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Background Infantile haemangiomas (IHs) are benign vascular tumours that affect up to 10% of infants and arise in the first few weeks-to-months of life. Some are associated with an increased risk of complications and poor cosmetic outcomes. General practitioners (GPs) are ideally placed to identify high-risk IHs and coordinate their management. Objective The aim of this article is to outline strategies to identify high-risk IHs and when to involve a multidisciplinary team. Discussion IHs that involve the lower face or neck can be associated with airway obstruction. Having five or more IHs is associated with hepatic haemangiomas, high-output cardiac failure and hypothyroidism. IHs that involve the eyes or mouth can cause functional impairments such as amblyopia and feeding difficulties, respectively. Large segmental IHs maybe associated with syndromic presentations including PHACE syndrome when on the lower face, PELVIS syndrome when on the perineum and LUMBAR syndrome when on the lower back.

Aim Pelvic exenteration is the only surgical option for locally advanced pelvic malignancies infiltrating the surrounding organs. The resultant pelvic void after the procedure is responsible for a number of complications, collectively termed empty pelvis syndrome (EPS). We aim to show how EPS can be minimized by presenting a case series demonstrating the surgical technique of laparoscopic total pelvic exenteration with bilateral pelvic node dissection along with a novel use of the Bakri balloon. Method This is a case series of three successive patients undergoing laparoscopic total pelvic exenteration for locally advanced primary, nonmetastatic rectal adenocarcinoma over a period of 1 month in a specialized colorectal unit at a tertiary cancer centre. The Bakri balloon was deployed in all three patients and retained for variable time intervals postoperatively. Features of EPS were prospectively documented. Results In the first patient, the Bakri balloon was completely deflated and removed on postoperative day (POD) 5. The patient developed subacute intestinal obstruction which resolved with conservative management by POD 12. In the second and third patients, the Bakri balloon was deflated in a sequential manner, beginning on POD 8, until it was finally removed on POD 11. Neither of these patients had any abdominal complaints. A postoperative CT scan of both these patients showed the small bowel loops clearly above the pelvic inlet. Conclusions The Bakri balloon is a simple, safe and cost-effective method to reduce the complications of EPS following laparoscopic total pelvic exenteration. A prospective study is ongoing to objectively quantify the benefits of this technique.

AIM Empty pelvis syndrome is a major contributor to morbidity following pelvic exenteration. Several techniques for filling the pelvis have been proposed; however, there is no consensus on the best approach. We evaluated and compared the complications associated with each reconstruction technique with the aim of determining which is associated with the lowest incidence of complications related to the empty pelvis. METHOD The systematic review protocol was prospectively registered with PROSPERO (CRD42021239307). PRISMA-P guidelines were used to present the literature. PubMed and MEDLINE were systematically searched up to 1 February 2021. A dataset containing predetermined primary and secondary outcomes was extracted. RESULTS Eighteen studies fulfilled our criteria; these included 375 patients with mainly rectal and gynaecological cancer. Only three studies had a follow-up greater than 2 years. Six surgical interventions were identified. Mesh reconstruction and breast prosthesis were associated with low rates of small bowel obstruction (SBO), entero-cutaneous fistulas and perineal hernia. Findings for myocutaneous flaps were similar; however, they were associated with high rates of perineal wound complications. Omentoplasty was found to have a high perineal wound infection rate (40%). Obstetric balloons were found to have the highest rates of perineal wound dehiscence and SBO. Silicone expanders effectively kept small bowel out of the pelvis, although rates of pelvic collections remained high (20%). CONCLUSION The morbidity associated with an empty pelvis remains considerable. Given the low quality of the evidence with small patient numbers, strong conclusions in favour of a certain technique and comparison of these interventions remains challenging.

Objective − PELVIS syndrome is an association between large perineal hemangiomas and congenital anomalies, including anorectal, urinary tract, spine, and external genitalia malformations. We present a 7-year-old boy with PELVIS syndrome. Case report − . The boy had been monitored since early intrauterine age by ultrasound (US) due to left kidney cysts. Clinical examination at birth found large segmental hemangiomas in the perineal, gluteal region, and the left leg. At the age of 4 weeks US showed a multicystic, dysplastic left kidney with compensatory hypertrophy of the right kidney. Voiding cystography (VCUG) did not show vesicoureteral reflux and morphology of the bladder, and the urethra was normal. PELVIS syndrome was suspected and the child underwent further diagnostic tests which showed: undescended left testis with a hypoplastic left scrotum and micropenis, right-sided angulation of the sacrum and closed spinal dysraphism from S1 to S5, with lipoma of the filum terminale, without tethering. The urodynamics investigation was normal. During the seven-year follow-up there was spontaneous regression of the left kidney cysts; the growth and function of the right kidney was normal, as was also sphincter control. There was significant spontaneous regression of the hemangiomas. Follow-up magnetic resonance imaging (MRI) of the lumbosacral spine showed no progression of the lipoma. Conclusion − Large perineal hemangiomas should be recognized as indicators of underlying congenital anomalies, including urogenital, anorectal and spinal. Therefore, additional diagnostic evaluation, including kidney ultrasound and spine imaging, are necessary.

Aim Empty pelvis syndrome and radiation-induced bowel injury are two major clinical issues resulting from the pelvic dead space after pelvic exenteration (PE). In order to avoid these complications, different methods of pelvic floor reconstruction have been proposed. We report our experience on the use of breast prosthesis. Method Fifty-three patients who underwent PE and three who underwent palliative surgery with silicone breast prosthesis placement were included. Results Forty-seven posterior PE, six total PE and three palliative procedures were identified. Sphincter preservation was feasible in 34 patients (62.3%). There were no deaths. Overall morbidity was 37.5%. There were no complications such as sepsis or obstruction related to the prosthesis. Adjuvant radiotherapy was delivered in 16 cases (30.1%) without any side-effects. Reconstruction of intestinal continuity was possible in 12 patients (36.3%) with sphincter preservation and the prosthesis allowed a prompt identification of the rectal stump. Conclusion Breast prosthesis placement is a simple and safe method to minimize complications resulting from empty pelvis syndrome and can be adopted to exclude bowel loops from the radiation field. Reconstruction of intestinal continuity after resection is also simplified.

This review outlines the role of pelvic exenteration (PE) in the management of certain locally-advanced primary and recurrent rectal cancers. PE has undergone significant evolution over the past decades. Advances in pre-, intra-, and post-operative care have been directed towards achieving the 'holy grail' of an R0 resection, which remains the most important predictor of survival, quality of life, morbidity, and cost effectiveness following PE. Patient selection for surgery is largely determined by assessment of resectability. Pelvic magnetic resonance imaging determines the extent of local disease, while positron emission tomography remains the most accurate tool for exclusion of distant metastases. PE in the setting of metastatic disease or with palliative intent remains controversial. The intra-operative approach is based on the anatomical division of the pelvis into five compartments (anterior, central, posterior, and two lateral). Within each compartment are various possible dissection planes which are elected depending on the extent of tumour involvement. Innovations in surgical technique have allowed 'higher and wider' dissection planes with resultant en bloc excision of major vessels, major nerves, and bone. Evidence of improved R0 resection and survival rates with these techniques justifies the radicality of these novel approaches. Post-operative care for PE patients is technically demanding with a substantial hospital resource burden. Unique considerations for PE patients include the 'empty pelvis syndrome', urological complications, and management of post-operative malnutrition. While undeniably a morbid procedure, quality of life largely returns to baseline at six months, and for long-term survivors is sustained for up to five years.

Background/objective The pathogenesis of infantile hemangiomas (IH), PHACE, and LUMBAR syndromes remains unknown. We aim to describe histopathologic features of midline anomalies associated with IH, including patients with PHACE and LUMBAR syndromes. Methods A multicenter retrospective chart review was performed to identify patients with IH, PHACE, and LUMBAR syndrome with histopathologic specimens from sternal or midline anomalies. A total of 18 midline lesions from 13 patients were included. Out of 18, 14 midline lesions underwent both histopathologic and clinical review. Three hamartoma-like chin plaques and one supraumbilical raphe underwent only clinical review. Results All 13 patients had midline lesions and IH. Histopathologic diagnoses were as follows: rhabdomyomatous mesenchymal hamartoma (3), folliculosebaceous cystic hamartoma (1), fibroepithelial polyp (1), verrucous epidermal hyperplasia with vascular proliferation and fibroplasia (1), congenital midline cervical cleft (1), pericardium with fibrosis (1), fibrous components with increased collagen (1), atrophic skin/membrane (3), angiolipomatous mass with neural components (1), and lipomatous mass (1). Due to the retrospective nature of this study, it was not possible to obtain pathology slides for all midline lesions that had previously been biopsied or resected. We show clinically and histopathologically a new association between PHACE syndrome and rhabdomyomatous mesenchymal hamartoma (RMH), in addition to demonstrating the association between PHACE syndrome and chin hamartomas. We also display histopathologic findings seen in midline lesions resected from LUMBAR patients. Conclusion Rhabdomyomatous mesenchymal hamartoma is thought to be related to aberrations of mesenchymal cells during development; therefore, this may provide clues to the pathogenesis of IH and related syndromes.

Two cases of infantile perianal pyramidal protrusions (IPPP), one pyramidal in shape and one leaf shaped, are being described by us. Both were notable for coexisting hemangiomas in the close vicinity. To the best of our knowledge we are reporting these two variants of IPPP with the associated neighboring hemangiomas for the first time in Indian dermatologic literature. We suggest that this presentation may be a part of one of the syndromes that comprise anorectal malformations with hemangiomas like PELVIS syndrome and others mentioned in the table.

Objective To describe the technique and report experiences with pelvic floor reconstruction by modified rectus abdominis myoperitoneal (MRAM) flap after extensive pelvic procedures. Methods Surgical technique of MRAM harvest and transposition is carefully described. The patients in whom pelvic floor reconstruction with MRAM after either infralevator pelvic exenteration and/or extended lateral pelvic sidewall excision was carried out were enrolled into the study (MRAM group, n =16). Surgical data, post-operative morbidity, and disease status were retrospectively assessed. The results were compared with a historical cohort of patients, in whom an exenterative procedure without pelvic floor reconstruction was performed at the same institution (control group, n =24). Results Both groups were balanced in age, BMI, tumor types, and previous treatment. Substantially less patients from the MRAM group required reoperation within 60days of the surgery (25% vs. 50%) which was due to much lower rate of complications potentially related to empty pelvis syndrome (1 vs. 7 reoperations) ( p =0.114). Late post-operative complication rate was substantially lower in the MRAM group (any grade: 79% vs. 44%; grade≥3: 37% vs. 6%) ( p =0.041). The performance status 6months after the surgery was ≤1 in the majority of patients in MRAM (81%) while in only 38% of patients from the control group ( p =0.027). There was one incisional hernia in MRAM group while three cases were reported in the controls. Conclusions Pelvic floor reconstruction by MRAM in patients after pelvic exenterative procedures is associated with a substantial decrease in postoperative complications that are potentially related to empty pelvis syndrome.

Resume Introduction Le « PELVIS » ou « SACRAL » syndrome designe l’association d’un hemangiome regional a un syndrome malformatif de la region pelvienne. Nous en rapportons un cas, remarquable par l’aspect initialement livedoide de l’hemangiome et l’association a une bride amniotique d’un membre superieur. Observation Un nouveau-ne de sexe masculin subissait le jour de sa naissance une colostomie gauche dans un contexte d’imperforation anale et d’anomalies des organes genitaux externes avec ambiguite sexuelle. L’examen du tegument notait une hypopigmentation mal limitee de la region lombosacree et un sillon fibreux circonferentiel du bras droit, caracteristique d’une bride amniotique. L’IRM lombosacree et pelvienne montrait une deviation vers la gauche des trois dernieres vertebres sacrees, sans anomalie medullaire. La cystographie retrograde mettait en evidence une fistule recto-uretrale. L’evolution etait marquee par l’apparition des le premier mois d’une lesion angiomateuse violacee, plane, livedoide, de topographie mediane sur les fesses et le perinee et lineaire sur la face posterieure du membre inferieur droit. La biopsie cutanee de cette lesion montrait un unique lobule capillaire a la jonction dermo-hypodermique, d’aspect non specifique mais dont l’intense expression de Glut1 par les cellules endotheliales etait tres en faveur d’un hemangiome infantile. Discussion Les hemangiomes segmentaires sont volontiers associes a des anomalies extra-cutanees. Par analogie au « PHACE syndrome », defini par l’association d’un hemangiome segmentaire facial a des anomalies cerebrales, oculaires et cardio-aortiques, le « PELVIS »/« SACRAL » syndrome decrit l’association d’un hemangiome segmentaire de la region des langes (region lombosacree, fessiere ou perineale = napkin hemangioma) avec une dysraphie touchant dans des proportions variables le rachis lombosacre, le cone medullaire terminal, les organes genito-urinaires et la region anale. L’hemangiome du PELVIS/SACRAL syndrome peut etre de diagnostic retarde ou difficile du fait d’un caractere volontiers maculeux, telangiectasique ou livedoide. A notre connaissance, l’association d’une bride amniotique a un hemangiome ou a une dysraphie perineale n’avait pas ete decrite jusqu’ici.

Background PELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction. Case presentation Female infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring. Conclusions Large perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment.

While localized infantile hemangiomas (IHs) are often benign, except when they are located near a noble structure such as the airways or the orbital area, segmental IHs may be associated with birth defects. These cases should be very carefully studied for life-threatening conditions (heart failure, respiratory distress), functional risks (amblyopia, swallowing disorders, digestive and renal alterations, etc.), aesthetic risks (especially IHs of the face), and painful ulcerations. PHACE is an acronym indicating a disorder characterized by the association of posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and other cardiac defects, and eye abnormalities. Indeed the ISSVA’s classification separates clearly vascular tumors (the group that includes IHs) from vascular malformations, indicating, at the same time, that errors in angiogenesis or vasculogenesis that occur in embryonic phase may lead to stable lesions (i.e., vascular malformations) but, quite obviously, not to proliferative lesions (i.e., IHs). From this starting point, it was conceptually clear that vascular tumors and vascular malformations could not be present in the same individual, unless by chance. However, despite the fact that the etiopathogenesis of most vascular anomalies is still unknown, the description of many cases of PHACE syndrome and, in parallel, the description of LUMBAR/PELVIS/SACRAL syndrome in other patients, make likely that a pathogenic noxa can provoke either vascular tumors or vascular malformations in the same individual.

We report a case of PELVIS (perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag) syndrome in which hemangioma in the perineal area was misdiagnosed at birth as diaper rash. Investigations revealed associated vesicorenal and spinal abnormalities. We emphasize careful diagnosis of suspicious lesions at birth and confirm the successful use of propranolol in treating ulcerated segmental hemangiomas.

Infantile hemangiomas (IHs) belong to a family of lesions called vascular anomalies. Vascular anomalies are classified into either vascular tumors or vascular malformations, with the IH being the most common vascular tumor, affecting approximately 5% of infants (Frieden, Haggstrom et al 2005). Despite their prevalence, the origin and pathogenesis of IHs remains poorly understood. Clinically, IHs undergo a predictable course of rapid proliferation shortly after birth, followed by stabilization and involution throughout childhood. Occasionally, a fibrofatty residuum results after involution is complete. Despite its predictable clinical course, the regulatory mechanisms throughout different phases are only recently being elucidated (Frieden, Haggstrom et al. 2005). During the proliferative phase, IH can cause serious morbidity and even mortality. Rapidly proliferating hemangiomas can be dangerous as they have potential to ulcerate and bleed. While disorganized, IHs are high-flow lesions; occasionally, life-threatening bleeding can occur. The location of the IH can also be detrimental. Hemangiomas in the peri-orbital area can cause obstructive amblyopia and astigmatism, and airway hemangiomas can cause stridor and respiratory distress. Visceral hemangiomas of the liver can cause congestive heart failure, hepatomegaly, and anemia; the mortality rate with treatment is significant, up to 30% (Arneja and Mulliken, 2010; Bitar et al., 2005; Boon et al., 1996; Ceisler & Blei, 2003; Chamlin et al., 2007; Haggstrom et al., 2006a; Schwartz et al., 2006). In recent years, PHACE syndrome has been described and characterized. PHACE syndrome comprises a constellation of findings including Posterior fossa anomalies, large facial Hemangioma, Arterial anomalies, Cardiac abnormalities/aortic Coarctation, and Eye anomalies (Frieden et al., 1996). Infants with PHACE syndrome are at increased risk for strokes, neurological and cardiac consequences (Burrows et al., 1998; Drolet et al., 2006). Another syndrome featuring a large hemangioma over an area with aberrant underlying anatomical structures have also been described. PELVIS syndrome describes Perineal hemangioma, External genitalia malformations, Lipomyelomeningocele, Vesicorenal anomalies, Imperforate anus, and Skin tag (Girard et al., 2006). PHACE and PELVIS syndromes suggest that there may be an association between a cutaneous hemangioma and

(2004). Empty pelvis syndrome. Journal of Obstetrics and Gynaecology: Vol. 24, No. 6, pp. 714-715.