Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence).

BACKGROUND: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. MAIN BODY: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. CONCLUSION: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.

The Impact of Preoperative Negative-Pressure Wound Therapy on Pectoralis Major Muscle Flap Reconstruction for Deep Sternal Wound Infections.

BACKGROUND: Deep sternal wound infection (DSWI) represents a life-threatening complication following open-heart surgery and pectoralis major muscle flap reconstruction has led to a significant reduction in mortality and morbidity. Negative-pressure wound therapy represented a step forward in DSWI treatment, both as a single procedure or as a preparation for reconstructive surgery.In the present study, we report our 13 years' experience with sternal reconstruction in order to evaluate the impact of preoperative vacuum-assisted closure (VAC) therapy on reconstructive outcome. METHODS: Seventy-three patients diagnosed with DSWI undergoing pectoralis major muscle flap reconstruction were divided into 2 subgroups: preoperative VAC treatment group (n = 37) and no preoperative VAC (NVAC n = 36). We collected patients' DSWI and reconstructive surgery clinical data, and we analyzed surgical outcome in terms of complication rate, reoperation rate, defects closure times, and intraoperative/30-day and 1-year mortality. RESULTS: Eighty-three flaps were used, bilateral flaps were used more in the NVAC subgroup (P = 0.005), and operative time was significantly shorter in the VAC subgroup (P < 0.001). Complication rate was 9.6%, with no significant differences between the 2 subgroups (P = 0.723). There was no recurrence of mediastinitis, and all flaps survived. Sternal closure time was significantly lower in the VAC subgroup (P < 0.001). No intraoperative death occurred; 30-day and 1-year mortality were 2.7% and 19.2%, respectively, with no significant difference between the 2 groups (P = 0.596). CONCLUSIONS: Preoperative VAC therapy makes reconstructive surgery easier and faster, even though it has no impact on complication rate and overall success of the reconstruction. Pectoralis major muscle flap represents a reliable solution even if not associated with preoperative VAC.

MRI anatomical preoperative evaluation of distally based peroneus brevis muscle flap in reconstructive surgery of the lower limb.

BACKGROUND: The distally based peroneus brevis muscle flap has proved to be a simple solution for small- to moderate-sized wounds of the lower limb. The length of the muscle belly suitable for coverage is a crucial parameter. In this study, we evaluated the capability of 3D MRI of the lower limb to measure it preoperatively. METHODS: Between 2008 and 2017, 32 patients with lower limb defects underwent preoperative MRI to measure the peroneus brevis muscle length. All patients underwent reconstruction, and the muscle was measured again intraoperatively during surgical dissection. Surgical measurements were then compared to the MRI ones. RESULTS: MRI measures of the peroneus brevis muscle belly ranged from 9 to 21 cm (µ = 14.44 ± 3.43 cm), and intraoperative measures ranged from 9 to 20 cm (µ = 14.2 ± 2.3 cm). Thirty of 32 intraoperative measures corresponded to the MRI ones (variation = ± 1 cm, r = 0.92, p = 0.002). One patient showed an intraoperative muscle length 3 cm shorter than the MRI measure, and another patient had intraoperative muscle length 3 cm longer than the MRI one. All flaps survived, and no secondary local flap coverage was required, with no flap-related complication, limited donor site morbidity, and acceptable patient discomfort. CONCLUSIONS: The reverse peroneus brevis muscle flap is a versatile alternative to free flap reconstruction in small- to moderate-sized defects of the lower limb. Preoperative 3D MRI is accurate to evaluate the anatomy of the muscle when performed by an expert radiologist. In our experience, it should become part of preoperative workup before performing a peroneus brevis flap procedure.

Sentinel Node Mapping in Melanoma of the Back: SPECT/CT Helps Discriminate "True" and "False" in-Transit Lymph Nodes.

A 32-year-old man with melanoma on the right paramedian region of the lower back underwent lymphoscintigraphy for radioguided sentinel node (SN) biopsy. Planar imaging showed the presence of 2 sites of radioactivity accumulation corresponding to an axillary SN and to an "in-transit" SN, located on the right side of the upper trunk. A further "hot spot" placed on the left paramedian region of the lower back was identified by planar lymphoscintigraphy. This last finding could be mistaken for another "in-transit" SN, but SPECT/CT demonstrated it was actually a nonspecific radiopharmaceutical accumulation at the level of the right renal pelvis.

Intraoperative hemodynamic evaluation of the latissimus dorsi muscle flap: a prospective study.

The aim of this study was to assess intraoperatively the hemodynamic changes in the donor vessel of free latissimus dorsi (LD) flap before and after denervation and to analyze flow changes after flap transfer. Twenty-seven patients underwent LD muscle microvascular reconstruction for lower-limb soft tissue defects. Measurements of blood flow were performed intraoperatively by using a 2- to 5-mm probe ultrasonic transit-time flowmeter around the dissected vessels. Registrations were made in the thoracodorsal artery before and after harvesting the flap, after compressing and cutting the motor nerve, and after anastomosis. Mean blood flow of in situ harvested thoracodorsal artery as measured intraoperatively by transit-time flowmeter was (mean ± standard deviation) 16.6 ± 11 mL/min and was significantly increased after raising the flap to 24.0 ± 22 mL/min (p <0.05); it was 25.6 ± 23 mL/min after compressing the motor nerve and was significantly increased after cutting the motor nerve to 32.5 ± 26 mL/min (p <0.05). A significant increase of blood flow to 28.1 ± 19 mL/min was also detected in the thoracodorsal artery after flap transplantation with end-to-side anastomosis (p <0.05). Vascular resistance in the thoracodorsal artery significantly decreased after flap raising and anastomosis (from 7.5 ± 3.4 to 4.0 ± 1.9 and to 4.5 ± 2.4, respectively, p <0.05). LD flap harvesting increases blood flow and decreases resistance in the thoracodorsal artery, especially after denervation.

Periorbital necrotising fasciitis.

Necrotising fasciitis involving the periorbita is a devastating infection. Potential outcomes range from severe disfigurement, loss of the eye and even to death. Early recognition is critical, although its initially non-distinctive appearance frequently delays diagnosis and treatment. Herein, the authors have performed a systematic review of previously published cases including clinical features, diagnoses and differential diagnoses, pathological characteristics and management. Periorbital necrotising fasciitis is seen mainly in adults with a female predominance (54%); about one-half (47%) of the patients were previously healthy. The infection can follow local blunt trauma (17%), penetrating injuries (22%) and face surgery (11%), but in about one-third of cases (28%) no cause was identified. Non-specific erythema and localised painful swelling of the eyelids characterise the earliest manifestation of the disease, followed by formation of blisters and necrosis of the periorbital skin and subcutaneous tissues. The causative organism in periorbital infection was mainly ß-haemolytic Streptococcus alone (50%), occasionally in combination with Staphylococcus aureus (18%). The overall mortality rate was 14.42%. The main risk factor for mortality was the type of causative organism, since all reported cases of death were caused by ß-haemolytic Streptococcus alone or associated with other organisms. Unlike necrotising fasciitis affecting other body sites, there was not a strong correlation with age >50 years or the presence of associated chronic illness. Management of periorbital necrotising fasciitis is then based on early distinction of symptoms and signs and aggressive multidisciplinary treatment. Thus, the delay between initial debridement and initiating parenteral broad-spectrum antibiotic therapy should be considered the most critical factor influencing morbidity and mortality.

Intraoperative hemodynamic evaluation of the radial and ulnar arteries during free radial forearm flap procedure.

The purpose of this prospective study was to assess the blood flow of the radial and ulnar arteries before and after radial forearm flap raising. Twenty-two patients underwent radial forearm microvascular reconstruction for leg soft tissue defects. Blood flow of the radial, ulnar, and recipient arteries was measured intraoperatively by transit-time and ultrasonic flowmeter. In the in situ radial artery, the mean blood flow was 60.5 +/- 47.7 mL/min before, 6.7 +/- 4.1 mL/min after raising the flap, and 5.8 +/- 2.0 mL/min after end-to-end anastomosis to the recipient artery. In the ulnar artery, the mean blood flow was 60.5 +/- 43.3 mL/min before harvesting the radial forearm flap and significantly increased to 85.7 +/- 57.9 mL/min after radial artery sacrifice. A significant difference was also found between this value and the value of blood flow in the ulnar and radial arteries pooled together ( P < 0.05). The vascular resistance in the ulnar artery decreased significantly after the radial artery flap raising (from 2.7 +/- 3.1 to 1.9 +/- 2.2 peripheral resistance units, P = 0.010). The forearm has a conspicuous arterial vascularization not only through the radial and ulnar arteries but also through the interosseous system. The raising of the radial forearm flap increases blood flow and decreases vascular resistance in the ulnar artery.

Distally based peroneus brevis muscle flap in reconstructive surgery of the lower leg: Postoperative ankle function and stability evaluation.

BACKGROUND: Defects of the distal third of the lower leg with exposed tendons or bone require either local or free flap coverage. Several flaps have been developed, and the distally pedicled peroneus brevis muscle flap has been proven to be a valid local flap alternative. PATIENTS AND METHODS: Peroneus brevis muscle is dissected from the lateral surface of the fibula from proximal to distal, but no further than approximately 3-6 cm proximally to the lateral malleolus, where the most distal vascular pedicle from the peroneal artery enters the muscle consistently. This allows the muscle to be transposed to more distal lesions. The muscle is then covered with meshed split-skin graft. Between 2002 and 2008, 10 patients with defects of the lower leg in the distal lower third have been treated using this muscle flap. The defects were located over the Achilles tendon, the medial and lateral malleolar, the anterior region of the ankle and the heel area. Each patient was examined through assigning clinical demerit points according to a modified Weber scale, and through Olerud-Molander ankle score to analyse postoperative ankle stability and functionality after reverse peroneus brevis flap reconstruction of the defect. RESULTS: All flaps survived completely, and no secondary local flap was required. In our experience, no complication or patient discomfort was noted. Donor-site morbidity was acceptable and restricted to the scar in the lateral lower leg. As demonstrated by the two score evaluations, the functions of foot eversion and plantar flexion as well as ankle functionality and stability were maintained due to preservation of peroneus longus muscle. CONCLUSION: The reverse peroneus muscle flap is ideally suited for small-to-moderate defects of the distal third of the lower leg. This flap offers a convincing alternative for covering defects in the distal leg region. Its arc of rotation allows coverage of more anterior defects of the ankle, of defects of the Achilles tendon and of the heel area as well as of lateral and medial malleolus areas. It is simple to raise and is often transposed easily within the wound without further dissection. As long as the peroneus longus is preserved, ankle instability is not expected.

Dystrophic-like alterations characterize orbicularis oris and palatopharyngeal muscles in patients affected by cleft lip and palate.

OBJECTIVE: This study analyzed histological and histochemical features of specimens of the orbicularis oris muscle, and palatal and pharyngeal muscles biopsied during surgery from 33 patients affected by cleft lip and palate. DESIGN: Three groups were studied: 20 patients affected by cleft palate with or without cleft lip (at the time of primary palatoplasty), seven by cleft lip with or without cleft palate (primary lip closure), and six by cleft lip with or without cleft palate (secondary cheiloplasty). Muscle sections were stained with hematoxylin-eosin, modified Gomori trichrome, ATPase reaction at pH 9.4, and NADH-TR. Analyzed parameters included organization, muscle fiber size and type, nuclear changes, presence of ragged-red fibers, degree of fibrosis, and presence of inflammatory infiltrate. RESULTS: In all patients who underwent primary palatoplasty and lip closure we noted dystrophic-like alterations of orbicularis oris and palatopharyngeal muscles, such as variability of fiber size, fiber disorganization, and increased fibrosis. The same alterations were found in adult patients submitted to secondary cheiloplasty, notwithstanding surgical repair. Furthermore, in all groups neither neurogenic atrophy nor ragged-red fibers or inflammatory infiltrate were detected. CONCLUSIONS: Muscle damage is a constant event in this deformity, and it could play an important role in its etiopathogenesis. Muscular biopsy during cheiloplasty and palatoplasty could offer useful information about muscle condition and possible functional recovery in cleft lip and palate patients.

Long-term morphometric and immunohistochemical findings in human free microvascular muscle flaps.

Reinnervation, muscle regeneration, density of microvessels, and muscle-type specific atrophy were studied 3-4 years after surgery in surgically nonreinnervated free microvascular muscle flaps to 13 patients transplanted to the upper or lower extremities. Routine histology and immunohistochemistry for PGP 9.5 and S-100 (neuronal markers), Ki-67 (cell proliferation), myosin (muscle fiber types), and CD-31 (endothelium) were carried out, and results were analyzed morphometrically. Three to 4 years after surgery, severe atrophy of predominantly slow-type fibers was seen in 9 cases. In 4 cases, muscle-fiber diameter and fiber-type distribution were close to normal. Long intraoperative muscle ischemia and postoperative immobilization were associated with poor muscle bulk in flaps. The density of microvessels in flaps did not differ from control muscles. PGP 9.5 and S-100 immunopositive nerve fibers were detected in 7 patients. Reinnervation was associated with good muscle bulk. In 4 patients, activation of satellite cells was evident. The results suggest that in some cases, spontaneous reinnervation may occur in free muscle flaps, and that several years after microvascular free flap transfer, the muscle still attempts to regenerate.

Colour Doppler ultrasound evaluation of haemodynamic changes in free tram flaps and their donor sites.

Colour Doppler ultrasound (US) was used to measure the blood flow in the donor and recipient arteries as well as in the deep superior epigastric artery of 10 patients having free transverse rectus abdominis myocutaneous (TRAM) flaps. The peak, minimum and mean velocities, the diameter of the vessel, and the resistance index of both the deep superior and inferior epigastric arteries and thoracodorsal arteries were recorded preoperatively and at 4-6 and 15-30 days postoperatively. Colour Doppler US showed increased minimum velocity and decreased resistance index in the pedicle (p < 0.05) throughout the follow-up when compared with the baseline. In the ipsilateral superior epigastric artery the mean and minimum velocities increased (p < 0.05) while the resistance index decreased (p < 0.05) during the first month postoperatively. No changes were recorded in the opposite epigastric arteries or in the control vessel (opposite thoracodorsal artery). In all patients the diameter of the deep inferior epigastric artery was larger than that of the superior epigastric and remained so after the transfer. From the fourth to the thirtieth postoperative day blood flow increased in the free TRAM flap, presumably because of decreased vascular resistance. Blood flow also increased in the superior epigastic artery on the donor side after free TRAM transfer as expected (indicating the delay phenomenon), but harvesting the flap did not affect the circulation in the opposite rectus abdominis muscle. The inferior epigastric arterial system was dominant in all patients.