underwent surgical excision as their index procedure, and 9 had sclerotherapy before surgery. Thirty-nine patients (60%) had subcutaneous excisions, and the remainder required subfascial or intramuscular excisions. Nearly all excisions were closed primarily (97%). Of the 53 patients with documented follow-up, 32 patients (60%) had complete resolution of their lesion as of their final visit. Thirty of these 32 patients with no clinical evidence of residual VM had only 1 surgery for excision. CONCLUSION: Upper extremity VMs were composed of diverse conditions with varying vessel types, size, depth, and anatomic sites. Surgical excision of VMs of the UE was safe and effective. A majority of VMs were fully excised after 1 procedure and frequently closed primarily with relatively low complication rates. Future work should investigate decision-making and outcomes of all treatment options of VMs of the UE for optimal functionality and aesthetics.

Progress about the fibro-adipose vascular anomaly: A review.

Fibro-adipose vascular anomaly (FAVA) is a rare and complex vascular malformation associated with persistent pain, limb contracture, and even restriction of activity. However, the pathophysiology of FAVA remains unclear. Although FAVA is a benign vascular malformation, it is highly misdiagnosed and often thus undergoing repeated surgical resection and interventional sclerotherapy, resulting in worsening of symptoms and irreversible dysfunction. Therefore, aggressive diagnosis and treatment are essential. There are several different treatment options for FAVA, including surgical resection, sclerotherapy, cryoablation, drug therapy, and physical therapy. This article reviews the clinical manifestations, pathological features, pathogenesis, and treatment methods of FAVA.

Robotic-assisted right upper lobectomy with systemic pulmonary vein anomaly: a case report.

BACKGROUND: While the role of low-dose computed tomography (CT) in lung cancer screening is established, its limitations in detailing pulmonary vascular variations are less emphasized. Three-dimensional reconstruction technology allows surgeons to reconstruct a patient's bronchial and pulmonary vascular structures using CT scan results. However, low-dose CT may not provide the same level of clarity as enhanced CT in displaying pulmonary vascular details. This limitation can be unfavorable for preoperative detection of potential pulmonary vascular variations, especially in cases involving planned segmentectomy. CASE PRESENTATION: We report a case of a 58-year-old female with lung cancer, initially planned for Da Vinci robot-assisted thoracoscopic segmentectomy. Unexpectedly, during surgery, a pulmonary vein variation in the right upper lobe was discovered, leading to a change in the surgical method to a lobectomy. The patient had four variant right upper lobe veins draining into the superior vena cava and one into the left atrium. The surgery was complicated by significant bleeding and postoperative pulmonary congestion. Postoperative pathology confirmed adenocarcinoma. CONCLUSIONS: This case highlights the importance of meticulous intraoperative exploration, particularly in cases involving planned segmentectomy, as unexpected pulmonary vein variations can significantly affect surgical decision-making. While three-dimensional reconstruction based on preoperative CT data is a valuable tool, it may not capture the full complexity of the anatomical variations. We discuss potential preoperative imaging techniques, including contrast-enhanced CT and CT angiography, as methods to better identify these variations. The enhanced visualization provided by robot-assisted surgery plays a crucial role in identifying and adapting to these variations, underscoring the advantages of this surgical approach. Our report contributes to the existing literature by providing a detailed account of how these principles were applied in a real-world scenario, reinforcing the need for surgical adaptability and awareness of the limitations of low-dose CT in complex cases.

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension.

Abernethy malformation is a congenital extra-hepatic porto-systemic shunt. This malformation is characterized by an abnormal connection between the portal vein or its branches and one of the systemic veins. Though rare, this anomaly can lead to pulmonary hypertension. Drainage of Abernethy malformation into coronary sinus is extremely rare. We describe a child with Abernethy malformation with unusual drainage into coronary sinus. The abnormal channel was successfully closed by trans-catheter technique with normalisation of pulmonary arterial pressures.

Clinical outcomes for 20 cats with congenital extrahepatic portosystemic shunts treated with ameroid constrictor ring attenuation (2002-2020).

OBJECTIVE: To report the clinical perioperative, short-term, and long-term outcomes for cats undergoing ameroid ring constrictor (ARC) attenuation of a congenital extrahepatic portosystemic shunt (EHPSS). STUDY DESIGN: Retrospective case series from a single veterinary teaching hospital (2002-2020). ANIMALS: Twenty client-owned cats with EHPSS. METHODS: Data collected from medical records included signalment, history, physical examination, clinicopathologic testing, medications, diagnostic imaging, intraoperative findings, perioperative complications, and postoperative clinical outcomes. Long-term clinical outcome was obtained from a standardized owner interview or medical records. RESULTS: Perioperative complications were reported in five cats out of 20, including blindness (two cats), ascites (one cat), head pressing (one cat), and seizures and death (one cat). Short-term clinical outcome was excellent in 14/18 cats, good in 2/18 cats, and poor in 2/18 cats that were available for follow up, and long term clinical outcome was excellent in 15/18, good in 1/18 cats, and poor in 2/18 cats that were available for follow up. CONCLUSION: Long-term clinical outcome was good or excellent in 16/18 of cats available for follow up. Perioperative complications were reported in five cats. CLINICAL SIGNIFICANCE: Surgical attenuation of EHPSS with an ARC can result in resolution of clinical signs and biochemical abnormalities in the majority of cats. The perioperative complication rate for feline patients with EHPSS attenuated with an ARC was lower than reported historically. Seizures may persist in the long term despite normal bile acid stimulation test results, complete blood count, and biochemistry analysis.

Wide Resection for Pediatric Extremity Vascular Malformation-A Tertiary Hospital's Experience.

BACKGROUND: Vascular malformations of the extremities (VM) are common benign lesions that tend to grow throughout the patient's lifetime. They can cause various issues like pain, swelling, and even limb length discrepancies. Sclerotherapy was the preferred treatment choice in previous studies. However, sclerotherapy and many other treatments have the potential to result in higher recurrence rates. Surgical treatment has been shown to be effective and safe in many cases. Hence, this study aims to evaluate the suitability of wide resection surgery for VM to reduce recurrence. METHODS: Fort-seven VM cases that underwent wide resection were identified retrospectively in the institution of study. Demographics, depth of malformation, whether malformations were local or diffuse, location and size of malformations, and histology records were taken note of. Records of recurrence and postoperative function were also gathered. We utilized self-reported questionnaires, QuickDASH and Lower Extremity Functional Scale, to determine patients' postsurgical physical function. RESULTS: Out of 47 cases that underwent wide resection, we found a recurrence rate of 2.1%. No patients sustained any loss of function postsurgery, with few patients experiencing minor complications like tenderness, hypertrophic scars/keloids, as well as numbness. Good functionality posttreatment was also seen through self-reported questionnaires, with an average score of 2.12 for QuickDASH and 99.96% for LEFS. CONCLUSION: Where margins can be obtained without functional impairment, surgical-wide resection for VM is a viable treatment option to minimize recurrence. LEVEL OF EVIDENCE: Level-IV.

Endovascular closure of a congenital extrahepatic portosystemic shunt for the treatment of hepatopulmonary syndrome in an infant.

Congenital portosystemic shunts may result in the development of hepatopulmonary syndrome, typically presenting with progressive hypoxemia in later childhood. We describe a case of a 5-month-old male with heterotaxy with polysplenia presenting with new onset hypoxemia. Subsequent evaluation identified an extrahepatic portosystemic shunt arising from the confluence of the main portal and superior mesenteric veins draining into the left renal vein. To treat his hypoxemia and prevent future complications of shunting, the patient underwent a successful single-stage endovascular closure.

Fibro-adipose vascular anomaly (FAVA) - diagnosis, staging and management.

BACKGROUND: The diagnosis and treatment of fibro-adipose vascular anomaly (FAVA) of the limb remains challenging since this entity is rare and complex. This paper is aimed to describe the clinical and imaging features, staging and management of this underrecognized disease of the limb. MATERIAL AND METHOD: Patients diagnosed with FAVA and managed between September 2019 and May 2022 in department of pediatric surgery & vascular anomalies of Xi'an international medical center hospital were retrospectively reviewed. Data extracted include age at presentation, previous diagnosis, affected muscles, symptoms, previous treatment, our management, and follow-up. RESULTS: Thirty-two patients with FAVA were diagnosed and managed in our center. There was a female sex predominance, with 23 female (72%) and 9 male (28%) in the cohort. Only one lesion was noticed during infancy; the remaining presented at age 1 to 20 years (median, 7 years). The most commonly involved muscles were gastrocnemius (14/32, 44%) and soleus (13/32, 40%). Swelling (mass), pain and contractures were the most common presentations. MRI featured a heterogeneous and ill-defined intramuscular high signal intensity. Diseases were staged according to clinical features: stage I (pain stage, n = 4), stage II (contracture stage, n = 20) and stage III (deformity stage, n = 8). Patients with stage I disease underwent radical resection and obtained a cure. Patients with stage II disease received radical resection and possible Achilles lengthening, having an outcome of cure. Personalized treatment was required in patients with stage III disease, including radical/partial/staged resection, Achilles lengthening/tenotomy, joint capsulotomy, neurolysis/neurectomy, tendon transfer, stretching exercises, and oral sirolimus/alpelisib. Significant improvement of symptoms was achieved in most. CONCLUSION: The most distinct features of FAVA include enlarging mass, severe pain and contracture. Based on distinct clinical and radiologic features, it is not difficult to make the diagnosis of FAVA. Earlier awareness of this disease can reduce misdiagnoses. Surgery-based comprehensive management can typically improve pain and contracture. Oral sirolimus or alpelisib plays an important role in treatment of unresectable lesions and major nerve involvement. Surgery alone can be curative in early stage FAVA.

Surgical treatment of anomalous right upper lobe pulmonary vein obstruction caused by compression between pulmonary artery and trachea: a case report.

The normal anatomical course of right upper lobe pulmonary vein involves drainage anteriorly to the pulmonary artery, ultimately reaching the left atrium. However, anomalies can occur with the most common variation involving the convergence of the right upper lobe pulmonary vein with the superior vena cava. In a rare pulmonary vascular malformation, the anomalous right upper lobe pulmonary vein takes a path between the right pulmonary artery and right main bronchus [1]. During a clinical consultation, a patient presented in our hospital with this specific anomalous right upper lobe pulmonary vein, along with an atrial septal defect and a patent ductus arteriosus. As a consequence of this aberrant positioning, the right upper lobe pulmonary vein was compressed between the pulmonary artery and trachea, leading to pulmonary vein obstruction. Thus, a successful pulmonary vein replantation was performed to correct the congenital malformation.

Intraosseous venous malformation of the zygoma: Case report and pooled analysis.

The objective of this study is to provide a comprehensive summary of the clinical and radiological features of zygomatic intraosseous venous malformations (IVM), as well as its treatment strategies. The aim is to establish preoperative diagnostic bases that will aid in the identification of zygomatic IVM and facilitate the implementation of effective treatment. Four cases of pathologically diagnosed zygomatic IVM with typical clinical and radiological features were reported. They exhibited comparable clinical and radiological features, and the En bloc excision and reconstruction yielded satisfactory outcome. Pooled data analysis was performed with additional 74 cases collected from 63 previous studies. The results revealed a higher incidence of zygomatic IVMs in middle-aged females, with swelling, pain and ocular dysfunction being the typical clinical manifestations. The characteristic radiological features of these lesions were well-defined, round bony structures with specific internal trabecular patterns. A diagnostic flow-chart assisting the differential diagnosis of IVM was established. En bloc excision was deemed the most advantageous treatment option, as it presented minimal risk of haemorrhaging and no instances of recurrence. The decision to pursue reconstruction was contingent upon the extent of the defect. Alloplastic material has emerged as the most frequently employed reconstruction material in recent reports. The summarized characteristics of zygomatic IVM and the proposed diagnostic and treatment strategies, derived from the pooled analysis of reported cases, may help to improve diagnosis and management in further clinical practice.

Repeated specific canine pancreatic lipase measurements do not identify multiple acquired portosystemic shunts in dogs after extrahepatic portosystemic shunt attenuation.

BACKGROUND: In dogs with portal hypertension (PH), spec cPL is suggested to be increased despite normal pancreatic histology. After attenuation of congenital extrahepatic portosystemic shunts (cEHPSS), multiple acquired portosystemic shunt (MAPSS) can develop as consequence of sustained PH. Presence of MAPSS affects future therapeutic options and prognosis. OBJECTIVE: Evaluate if spec cPL concentrations increase postoperatively in dogs that develop MAPSS and can thus serve as an indicator of PH. ANIMALS: Twenty-four dogs with cEHPSS. METHODS: Dogs classified according to surgical outcome after cEHPSS attenuation (8 with MAPSS [group M], 9 with closed cEHPSS [group C] and 7 with patent blood flow through the original cEHPSS, without evidence of MAPSS [group P]). Spec cPL was measured in preoperative samples (T0), 4 days (T1) and 1 (T2) and 3- to 6-months (T3) after surgery. RESULTS: Spec cPL was within reference interval (<200 µg/L) at all timepoints except at T1. At T1, 2 dogs in group M (321 and >2000 µg/L) and also 1 in group C (688 µg/L) and 1 in group P (839 µg/L) had increased spec cPL concentrations. No differences in spec cPL concentrations between groups or changes over time were identified. CONCLUSIONS AND CLINICAL IMPORTANCE: Spec cPL is not consistently increased in dogs that develop MAPSS after cEHPSS attenuation and has no potential as a biomarker for the identification of MAPSS after cEHPSS attenuation.

Successful Hybrid Approach Treatment of a Large Persistent Sciatic Artery Aneurysm-A Case Report.

Background and Objectives: Persistent sciatic artery (PSA) is a rare congenital vascular anomaly that is often asymptomatic, but can be associated with aneurysm formation and potential complications, such as thromboembolism or aneurysm rupture in some cases. We present a case of a 75-year-old woman with a symptomatic thrombus-containing aneurysm of the left PSA. Materials and Methods: The treatment of the PSA aneurysm involved a successful hybrid approach, which included open surgical bypass and endovascular embolization. The open surgical bypass was performed from the left common femoral artery to the left above-the-knee popliteal artery using a synthetic graft, while the aneurysm exclusion was achieved through endovascular plug embolization. Results: Control angiography revealed complete exclusion of the PSA aneurysm. At the 1-month follow-up, there were no palpable pulsatile masses in the left gluteal region, and the patient reported no symptoms. Conclusions: Given the high incidence of limb- and life-threatening complications associated with a PSA aneurysm, accurate diagnosis and appropriate treatment are crucial. In this case, a combination of open surgical and endovascular techniques resulted in a favorable outcome for the patient, highlighting the effectiveness of the hybrid approach in managing PSA aneurysms. Further studies are warranted to explore and refine treatment strategies for these complex vascular anomalies.

Patient-Reported Outcomes of Medical Tattooing for Capillary Malformations.

BACKGROUND: Patients with capillary malformations (CMs) may undergo medical tattooing (MT) as an alternative to laser therapy. But little is known about treatment results and impact from the patients' perspective. OBJECTIVES: In this cross-sectional digital survey study, we evaluated the patient-reported outcomes of MT for CMs. METHODS: MT practices were identified via the Dutch Association of Skin Therapists and Google. These practices invited all their CM patients who had undergone MT between January 2011 and September 2021 to participate. Baseline and treatment characteristics, tattooing effectiveness, patient satisfaction with treatment outcomes, and complications were evaluated using a custom-made online survey. Quality of life was assessed with the Dermatology Life Quality Index (DLQI) questionnaire. Factors associated with treatment effectiveness and patient satisfaction were identified via bivariate analysis and ordinal logistic regression analysis. RESULTS: Most of the 89 respondents were female (69%). Almost all CMs were located on the face (90%) and mainly (dark) red (74%). Nearly all patients had undergone laser therapy (91%). Median number of tattooing sessions was 5 (IQR: 4.0-8.0). Thirty-seven percent of the patients perceived >75% color reduction. Younger patients were more likely to obtain lower treatment effectiveness (OR 0.44, 95% CI: 0.20-0.97). Most patients (83%) were satisfied with treatment results. Patients with lighter (OR 0.30, 95% CI: 0.13-0.72), non-facial (OR 0.15, 95% CI: 0.03-0.89), and hypertrophic CMs (OR 0.30, 95% CI: 0.11-0.82) were less likely to be satisfied with treatment outcomes. Patients with lighter skin types were more satisfied (OR 2.89, 95% CI: 1.23-6.80). Complications included transient pain (23%), bleeding (3.4%), hypertrophic scarring (1.1%), hypopigmentation (1.1%), and a halo around the tattoo (1.1%). CONCLUSION: MT seems a valid alternative treatment in addition to laser therapy for CMs, with mild complications. Most patients are (very) satisfied with treatment results, while color reduction is incomplete. Hence, it seems appropriate to decide together with patients whether or not to use MT as primary treatment or secondary to laser therapy.

A patient with spinal cavernous vascular malformation: case report and review of the literature.

BACKGROUND: Spinal cavernous vascular malformation (SCM) is a rare type of spinal vascular malformation that can be easily misdiagnosed and overlooked, accounting for 5%-12% of all spinal vascular malformations. To date, surgical resection has been the gold standard for treating SCM, particularly in symptomatic patients. The risk of secondary hemorrhage in SCM is as high as 66%. Therefore, early, timely, and accurate diagnosis is crucial for patients with SCM. CASE REPORT: In this report, we describe a 50-year-old female patient who was admitted to the hospital with recurrent bilateral lower extremity pain and numbness for 10 years, with recurring symptoms for 4 months. The patient's symptoms initially improved after conservative treatment but then worsened again. An MRI revealed a spinal cord hemorrhage, and after surgical treatment, the patient's symptoms improved significantly. A postoperative pathological examination confirmed the diagnosis of SCM. CONCLUSIONS: This case, along with a review of the literature, suggests that for SCM, early surgery using techniques such as microsurgery and intraoperative evoked potential monitoring may result in better outcomes for the patient.

Laparoscopic surgery for rectal cancer with a coexisting Retzius shunt and inferior mesenteric arteriovenous malformation: A case report.

A coexisting short-circuit from the inferior mesenteric vein (IMV) to the inferior vena cava, known as a Retzius shunt, and arteriovenous malformation (AVM) of the inferior mesentery are extremely rare conditions. We encountered a case of rectal cancer with coexisting Retzius shunt and inferior mesenteric AVM successfully treated with laparoscopic surgery. Contrast computed tomography (CT) in a 62-year-old man with rectal cancer showed multiple dilated veins at the mesenterium of the descending sigmoid colon. These dilated veins were connected between the IMV and the left renal vein. A diagnosis of Retzius shunt was made, and laparoscopic low anterior resection with lymph node dissection was performed. A pathological examination of the colonic mesenterium revealed AVM communicating with the dilated IMV and Retzius shunt. The preoperative evaluation of aberrant vessels by three-dimensional CT is particularly useful for patients with vascular malformations to ensure safe laparoscopic surgery.

Venous malformations of the lower limb with severe localized intravascular coagulopathy treated with radiofrequency ablation and resection.

Diffuse venous malformations (VMs) are relatively rare, especially the lesions locting special anatomical sites, and they are prone to casuse localized intravascular coagulopathy (LIC). Diffuse VMs can also cause bleeding and life-threatening disseminated intravascular coagulopathy (DIC) from trauma, surgery, and improper treatments. Thus, the treatment of diffuse VMs with LIC is quite tough. We report of a diffuse VMs with severe LIC that was treated with the combined use of minimally invasive treatment and open surgery.

Surgical Resection of Vulvar Labial Venous Malformations: A Single Center Experience.

BACKGROUND: Venous malformations (VMs) involving the vulva are rare but often cause debilitating pain, aesthetic concern, and functional impairment. Treatment with medical therapy, sclerotherapy, operative resection, or a combination thereof may be considered. The optimal therapeutic approach remains unclear. We report our experience resecting labial VMs in a large cohort of patients. METHODS: A retrospective review of patients who underwent partial or complete resection of a labial VM was conducted. RESULTS: Thirty-one patients underwent forty-three resections of vulvar VMs between 1998 and 2022. Physical examination and imaging demonstrated: 16% of patients had focal labial lesions, 6% had multifocal labial lesions, and 77% had extensive labial lesions. Indications for intervention included pain (83%), appearance (21%), functional impairment (17%), bleeding (10%), and cellulitis (7%). Sixty-one percent of patients underwent a single resection, 13% multiple partial resections, and 26% a combination of sclerotherapy and operative resection(s). Median age was 16.3 years at first operation. All patients requiring multiple operations had extensive VMs. Median blood loss was 200 mL. Postoperative complications included wound infection/dehiscence (14%), hematoma (2%), and urinary tract infection (2%). The median follow-up assessment was 14 months: 88% of patients had no complaints and 3 patients were experiencing recurrent discomfort. CONCLUSIONS: Surgical resection is a safe and effective approach to treating vulvar labial VMs. Patients with focal or multifocal VMs can be successfully treated with a single resection, whereas patients with an extensive VM may require multiple partial resections or combined sclerotherapy and resection(s) to achieve long-term control. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: IV.

Outcome after surgical treatment of venous malformations of the hand in childhood.

OBJECTIVE: Surgical treatment of venous malformations (VMs) of the hand is challenging. The hand's small functional units, dense innervation, and terminal vasculature can be easily compromised during invasive interventions like surgery or sclerotherapy, leading to an increased risk of functional impairment, cosmetic consequences, and negative psychological effects. METHODS: We have conducted a retrospective review of all surgically treated patients diagnosed with VMs of the hand between 2000 and 2019 and evaluated their symptoms, diagnostic investigations, complications, and recurrences. RESULTS: Twenty-nine patients (females, n = 15) with a median age of 9.9 years (range, 0.6-18 years) were included. Eleven patients presented with VMs involving at least one of the fingers. In 16 patients, the palm and/or dorsum of the hand was affected. Two children presented with multifocal lesions. All patients presented with swelling. Preoperative imaging was done in 26 patients and consisted of magnetic resonance imaging in nine patients, ultrasound in eight patients, and both modalities in nine patients. Three patients underwent surgical resection of the lesions without any imaging. Indications for surgery were pain and restriction of function (n = 16), and when lesions were preoperatively evaluated as completely resectable (n = 11). In 17 patients, a complete surgical resection of the VMs was performed, whereas in 12 children, an incomplete resection of VM was deemed due to nerve sheath infiltration. At a median follow-up of 135 months (interquartile range, 136.5 months; range, 36-253 months), recurrence occurred in 11 patients (37.9%) after a median time of 22 months (range, 2-36 months). Eight patients (27.6%) were reoperated because of pain, whereas three patients were treated conservatively. The rate of recurrences did not significantly differ between patients presenting with (n = 7 of 12) or without (n = 4 of 17) local nerve infiltration (P = .119). All surgically treated patients who were diagnosed without preoperative imaging developed a relapse. CONCLUSIONS: VMs in the region of the hand are difficult to treat, and surgery is associated with a high recurrence rate. Accurate diagnostic imaging and meticulous surgery may contribute to improve the outcome of the patients.

Cochlear implantation in a patient with congenital microtia, cochlear hypoplasia, venous anomalies of the temporal bone and laryngomalacia: Challenges and surgical considerations.

RATIONALE AND PATIENT CONCERNS: Congenital hearing loss is often caused by an inner ear malformation, in such cases, the presence of other anomalies, such as microtia, and venous anomalies of the temporal bone and laryngomalacia makes it challenging to perform cochlear implantation surgery. DIAGNOSES: This study reports the case of a 28-month-old girl with congenital profound hearing loss, laryngomalacia, and malformed inner ear, who received cochlear implantation surgery. The bony structure, vessels and nerves were first assessed through magnetic resonance imaging and computed tomography before exploring the genetic basis of the condition using trio-based whole exome sequencing. Perioperative evaluation and management of the airway was then performed by experienced anesthesiologist, with the surgical challenges as well as problems encountered fully evaluated. INTERVENTIONS: Cochlear implantation was eventually performed using a trans-mastoid approach under uneventful general anesthesia. OUTCOMES: Due to the small size of the cochlea, a short electrode FLEX24 was inserted through the cochleostomy. LESSONS: Considering the high risk of facial nerve injury and limited access to the cochlea when patients present significant bony and venous anomalies, cochlear implantation in such patients require careful preoperative evaluation and thoughtful planning. In these cases, airway assessment, magnetic resonance venography, magnetic resonance arteriography, and magnetic resonance imaging and computed tomography can be useful to minimize the risks. Intraoperative facial nerve monitoring is also recommended to assist in the safe location of facial nerve.