Surgical Approach to Hemipalatal Discrepancy in Tessier 7 Reconstruction: Review of Literature and Case Series.

OBJECTIVE: The authors present findings and techniques to address hemipalatal discrepancy in patients with Tessier 7 cleft and associated cleft palate during cleft palatoplasty. DESIGN: The authors report 2 cases of pediatric patients with Tessier 7 facial clefts and associated cleft palate. One patient presents on the broader oculo-auriculo-vertebral spectrum and the other is has isolated Tessier cleft 7. Additionally, a PubMed search was performed using the MeSH terms "tessier 7," "cleft palate", "macrostomia," "tessier 7 AND cleft palate," "macrostomia AND cleft palate," AND "hemipalatal discrepancy." All relevant literature was identified and underwent full review for qualitative analysis. RESULTS: Two patients met criteria for inclusion in this article. The surgical techniques utilized to mitigate the hemipalatal length discrepancy are detailed, and intraoperative photographs are provided. The results of the literature review are also presented. Tessier 7 craniofacial cleft and palatal clefts, when occurring in combination, is noted to result in discrepant hemipalatal length with short maxillary palate length on the affected side as well hypoplasia of the associated speech musculature. The postoperative palatal length after palatoplasty in both patients was longer than the preoperative hypoplastic palatal length. CONCLUSIONS: When occurring in combination, Tessier 7 craniofacial cleft and concomitant palatal cleft results in discrepant hemipalatal length, and deficiency of the bony maxillary palatal shelves, and associated speech musculature and soft tissues. The techniques described in this article may assist in maximizing postoperative palatal length.

Current Trends in Management of Marjolin's Ulcer: A Systematic Review.

Marjolin's ulcer (MU) is a rare, aggressive entity with frequent delay in diagnosis for a variety of regions. Although well described and classically taught in medical school, aspects of its treatment remain ill-defined and controversial. A systematic review was performed according to PRISMA guidelines to identify studies discussing patients who underwent surgical treatment of MU. A total of 31 papers, reporting on 1016 patients, were included. Burns were the most common etiology of malignant degeneration (68%), followed by trauma. The lower extremity was most affected (51%) and Squamous Cell Carcinoma (SCC) was found in 94% of cases, with the majority being well differentiated. Basal cell carcinoma and melenoma composed a minority of cases. Melanoma occurred more frequently in previously skin grafted wounds and had a higher rate of metastases than SCC. Most patients did not have associated regional or distant metastases present at diagnosis. Wide local excision (71%) was performed in most cases, unless amputation was indicated for severe disease or bone involvement. Lymphadenectomy and sentinel lymph node biopsy were variably reported, with conflicting evidence on the efficacy. Lymphadenectomy was most commonly indicated for known lymph node involvement. In cases of metastatic disease chemotherapy and radiation were used in conjunction with surgical treatment. Despite numerous articles on this topic, controversy remains in the management of MU. Early diagnosis of suspicious chronic wounds and prompt surgical intervention remains imperative to its treatment.

Efficacy of Staged Oronasal Fistula Reconstruction and Improved Bone Graft Survival in Cleft Patients Undergoing Secondary Reconstruction.

OBJECTIVE: To evaluate the efficacy of the senior author's technique of staged reconstruction in patients with recalcitrant oronasal fistulas. DESIGN: A retrospective review of the Pediatric Plastic Surgery Cleft & Craniofacial Surgery Database of cases from September 2013 to December 2018 was conducted. PATIENTS: A total of 31 patients who had previously undergone >1 surgical attempt to repair a fistula or patients who have failed >1 attempt at bone graft were included in this study. All patients were referrals from outside facilities. MAIN OUTCOME MEASURES: Primary outcomes examined included fistula recurrence, infection rates, ability to proceed with second stage bone grafting after first stage fistula takedown and reconstruction, and bone graft loss. RESULTS: Charts of 1053 patients were reviewed and 31 (2.94%) cases met inclusion criteria for this study. Nineteen (61.3%) of these patients proceeded with the second stage of reconstruction and 100% did not experience any graft loss. Seven patients who completed the first stage are undergoing orthodontic optimization prior to bone grafting. The remaining 5 are adult patients not interested in pursuing bone grafting. All 31 patients with recurrent and recalcitrant fistulas had successful fistula reconstruction with our approach, despite multiple previous failed reconstructions. CONCLUSIONS: The 2-staged reconstructive approach described herein effectively resulted in resolution of prior recurrent recalcitrant fistulas and resulted in eventual bone grafting. By employing this technique, we report successful fistula repair and bone grafting in patients who had previously undergone multiple surgical reconstructions.

Diagnosis and Treatment of Scalp Arteriovenous Malformations With Intracranial Extension.

Scalp arteriovenous malformations (AVMs) are rare entities that may present as large, tortuous vascular lesions with resultant craniofacial deformity. Radiologic findings include feeding arteries and veins associated with dilated tortuous vessels. Intracranial extension is rare. A 5-year-old African American male presented with an occipital mass presumed to be a dermoid cyst on referral. Further workup revealed the presence of a scalp AVM that demonstrated extension into the left sigmoid sinus. Radiologic and intraoperative images are presented. Literature search revealed only 4 patients with scalp AVM extending intracranially into the sagittal sinus, 2 of which were managed with coagulation and division of the feeding vessels and the other 2 underwent preoperative embolization. Our patient is the 1st to be reported to have sigmoid sinus extension. Scalp AVMs with intracranial extension are rare, and require further preoperative workup. Following ultrasound evaluation, computed tomography angiography, magnetic resonance angiography, magnetic resonance imaging, or cerebral angiography can be considered. Treatment entails resection, but preoperative embolization is increasingly used, mirroring patients without intracranial extension. Further studies are needed to evaluate the efficacy and risk profile of these treatments.

Cranial Erosion Associated With Non-Midline Dermoid Cysts in the Pediatric Population.

BACKGROUND: While the risk for intracranial extension of midline nasoglabellar dermoid cysts is well-described, the risk in non-midline dermoid cysts and role of preoperative imaging in these lesions is less understood. This study aims to address this gap and provide preoperative recommendations. MATERIALS AND METHODS: A retrospective review was performed evaluating patients who underwent excision of craniofacial dermoid cysts by a single surgeon. Findings on preoperative physical examination and imaging modalities were compared to intraoperative findings to assess effectiveness of each in predicting bony erosion. A treatment algorithm is proposed. RESULTS: Twenty-eight patients were included and 50% had intraoperatively confirmed bony erosion. Bony erosion was identified in 100% of lesions immobile on examination, compared to 30% of mobile lesions (P = 0.001). Bony erosion was identified in 40.9% of lateral brow cysts compared to 83.3% of lesions located elsewhere (P = 0.03), in 60% of patients with periorbital sequelae compared to 47.8% without periorbital sequelae, and in 66.7% of patients with reported change in cyst size with Valsalva compared to 48.0% without change with Valsalva. Sensitivities for preoperative identification of bony erosion were as follows: physical examination 57.1%, US 11.1%, MRI 66.7%, and CT 100%. CONCLUSIONS: A management algorithm for non-midline dermoid cysts is presented. CT or MRI is recommended for lesions that are immobile, in atypical locations, or have associated periorbital sequelae or change with Valsalva. The authors feel there is no role for ultrasound and don't recommend routine imaging in patients with non-midline dermoid cysts without features suggestive of bony erosion.

Surgical Management of Pediatric Lymphedema: A Systematic Review.

BACKGROUND: Lymphedema is a rare, progressive, and debilitating condition caused by failure of the lymphatic system to adequately drain the protein-rich fluid exiting the capillaries. Conservative management is often emphasized in pediatric patients, resulting in a paucity of literature describing surgical treatment in this population. METHODS: A systematic review was performed using PubMed, ScienceDirect, and Google Scholar to identify all studies describing surgical management of lymphedema in the pediatric population. RESULTS: Of the 343 relevant articles identified, 14 met the criteria for full review. Articles were divided into the following treatment categories: genital lymphedema, excisional procedures for extremity lymphedema, and physiologic procedures for extremity lymphedema. Outcomes for genital lymphedema were overall positive. For extremity lymphedema, excisional procedures yielded good results overall and included the majority of patients in this study. Physiologic procedures had mixed outcomes in the small population included in this study, but definitive conclusions cannot be drawn due to the paucity of existing data. CONCLUSION: While conservative management of pediatric lymphedema is well described, the literature remains sparse regarding surgical treatment. Further studies are needed to evaluate efficacy and outcomes in all three categories in this population.

The distribution and extent of reflux and obstruction in patients with active venous ulceration.

OBJECTIVES: This study was performed to precisely define the underlying pathophysiology in patients with active venous ulcers. METHODS: A PubMed search was conducted from 1991 to 2013 to select papers reporting the anatomic and physiologic etiology of ulceration in CEAP Class 6 patients. Studies which did not decipher between active and healed ulcers, did not use clear definitions, or did not give detailed accounts on the distribution/extent of venous pathology were excluded. Using the PRISMA guidelines, 12 studies were selected for further analysis. RESULTS: Primary insufficiency was reportedly the most common etiology of ulcers. Reflux most frequently occurred in the superficial system, either isolated or in conjunction with perforating and/or deep systems. Combined superficial and deep disease was observed in a median of 11.6% of limbs (range of 0-48%). Triple system disease was seen in a median of 31.6% of limbs (range of 22-52%). Isolated deep reflux was infrequently reported (2.1-28.4% of limbs). Previous deep venous thrombosis, reported in a median of 33% of patients, is likely underreported as it may resolve without detectable damage. CONCLUSION: There is a lack of data in the literature regarding the etiology of chronic active venous ulcers. Insufficiency of the superficial venous system from the micro- to the macro-vasculature has been frequently implicated in the development of venous ulceration. A prospective randomized controlled study is required for more conclusive results.