A 50-year-old single Filipino woman was referred to our clinic by the Dermatology Department due to multiple large nevi on the face. Her history started 29 years prior to consult when a 0.5 cm by 0.5 cm nevus appeared on her right lower eyelid. Excision of the mass and histopathology revealed basal cell carcinoma of the skin and she ceased followup visits. Meanwhile, progressively enlarging nevi appeared over multiple sites of her face. Some of the lesions developed ulceration and occasionally bled. Finally, she consulted again at our institution due to disfiguring multiple large nevi, and was seen by Dermatology and Ophthalmology services and underwent excision biopsy revealing basal cell carcinoma. She was then referred to us for definitive surgical management

The patient was a non-smoker, non-alcoholic beverage drinker and work did not undergo any prolonged sun exposure. She recalled that her mother had a similar condition and expired due to complications of the disease

Physical examination revealed many large nevi over multiple sites of the face, the largest over the left nasolabial area. (Figure 1A) There were hyperpigmented nevi over the central forehead and left infraorbital area, and the patient's left eye was closed due to scarring from the previous excision in the left medial canthal area. (Figure 1B) An ulcerating lesion that occasionally bled, involved multiple subsites of the nose. (Figure 1C)

Due to the recurrent multiple basal cell carcinoma on the face, we suspected a possible syndromic disease. Complete systemic physical examination revealed multiple nevi over the chest and back as well as plantar and palmar pits. (Figure 2A, B) Chest radiography revealed an incidental finding of a bifid third rib on the left. (Figure 3) With these findings, we diagnosed her condition as Gorlin-Goltz syndrome with multiple basal cell carcinoma on the face.

Our goal of treatment was complete excision of tumors with preservation of function and cosmesis. Following the National Comprehensive Cancer Network (NCCN) Guidelines1 surgical excision with frozen section for adequate margins was performed. (Figure 4) Reconstruction with multiple skin grafts was performed to cover the defects. However, graft failures were noted over multiple sites two weeks post-operatively. (Figure 5) 

Our patient continued to follow-up for a year but declined any offers of reconstructive surgery. She maintained a good disposition and was satisfied with her appearance despite a less than ideal aesthetic postoperative outcome. (Figure 6)

DISCUSSION Nevoid Basal Cell Carcinoma or Gorlin-Goltz Syndrome is a rare autosomal dominant syndrome with near complete penetrance and extreme variable expressivity.2,3 This was first described in depth by Doctors Robert Gorlin and Robert Goltz in 1960. Genetic mutation in PTCH1 and SUFU that are related with the Hedgehog signalling pathway were identified in the pathogenesis of this disease.2 GorlinGoltz syndrome commonly presents with dermatologic, odontogenic and neurologic findings and affected patients have developmental anomalies and predisposition to cancer, specifically basal cell carcinoma (BCC). The incidence of Gorlin-Goltz syndrome ranges from 1 in 50,000 to 1 in 560,0004 with only one published case reported in the Philippines.5

To establish the diagnosis of Gorlin-Goltz syndrome, either one major and two minor criteria or two major criteria must be fulfilled.6,7 Our patient presented with multiple BCC, bifid third left rib and palmar and plantar pits, fulfilling three major criteria.

Only 67% of patients diagnosed with Gorlin-Goltz syndrome present with basal cell carcinoma with an equal male to female ratio.8 The mean age of BCC presentation in Gorlin-Goltz syndrome is roughly 25 years old and the probability of developing increases with age.9 There are racial differences in the occurrence of BCC; higher in Caucasians than in African-Americans and Asians.6,8 However, BCC in patients diagnosed with Gorlin-Goltz syndrome have the same histology and presentation as sporadic cases.

Palmar and plantar pits are among the common dermatologic findings in Gorlin-Goltz syndrome. These lesions are found in 45% to 87% of Gorlin-Goltz syndrome and the percentage rises with age.6 The presence of palmar and plantar pits in a child should prompt a complete physical evaluation due to its association with other diseases.

A bifid or forked rib is a developmental abnormality in which the sternal end is cleaved in two. This may be asymptomatic and is oftentimes an incidental finding, and can be observed as an isolated defect or may be associated with other multisystem malformations. In the general population, it was reported to occur at 3 to 6.3 per 1,000.10 Among the rib anomalies, bifid rib occurs in 28% of cases.11 In GorlinGoltz syndrome, it occurs in 36.4% of cases.12

Gorlin-Goltz Syndrome has a wide spectrum of presentations varying from livable symptoms until adulthood to detrimental complications even during childhood. Since this is a genetic mutation, there is no cure for disease and treatment is symptomatic. In our case, there is higher chance of recurrence or new lesions that may require multiple surgical procedures in the future. Other lesions associated with this syndrome may still appear and immediate consultation is advised to prevent complications. Genetic counselling is highly advised since it has high inheritance.

In summary, our experience taught us that a high index of suspicion for syndromic disease and a complete physical examination are especially important in such cases. The diagnosis and management are challenging, and should consider the biopsychosocial context of the patient. As long as full disclosure of the condition is made and all options are clearly communicated, the patient's wishes should be respected.

 

Human ; Female ; Middle Aged (a Person 45-64 Years Of Age) ; Gorlin Goltz Syndrome

Objective: To present the case of a patient with left facial swelling as the primary manifestation of Multiple Myeloma, and discuss the surgical management, diagnostic dilemma, and subsequent medical management done for this unusual presentation. Methods: Design: Case Report. Setting: Tertiary Government Hospital. Patient: One (1). Results: A 55-year old man with an enlarging left pre-auricular mass of one (1) year duration underwent superficial parotidectomy with facial nerve preservation and selective lymphadenectomy for pleomorphic adenoma based on initial clinical and cytologic findings. Histopathologic examination showed plasmacytoid proliferation, and subsequent work-ups finally revealed Multiple Myeloma. Conclusion Emphasized in this case report is the thorough work-up and targeted therapy needed for the timely diagnosis and treatment of a patient with Multiple Myeloma.
Multiple Myeloma ; Plasmacytoma ; Parotid Gland ; Adenoma, Pleomorphic

OBJECTIVE: To determine if the anatomic dimensions (length, cross-sectional width, cortical thickness) of the Filipino fibula are ideal for mandibular reconstruction.
METHODS:
Design: Cross-Sectional Study
Setting: Anatomy dissection laboratory
Participants: 40 fibulas from 20 adult cadavers
RESULTS: Morphometric examination showed the mean length of the harvested fibulas was 33.5 cm. The mean horizontal (a-d) and vertical (b-c) widths of the proximal cross-section (point B) were 15.1 ± 0.28 mm and 9.9 ± 0.15 mm respectively. The mean horizontal (a-d) and vertical (b-c) widths of the distal cross-section (point D) were 15.4 ± 0.24 mm and 10.3 ± 0.49 mm, respectively. The mean cortical thickness of the anterior (a), lateral (b), posterior (c) and medial (d) aspects of the proximal cross-section (point B) were 5.2 ± 0.1 mm, 3.2 ± 0.04 mm, 3.6 ± 0.01 mm, and 2.9 ± 0.06 mm, respectively. The mean cortical thickness of the anterior (a), lateral (b), posterior (c) and medial (d) aspects of the distal cross-section (point D) were 5.1 ± 0.21 mm, 3.1 ± 0.11 mm, 3.5 ± 0.04 mm, and 2.9 ± 0.09 mm, respectively.
CONCLUSION: Our findings show that the Filipino fibulas studied have dimensions that are ideal for mandibular reconstruction.

Human ; Male ; Female ; Mandibular Reconstruction ; Fibula ; Cadaver ; Dissection

OBJECTIVE: To determine whether the interval from pathological diagnosis to treatment is significantly delayed, and the presence or absence of disease progression occurring in those with, and without treatment delay, among head and neck cancer patients in our institution.
METHODS:
Design: Retrospective Chart Review
Setting: Tertiary Government Hospital
Participants: Medical records of 70 patients with newly diagnosed head and neck cancer who underwent primary surgery from January 2011 to December 2015 were retrieved and available data were extracted.
RESULTS: A total of 28 patients were included in this study. Majority of the cancers were in the larynx (42.9%) and oral cavity (42.9%). The mean diagnostic-to-treatment interval (DTI) was 54 days but 5 (17.8%) out of the 28 had a DTI of more than 60 days. Four (80%) with a DTI more than 60 days had an upstage during surgery while 4 (17.4%) patients with DTI less than or equal to 60 days also had an upstage. 2 (60%) patients with treatment delay had tumor progression compared to 5 (21.7%) of those without treatment delay. Only 1 (20%) out of the 5 patients with treatment delay had increased nodal metastasis in contrast to 8 (34.8%) of those who did not have treatment delay.
CONCLUSION: A number of patients undergoing surgery in our institution experienced delay to initiate treatment of more than 60 days and majority of these patients were noted to have disease progression. However, even patients with treatment prior to 60 days had increases in tumor stage, which may suggest that the interval aimed for should be shorter than 60 days.

Human ; Male ; Female ; Head And Neck Neoplasms ; Neoplastic Processes ; Mouth ; Larynx ; Disease Progression ; Medical Records ; Government

OBJECTIVE: To determine the prevalence of, and describe transglottic cancer with thyroid cartilage invasion as a possible risk for, thyroid gland invasion in a series of patients with laryngeal carcinoma who underwent total laryngectomy with thyroidectomy.
METHODS:
Design: Retrospective Case Series
Setting: Tertiary Government Training Hospital
Participants: 61 laryngeal carcinoma patients who underwent total laryngectomy with hemi- or total thyroidectomy from January 2010 to August 2017.
RESULTS: Out of 61 patients with laryngeal carcinoma, 11 patients had supraglottic, 11 glottic, 2 subglottic and 37 had transglottic involvement. Eleven had thyroid cartilage invasion, all of whom had transglottic tumors. Of these 11 patients, only 1 had thyroid gland invasion. This was a case of a 78 year-old male patient with poorly differentiated SCC stage IVa transglottic tumor with thyroid cartilage invasion.
CONCLUSION: Thyroid gland invasion was uncommon in our sample of laryngeal carcinoma patients who underwent laryngectomy and thyroidectomy. Although transglottic involvement with thyroid cartilage invasion may increase the risk of thyroid gland invasion, it could not be confirmed by our series. Perhaps thyroidectomy should not be routinely performed on all patients with laryngeal carcinoma who undergo total laryngectomy but more rigorous studies are needed to establish this.

Human ; Male ; Female ; Aged ; Middle Aged ; Laryngectomy ; Thyroidectomy ; Thyroid Gland ; Thyroid Cartilage ; Prevalence ; Laryngeal Neoplasms ; Glottis

OBJECTIVE: To report a case of late-onset anterolateral thigh free flap failure in reconstruction of a defect from excision of buccal carcinoma.
METHODS:
Design: Case Report
Setting: Tertiary Government Training Hospital
Patient: One
RESULTS: A 57-year-old man with well-differentiated buccal squamous cell carcinoma underwent wide excision with segmental mandibulectomy, bilateral neck dissection and anterolateral thigh free flap reconstruction. Complete failure of the anterolateral thigh free flap was documented on the 29th post-operative day.
CONCLUSION: Late-onset flap failure is mainly non-vascular in etiology. However, flap failure is more likely multifactorial. Frequent follow-up after hospital discharge is recommended to monitor flap viability.

Human ; Male ; Middle Aged ; Free Tissue Flaps ; Thigh ; Neck Dissection ; Mandibular Osteotomy ; Reconstructive Surgical Procedures ; Carcinoma, Squamous Cell

Branchial cleft anomalies are among the most common causes of congenital anterior neck masses in the pediatric population. They present as epithelial-lined, single cysts. The definitive management is surgical excision. However, failure to remove the entire cyst and tract may lead to recurrence of the mass.

Unusual presentations of this condition may lead to incomplete excision if inadequately evaluated. There is a scarcity of material documenting atypical presentations of branchial cleft anomalies-- in particular, presentation as 2 distinct cysts in one region. In our literature search of PubMed, Google Scholar and HERDIN using the terms: "congenital mass," "branchial cleft cyst," and "multiple cysts," only 3 similar cases were found.

We report a case of a second branchial cleft anomaly presenting as a dumbbell-shaped mass (two cystic structures, connected by a tubular structure) in the right lateral neck, the subsequent management and outcomes.

Human ; Female ; Child Preschool ; Branchioma ; Branchial Cleft Anomalies ; Branchial Region ; Pharyngeal Diseases ; Craniofacial Abnormalities ; Neoplasm Recurrence, Local ; Head And Neck Neoplasms

p style=text-align: justify;strongOBJECTIVE:/strong To evaluate the necessity of placing a drain in post-thyroidectomy patients, we aimed to determine whether insertion of a passive drain as compared to no drain in post-thyroidectomy patients would significantly affect hematoma formation, wound infection, wound dehiscence and length of hospital stay.METHODS:br /Design:/strong Prospective randomized controlled trialbr /strongSetting:/strong Tertiary government training hospitalbr / strongSubjects:/strong Patients who underwent thyroidectomy for various pathologies were divided into two postoperative treatment arms: one group with insertion of a passive drain, and another group without a drain. Hematoma, wound infection, wound dehiscence and length of hospital stay were the outcomes measured per treatment arm.RESULTS:/strong A total of 66 patients were evaluated. There were 54 females (81.81%) and 12 males (18.18%). The mean age for the drain group was 44.88 years and 43.67 years for the no drain group. Four patients developed complications in the drain group and two developed complications in the no drain group. The rate of complications between both groups was not statistically significant. The mean hospital stay of the drain group was 3.15 days which in the no drain group was 2.51 days. The difference in length of hospital stay was statistically significant.CONCLUSIONS:/strong There was no difference in the development of complications among the drain and no drain group. Thyroidectomy without surgical drains was associated with a significant reduction in hospital stay compared to thyroidectomy with routine placement of drains./p
Human ; Male ; Female ; Aged 80 and over ; Aged ; Middle Aged ; Adult ; Thyroid Diseases ; Thyroidectomy-surgery ; Drainage ; Postoperative Care ; Thyroid Gland ; Postoperative Complications ; Hematoma

p style=text-align: justify;strongOBJECTIVE:/strong To determine the mean distance of the main trunk of the facial nerve from two commonly employed surgical landmarks (tragal pointer and tympanomastoid suture line) among a sample a Filipino adults undergoing parotidectomy.METHODS:br /Design:/strong Prospective descriptive studybr /strongSetting: /strongTertiary Government Training Studybr /strongSubjects:/strong 22 patients without facial paralysis undergoing surgery for parotid neoplasms were evaluated intraoperatively.RESULTS/strong: The main trunk of the facial nerve was found to be 9.0 mm (standard deviation of 2.8 mm) from the tragal pointer and 6.1 mm (standard deviation of 2.0 mm) from the tympanomastoid suture line.CONCLUSION/strong: The mean distance from the main trunk of the facial nerve to two of the most commonly utilized landmarks in identification of the nerve during parotidectomy was 9.0 mm (standard deviation of 2.8 mm) from the tragal pointer and 6.1 mm (standard deviation of 2.0 mm) from the tympanomastoid suture line. These may serve as reference values for surgeons in safer identification and preservation of the facial nerve during parotidectomy.
Human ; Male ; Female ; Adult ; Facial Nerve ; Sutures ; Anatomic Landmarks ; body regions

Objective: To present our application of a double anterolateral thigh (ALT) free flap in reconstruction of a large full thickness defect of the oral cavity, cheek and cervical area. Methods: Design: Case Report Setting: Tertiary Government Hospital Patient: One Results: A 77-year-old male with a 20 x 25 cm full thickness soft tissue defect on the facial and cervical area contiguous with a 6 x 6 cm buccal defect resulting from wide tumor ablation of a Stage IVA (T4aN2bM0) squamous cell carcinoma of the buccal mucosa underwent reconstruction using two ALT free flaps. An ALT flap was designed to cover the intraoral and cheek defect, while another ALT flap was used for external coverage of the cervical defect. The first ALT flap measured approximately 8 x 22 cm while the second ALT flap measured 6 x 22 cm harvested from the left and right thigh respectively. Temporary venous congestion was observed on the inferiorly placed ALT flap due to neck edema that spontaneously resolved on the 2nd post-operative day. Minimal donor site complications observed were linear scars, and a 1 x 4 cm dehiscence on the right thigh that healed spontaneously by secondary intention. Conclusion The utilization of a double anterolateral thigh free flap allowed single-stage reconstruction of the large soft tissue head and neck defect with little donor site morbidity, shorter operating time and shorter hospital stay.
Human ; Male ; Aged ; THIGH ; Free Tissue Flaps ; Mouth ; Cheek