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Background: In Crouzon syndrome, there is a growth disorder that affects the midface. it is retrusive and needs a facial advancement. We describe an intermediate midface osteotomy between Lefort 2 and 3, that respects the nose, combined with distraction on a Rigid External Distractor.

Material and method: We use an endobuccal approach. Pterygo maxillary disjunction, cut of nasal septum, oblique section of the zygomatic arch, section of the horizontal external orbital apophysis and of the lower orbital rim outside the nasal tear canal. The Rigid External Distractor is placed on the skull with two horizontal support bars, this allows four points for traction. After 1 mm a day, the mark of the correct positioning is the dental bite.Six weeks later the removal is easy
Discussion:Distraction in Crouzon syndrome has been used for many years. It allows important advances. Maxillary advancement is necessary because obstructive sleep apnea syndrome is common in Crouzon. Preoperatively polysomnography justifies the surgery

Results:seven children with Crouzon disease benefited from this technique between 2015 and 2022, the average age was 12 years, they all had sleep apnea syndrome, of varying intensity. All were in need of surgery due to unsightly facial appearance, complaining of facial retrusion and exophtalmos.The advance obtained corrects exorbitism, with its aesthetic and corneal benefit. The dental bite has been stable over time for 5 of them, two are still in orthodontic treatment, none has been reoperated since the intermediate osteotomy .They all improved on a respiratory level and on aesthetic appearance

Conclusion: The intermediate osteotomy is like a Lefort 3 osteotomy respecting the nose. The distraction with cranial support allows a very important advance stable over time. It corrects dental bite, eliminate exophthalmos and treat sleep apnea syndrome. It changes the nose aspect without rhinoplasty pushing the tip of the nose upward

Lymphatic malformations (LM) are rare congenital vascular anomalies, with the axilla being the second most common site after the cervicofacial region. They may present as well-circumscribed or diffuse lesions infiltrating adjacent tissues. Treatment ranges from surgical excision to systemic or interventional therapy, largely depending on lesion extent and anatomical involvement. Careful pretherapeutic assessment is crucial to guide management and improve long-term outcomes. This study evaluates the impact of localized versus diffuse axillary LM on therapeutic decision-making and clinical results.

We retrospectively reviewed patients diagnosed with axillary LM between 2010 and 2024. Data collected included age at the initial presentation, lesion size and extent, diagnostic modalities, treatments techniques, complications, recurrence up to the last follow up. Descriptive statistical analysis was applied.

Twenty-three patients (12 males, 11 females; median age 2.9 years, range 0–23) were included. All patients underwent ultrasound (US); five (22%) required no further imaging. In this group, median LM volume was 8 ml (range 3–11); four underwent surgery and one received no treatment. Eighteen patients (78%) underwent MRI (median age 2 years, range 0.5–23) with a median LM volume of 77 ml (range 10–1035). Fifteen of these patients had localized LMs and were treated surgically. Six had large lesions extending to the cervical (n=2) or upper arm (n=4) regions. . One patient was unsuccessfully treated with OK-432 sclerotherapy prior to successful surgical resection. Median follow-up time was 8 years (range 2–14). Two small asymptomatic recurrences (2 ml, 3 ml) occurred at 6 and 9 years without need for intervention. No neurovascular injury occurred despite extensive dissection. In three patients diffuse LM were founded, involving axilla, thorax, upper arm, neck and other areas of body. All these patients received Sirolimus for their treatment, but 2 of them also needed partial resections of their LM.

Surgical removal of localized axillary LM is associated with low morbidity, low recurrence, and excellent functional and aesthetic outcomes. Surgery is safe for both superficial and deep extensions of axillary LM. Diffuse axillary LMs are primarily managed with sirolimus, with partial resection reserved for complications.

Epidermolysis bullosa (EB) comprises rare inherited mechanobullous disorders. The recessive dystrophic form (RDEB) is caused by deficiency or absence of type VII collagen at the dermo-epidermal junction, resulting in extreme skin fragility. Patients develop chronic blistering, scarring, pseudosyndactyly and “cocoon hands” with complete digital fusion. Mucosal involvement and increased risk of squamous cell carcinoma (SCC) add to disease burden. Management is multidisciplinary and aims to preserve function and quality of life. We report our surgical strategy for hand and foot skin replacement in patients with RDEB and one case of junctional EB (JEB) focusing on outcomes of different wound-coverage techniques.

Between 01/2005 and 11/2025, 7 EB patients were treated surgically (6 RDEB, 1 JEB; 4 female). Median age at first surgery was 12.3 years (range 3.5–48.1). The JEB patient underwent commissural release of the feet; the remaining had hand surgery. Procedures included web space release with coverage by local flaps, biological skin substitutes or secondary intention healing. Postoperative care consisted of sodium hyaluronate cream, silicone interface, foam dressing and custom neoprene splints with interdigital straps. Dressings were changed weekly, initially under general anesthesia, occupational therapy provided splint adjustment and functional training.

In total, 31 procedures were performed using mixed coverage techniques, allowing intra-patient comparison. Two defects healed by secondary intention, 4 were covered with local flaps, 9 with living cellular skin substitute Apligraf® and 13 with Kerecis Omega-3®, a non-cellular biologically active fish-skin–derived matrix. Wounds treated with Kerecis® healed within 2 weeks and showed no postoperative complications, whereas one infection occurred in the Apligraf® group. Children resumed daily activities within 4 weeks. Revision surgery was necessary in pediatric patients due to growth and disease progression; the earliest at 20 months postoperatively in the Kerecis group. One patient developed aggressive SCC requiring hand amputation despite repeated excisions.

Surgical management of RDEB and JEB remains demanding and requires careful patient selection and coordinated multidisciplinary care. Coverage with Kerecis Omega-3® after web space release resulted in reliable healing and favorable functional outcomes.