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A dermal sinus/fistula is a common condition; the relevant department should be consulted for appropriate treatment. It is important for radiologists to have adequate knowledge of these conditions to provide the correct diagnosis and recommend subsequent management. This review describes the following lesions: preauricular sinus, midline sinus of the upper lip, nasal dermoid sinus cyst, cheek fistula, first branchial cleft anomaly/sublingual branchial cleft anomaly, thyroglossal duct cyst/fistula, lateral cervical sinus/fistula, congenital dermal sinus/fistula of the anterior chest region, congenital skin sinus/fistula with a sternal cleft, and congenital prepubic sinus. On the basis of the skin orifice location and ultrasound images, radiologists can provide useful information to physicians., (© 2019 by the American Institute of Ultrasound in Medicine.)

Background: Congenital prepubic sinus is a rare anomaly found in the midline of the lower abdomen. Congenital prepubic sinus is usually asymptomatic in neonates, and a diagnosis is often achieved later in life after spontaneous extrusion of purulent material from the pre-existing hole in the pubic region. We present a case of congenital prepubic sinus presenting with purulent discharge after circumcision. A 4-year-old Caucasian boy presented to our urology out-patient clinic with purulent discharge from the distal part of the dorsum of his penis. He had a history of circumcision performed at a different center, 6 months ago. His parents stated that although various antibiotics were used, the purulent discharge continued for 6 months and the child had no complaints before circumcision. His condition was reported as superficial dorsal venous thrombosis, known as penile Mondor disease, in magnetic resonance imaging that was performed in the previous hospital. A physical examination revealed a small pinhole lesion at the distal part of his penis and a rigid cylindrical tube extending to the proximal side of his penis. We performed fistulography by injecting contrast material through a small angiocatheter and confirmed the diagnosis of prepubic sinus. Surgical exploration was performed and a long sinus, apparently ending as a fibrous tract at the anterior surface of his pubic symphysis, was found and resected., Conclusions: Before congenital prepubic sinus surgery, it is critically important to rule out penile Mondor disease and the possibility of a circumcision complication (especially infective complications) mimicking congenital prepubic sinus.

Cervical congenital malformations are relatively common in children. They can also be found in adults. The embryological development of the cervical region is closely related to the branchial clefts. This must be a diagnosis made by elimination; a cervical tumor must evoke the diagnosis of cancer. A cutaneous fistula or a cervical tumor, chronic or recent appearance in an inflammatory context, are the clinical signs. The thyroglossal duct cysts and the second branchial clefts cysts are the most common causes of median and lateral cervical cysts, respectively. Imaging contributes greatly to the orientation and diagnostic evaluation of the extent of the lesions. Treatment is initially based on antibiotic therapy and then on complete surgical excision, away from an infectious episode, the sole guarantee for the absence of local recurrence., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Congenital anterior urethrocutaneous fistula (CAUF) is a rare anomaly characterized by fistulization of penile urethra to skin. It's usually seen as an isolated deformity or may accompany genitourinary or anorectal malformations. We aim to define the common properties of patients mentioned in literatures by systematic review. A comprehensive search of PubMed, Embase, Web of Science, and Cochrane Library was performed including cross-referencing independently by two assessors. Selections were restricted to human studies in English. Based on the systematic review, 63 patients in 34 articles were included in the study. Most common fistula site was subcoronal in 29 (46.0%) patients. Chordee was in 8 (14.5%) and associated genitourinary anomaly was detected in 19 (30.2%) of patients. Fistula recurrence ratio was 6/59 (11.3%) using different surgical techniques and 3/6 was closed spontaneously. CAUF is frequently located in subcoronal level and usually an intact urethra distal to it. Success rates are high with the principles of hypospadias surgery., Competing Interests: None

Objective: To investigate the relationship between Work Ⅱ type of congenital first branchial cleft anomaly (CFBCA) and facial nerve and discuss surgical strategies. Methods: Retrospective analysis of 37 patients with CFBCA who were treated from May 2005 to September 2016. Among 37 cases with CFBCA, 12 males and 25 females; 24 in the left and 13 in the right; the age at diagnosis was from 1 to 76 ( years, with a median age of 20, 24 cases with age of 18 years or less and 13 with age more than 18 years; duration of disease ranged from 1 to 10 years (median of 6 years); 4 cases were recurren after fistula resection. According to the classification of Olsen, all 37 cases were non-cyst (sinus or fistula). External fistula located over the mandibular angle in 28 (75.7%) cases and below the angle in 9 (24.3%) cases. Results: Surgeries were performed successfully in all the 37 cases. It was found that lesions located at anterior of the facial nerve in 13 (35.1%) cases, coursed between the branches in 3 cases (8.1%), and lied in the deep of the facial nerve in 21 (56.8%) cases. CFBCA in female with external fistula below mandibular angle and membranous band was more likely to lie deep of the facial nerve than in male with external fistula over the mandibular angle but without myringeal web. Conclusions: CFBCA in female patients with a external fistula located below the mandibular angle, non-cyst of Olsen or a myringeal web is more likely to lie deep of the facial nerve. Surgeons should particularly take care of the protection of facial nerve in these patients, if necessary, facial nerve monitoring technology can be used during surgery to complete resection of lesions.

Congenital parotid fistulas are extremely rare diseases. Herein, the authors report a patient with congenital salivary fistula from parotid main gland in a 15-year-old boy. Therefore, clinicians should consider the possibility of congenital parotid fistula form parotid main gland. Surgical excision of the fistula and parotid gland can be effective treatment option of congenital parotid fistula.

Nasal dermoids are rare congenital abnormalities, and there is only one previously reported case of a philtrum sinus tract extending to the skull base. A 2-month-old boy was presented with an incidental finding of a median philtrum sinus with no infective features and no other abnormal clinical findings. MRI demonstrated a sinus tract extending from the philtrum to the crista galli. On multidisciplinary review, a conservative approach was taken due to the asymptomatic nature of the patient. Given the rarity of nasal dermoids, diagnosis requires precise clinical examination and MRI to identify the extent of the tract. Management options require a multidisciplinary case-specific approach and include non-surgical as well as surgical approaches., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

A 24-year-old man presented with symptoms of difficulty in passage of urine, mixed with gas and stool from penoscrotal junction since birth. He had a history of surgery (posterior sagittal anorectoplasty) for imperforate anus in first week of his life. On physical examination, external meatus was adequate with small 5×5 mm fistulous opening noted at penoscrotal junction with normal anal tone and opening. On evaluation with retrograde urethrography, voiding cystourethrogram, cystoscopy and barium enema, he was found to have penile urethrocutaneous fistula of 0.5×0.5 cm with Y-type incomplete urethral duplication. Urethroanal fistula (Y-type urethral duplication) was of small size (<5 mm), so closed primarily through perineal route with interposition of ischiorectal fat. Penile urethrocutaneous fistula (4 mm) too was closed primarily and covered with tunica vaginalis flap in single sitting. It has been 6 months since surgery and the patient is fully continent and voiding well with no urinary or faecal leak., (2017 BMJ Publishing Group Ltd.)

Objective: To report rare cases of congenital neck cutaneous sinus with an orifice near the sternoclavicular joint and to investigate their origins and managements. Methods: A total of ten patients with congenital neck cutaneous sinus having an orifice near the sternoclavicular joint treated in the Guangdong General Hospital from January 2010 to June 2015 were retrospectively analyzed. Results: There four boys and six girls, aging from 11 months to 96 months with an average of 33.4 months, and they had a common feature showing a congenital cutaneous sinus with an orifice near sternoclavicular joint. Discharge of pus from the orifice or abscess formation was commonly seen soon after infection. With bacteriological study, staphylococcus aureus was positive in five cases and klebsiella pneumonia in a case. Another orifice of fistula/sinus was not depicted in pyriform with barium swallow X-ray in five cases Ultrasound studies of three cases demonstrated anechoic (i.e., nearly black) and solid-cystic lesion near sternoclavicular joint with posterior acoustic enhancement. Magnetic resonance imaging (MRI) showed isointensity of the lesion on T1 and T2 weighted images with heterogeneous enhancement and a close relationship with sternoclavicular joint. All patients underwent laryngoscopic examination, which showed no orifice of sinus in pyriform at same side. Surgical resection of fistula/sinus was performed in all cases. The lengths of the fistula varied from 5 mm to 22 mm with an average of 11 mm. Postoperative pathological examination showed all specimens were accordance with fistula. No complications were noticed. Recurrence was not observed in the cases by following-up of 6 months to 70 months (median: 33 months). Conclusion: Congenital neck cutaneous sinus with orifice near the sternoclavicular joint maybe a special clinical phenotype of the fourth branchial cleft sinus with skin orifice in cervicothoracic junction. Differential diagnoses between low cervical diseases are required. The curative treatment is a complete excision during inflammatory quiescent period.

Objectives/hypothesis: Preauricular sinuses are benign congenital malformations of preauricular soft tissues. Complete excision using either sinectomy or supra-auricular approach is advised to prevent recurrence. Reported recurrence varies between 0 and 42%. We evaluated which surgical technique resulted in lowest complication and recurrence rates., Study Design: PubMed, Embase, Scopus, Web of Science., Methods: Two authors appraised studies on directness of evidence and risk of bias. Original data were extracted and pooled when I(2) was smaller than 50%. Results are reported according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement., Results: Fourteen high directness of evidence studies were included. Reported complication rates were similar: sinectomy [0-31.4%] and supra-auricular approach (SAA) [0-18.2%]. Pooled recurrence rates showed that sinectomy resulted in significantly (P = .04) more recurrence 5.5% (95% confidence interval [CI] 3.6-8.3%) than SAA 2.2% (95% CI 0.7-7.0). Sinectomy using the microscope resulted in the lowest sinectomy recurrence rates (1.9%). SAA in combination with a Penrose drain resulted in 0% recurrence in revision cases. Drain use resulted in the lowest SAA recurrence rates; however, drain application was not advised due to higher complication rates (frequent wound infection [P = .003] and more [P = .002] and longer [P = .001] compression dressing use)., Conclusion: SAA could be the preferable technique for preauricular sinus removal. If despite evidence, sinectomy is elected over SAA, microscope use can further decrease recurrence rates comparable to SAA levels. Level of included evidence (Ib-IV) indicates the need for a prospective study comparing surgical outcomes between techniques. Laryngoscope, 126:1535-1544, 2016., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Congenital anterior urethrocutaneous fistula, is a rare anomaly characterised by fistulisation of penile urethra to skin and presence of a concomitant normal or hypospadiac external urethral meatus. It may be seen as an isolated anomaly or may accompany genitourinary or anorectal malformations. We aim to present 3 new cases and define the common properties of patients stated in literature., Method: Information of 3 patients aged 2, 3 and 6 with this diagnosis were reviewed retrospectively and features of 51 patients in 25 articles with literature search., Results: From the patients we operated, 2 had midpenile and 1 had subcoronal fistula. Urethral meatus was at tip of glans in all with 1 stenotic meatus. Two-layered primary repair was performed in 3 patients and deep ventral incision on urethral plate with meatotomy were added to fistula repair in one with stenotic meatus. Fistula recurred in this patient but resolved spontaneously after dilatations. In literature, most common fistula site was subcoronal in 27 (52.9%). Hypospadias was in 11.8% and associated genitourinary anomaly was detected in 21.5% of patients. Fistula recurrence ratio was 7.8% using different surgical techniques., Conclusion: Congenital anterior urethrocutaneous fistula is frequently located in subcoronal level and usually a normal urethra distal to it. Physical examination is important to detect additional anomalies. Success rates are high with primary repair techniques.

Congenital anterior urethrocutaneous fistula and megalourethra are both rare anomalies. These anomalies are commonly associated with other anorectal or genitourinary anomalies and evaluated with voiding cystourethrography. We examined a 34-month-old boy who presented with a fistula at the penoscrotal junction. A voiding cystourethrogram showed a jet of urine coming through the fistula and proximal saccular dilatation of the penile urethra. We present the imaging findings of the first case of an association between a congenital anterior urethrocutaneous fistula at the penoscrotal junction and a proximal penile megalourethra. We also discuss the etiology, management, and differential diagnosis of this entity, and review the literature.

We report a case series of 8 patients, presenting with a congenital sinus in the region of the sternoclavicular joint. This rare malformation has only been reported in the Japanese dermatological literature under the name of "congenital dermoid fistula of the anterior chest region". It has to be distinguished from other congenital anomalies and requires complete excision., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objectives: A fourth branchial pouch remnant is well known as a pyriform sinus fistula. However, there has been no report of a fistula composed of the complete remnant of the fourth branchial apparatus. We experienced patients with a congenital lower neck cutaneous fistula which was thought to be the skin-side remnant of the fourth branchial cleft., Materials and Methods: Seven children were referred to our hospital from 2009 to 2015 for the treatment of a cutaneous fistula situated near the sternoclavicular joint. All of them were surgically resected and their pathological characteristics were examined. Clinical charts were retrospectively reviewed., Results: In six cases, the left side was affected. All cutaneous fistulas had a small skin orifice near the sternoclavicular joint and they were situated at the anterior edge of the sternocleidomastoid muscle. Abscess formation was seen in four cases. Surgical resection was performed at the age of 6 months to 9 years. These fistulas ran deep into the subcutaneous tissue and had a blind end. Pathological examination showed that the epithelial layer was mainly composed of a stratified squamous epithelium. In two cases the epithelium was composed of ciliated columnar epithelium. Recurrence has not been observed in any of the cases., Conclusion: The seven cases had a common clinical feature and were a definite clinical entity. Judging from the characteristics of our cases and the previous literature, we concluded that this lower neck cutaneous fistula was most likely a congenital skin-side remnant of the fourth branchial cleft., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Patent urachus is one of the least commonly seen of the urachal anomalies. In this report, we present a case of a patent urachus in a15-day-old female who presented with leakage from the umbilical site. The purpose of this article is to discuss the embryology, clinical manifestations, diagnosis, and management of patent urachus.

We describe what we believe is only the third reported case of coexisting first and bilateral second branchial fistulas associated with nonfamilial branchio-otic syndrome. The patient was a 6-year-old girl who presented with bilaterally draining anterior neck puncta, a preauricular sinus, and moderately severe bilateral hearing loss. She had no family history of branchial anomalies. Compared with branchial cysts and sinuses, branchial fistulas are rare. Even more rare are bilateral second branchial fistulas coexisting with first branchial anomalies, as only 10 cases have been previously reported in the English-language literature. Of these 10 cases, 5 were associated with either branchio-otic syndrome or branchio-oto-renal syndrome; 2 patients had familial branchio-otic syndrome, 2 had nonfamilial branchio-otic syndrome, and 1 had nonfamilial branchio-oto-renal syndrome.

Congenital prepubic sinus is a tract originating in the skin overlying the base of the penis. Its embryologic basis is still debated. We present a 9-month-old boy with a recurrent muco-purulent discharge from a tiny opening in the midline prepubic area on the base of the penis. Examination revealed a ventrally hooded prepuce, dorsal chordee, penile torsion to the left, and a 3-mm-diameter prepubic sinus 1 cm from the base of the penis. Histology of the excised tract showed a transitional epithelium. We believe our case is an epispadiac variant of dorsal urethral duplication., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: Hairy polyps are rare, congenital malformations of the oropharynx and nasopharynx. To date, approximately 145 cases have been reported. However, the histogenesis of these lesions remains unclear., Case Report: We report the case of a 2-day-old neonate presenting with a hairy polyp attached to the left palate, who re-presented aged 16 months with a discharging first branchial arch sinus., Conclusion: We propose this case as supporting evidence for the theory that hairy polyps are a malformation of the first branchial arch system.

Cyst and fistulas of the gills grooves and thyro-glossal tracts are well documented. On the contrary, congenital neck salivary fistulas are uncommon., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Objective: The aim of this study was to primarily investigate the usefulness of computerized tomographic (CT) fistulography in the diagnosis and management of branchial cleft fistulae and sinuses., Study Design: Fifteen patients with confirmed branchial fistulae or sinuses who had undergone CT fistulography were included. The diagnoses were confirmed by clinical, radiologic, or histopathologic examinations. The internal openings, distribution, and neighboring relationship of the lesions presented by CT fistulography were analyzed to evaluate the usefulness in comparison with x-ray fistulography., Results: Nine patients were diagnosed with first branchial fistulae or sinuses, 2 with second branchial fistulae, and 4 with third or fourth branchial fistulae. The presence and location of the lesions could be seen on x-ray fistulography. The distribution of the lesions, internal openings, and neighboring relationship with parotid gland, carotid sheath, and submandibular gland could be clearly demonstrated on CT cross-sectional or volume-rendering images., Conclusions: CT fistulography could provide valuable information and benefit surgical planning by demonstrating the courses of branchial anomalies in detail., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: Two cases of first branchial cleft fistula with internal opening on the Eustachian tube are reported and the diagnosis, management and embryological hypothesis are discussed., Design: Retrospective study and review of the literature., Results: Both patients were young boys with first branchial cleft anomaly clearly identified by computed tomography fistulography scan and direct Methylene Blue dye injection. In both cases, surgical removal revealed a fistula with internal opening located on the Eustachian tube near the nasopharynx., Discussion: The main embryological theories and classification are reviewed. A connection between the theories of first branchial apparatus development and the classification by Work might explain the reported clinical association., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

The pattern of congenital oral and craniofacial anomalies (CFAs) in the Kenyan population remains unknown. The objective of this study was to describe the pattern of occurrence of CFAs at two hospitals in Nairobi. A descriptive cross-sectional study at the Kenyatta National Hospital and Pumwani Maternity Hospital was carried out from November 2006 to March 2007. Mothers who delivered at the hospitals consented to an interview and physical examination of their babies within 48 h of delivery. The anomalies were classified for type and magnitude. Data were analysed to determine the association of these anomalies with ages of the mothers, gender, weight, birth order, mode of delivery and birth status of the babies. During the study period, 7989 babies were born. The CFAs manifested in 1.8% of the total births and were more common in female (1.4%) than in male (1.0%) live births. 12.8% of stillbirths had CFAs, with lesions manifesting more in males (16.7%) than in females (6.9%). The commonest CFA was preauricular sinus (4.3/1000) followed by hydrocephalus (1.9/1000) then preauricular tags and cleft lip and palate (1.5/1000 and 1.3/1000 total births, respectively)., (Copyright © 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Branchial cleft anomalies are congenital remnants of the embryologic branchial clefts persisting past the embryo stage. Most occur singly and sporadically, though syndromic associations are described. Multiple branchial cleft anomalies coincident in the same patient are exceptionally rare, and rarer still are peripheral dermal sinus tracts on the extremities, with one prior documented case. We report the first case of multiple branchial cleft anomalies with a peripheral dermal sinus of the ipsilateral lower extremity. Numerous concurrent congenital anomalies exist in the patient, representing the first description of the Guarisco-Winters syndrome. The patient is intellectually and developmentally age-appropriate in all other regards., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

The author reports a case of congenital fistula from an accessory parotid gland and describes its diagnosis and treatment. The patient was referred to the author's clinic for evaluation of a continuous serous discharge from a small orifice in the left cheek near the angle of the mouth. A left preauricular appendix was also noted. Fistulography detected an aberrant duct leading to an accessory parotid gland. The main parotid gland and its duct were normal. The anomalous duct was dissected in continuity with a small ellipse of skin and sutured to the buccal mucosa. The patient's recovery was uneventful. The author also discusses the embryologic origin of this rare anomaly.

Objective: Dermoids presenting in early life with a sinus tract along the midline of the nasal dorsum are well described. We present three very unusual variants of this condition., Methods: Case one was a two-year-old child with a pit in the philtrum of the lip. The tract pierced the nasal spine and extended towards the crista galli. Case two was a 15-month-old child with two separate sinus openings on the dorsum, one below and one above a dermoid cyst. Case three was a 17-year-old adolescent with neurological disability. Scanning showed a dermoid cyst deep in the anterior skull base, but the sinus tract had been obliterated by bony overgrowth due to long term use of antiepileptic medication., Results: Cases one and two were successfully managed by complete excision via an external rhinoplasty approach. Case three, in contrast, was not operated upon due to bony obliteration of the sinus tract., Conclusion: Surgeons dealing with nasal dermoids must be able to adapt to the variable anatomical and clinical features of these lesions.

Objectives: We report two cases of congenital salivary fistula of an accessory parotid gland, and we discuss its occurrence in Goldenhar syndrome., Methods: Two teenagers complained of a congenital cheek fistula with constant salivary discharge. Computed tomography fistulography and sialography were performed. The diagnosis of Goldenhar syndrome was established based on clinical and imaging findings. Previously reported cases are reviewed and the clinical and radiological features summarised., Results: In these two patients, a salivary fistula of an accessory parotid gland was demonstrated on computed tomography fistulography, and did not communicate with Stensen's duct. Deformity of Stensen's duct and hypoplasia of the ipsilateral mandibular ramus were present. Tragal appendices have frequently been reported in such cases., Conclusion: A congenital cheek salivary fistula of an accessory parotid gland should be considered indicative of Goldenhar syndrome.

A rare case of congenital salivary gland fistula is reported. A 3-year-old girl presented with clear discharge from a dimple on the left cheek. An ectopic salivary gland fistula was unexpectedly diagnosed during operation. This is the first case of congenital salivary fistula draining from a minor salivary gland to a cutaneous pit. We completely excised the lesion, and the patient remains complication free., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: The orofacial clefts include 30 variant according to Tessier classification: the number 30 contain mandibular arc damage isolated or associated with damage of surrounding soft tissue., Case Report: Our patient was a newborn with median mandibular cleft associated with ankyloglossia, bifid tongue and a top cervical fistula. We have not found polymalformative syndrome. The early surgical management included one time and after-effect were simple within 11 months., Discussion: We point out difficulties for antenatal diagnosis and controversy about appropriate time for surgical management of the bone defect. The last physiopathologic hypotheses were explicated., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Introduction: Preauricular fistulas are not uncommon congenital disorders in children. They are associated with imperfect auricle formation as a consequence of incomplete fusion of the auditory hillocks of the first and second branchial arches., Aim: The aim of this study was to present diagnostic methods with evaluation of treatment results of preauricular fistulas in children., Materials and Methods: We analysed retrospectively clinical and epidemiological features, diagnostic methods and treatment results of the patients with preauricular fistula attended in the Otolaryngology Department of Medical University in Gdańsk., Results: Between 1995 and 2009 in the Department of Otolaryngology MGUed 23 children (13 girls and 10 boys) were treated for preauricular fistulas. The age of the children ranged between 2 to 16 years (median 7.7). The fistula was recognized on the left side in 14 children, in 6 on the right and bilaterally in 3 children. The diagnosis was based on the presence of a cutaneous fistula in the region of the helix's anterior crus with inflamed surrounding and purulent discharged in physical examination and in radiological imaging (MRI). Patients were treated surgically in the classical way and using Prasada's method including resection of the cutaneous fistula and cyst with a part of the helix. Squamous epithelium in the resected fistulas was found in 91.3% of the children. No recurrence was observed, good cosmetic outcome was achieved in all children., Conclusions: Each patient with bilateral preauricular fistula needs to be diagnosed for congenital disorders of the middle ear and kidneys. MRI may be useful in establishing the location of the fistula. Surgical treatment is not necessary in preauricular, blind-ended sinuses. In the postoperative material of the sinus squamous epithelium is found.

Isolated congenital anterior urethrocutaneous fistula (CAUF) is an extremely rare deformity and few cases have been reported in the English language literature. Moreover, adult CAUF has not been reported up to now. We present a rare adult patient with this unusual isolated CAUF deformity. The possible etiology and treatment strategy are discussed., (Copyright © 2010 S. Karger AG, Basel.)

Low anorectal malformation comprises about half of all anorectal anomalies. Most of the literature concerning management of anorectal anomalies is centred around the treatment and outcome of high anomalies. The management of low anomalies has been considered significantly less challenging than high anomalies. Also, the outcome of low anomalies has traditionally been considered good. However, recent more critical long-term follow-up reports show a different picture. Many patients with low anomalies suffer from long-term anorectal functional problems, especially constipation but also soiling that occurs in a significant percentage of patients. In this review, we compile the recent views on the diagnosis, surgical treatment and outcome of low anorectal anomalies. We also present an algorithm for the management of these anomalies. The emphasis on the surgical management of low anorectal anomalies is to use as minimally invasive operative methods as possible and preserve the native mechanisms of continence that usually are much better preserved than in more severe high anomalies.

Congenital prepubic fistula is a rare congenital anomaly. Complete removal of the fistular tract remains challenging because of the complicated course. Although conventional fistulography has been used widely as a diagnostic tool for congenital prepubic fistula, more detailed information such as accurate localization of the fistular end or relative position to the urinary tract cannot be preoperatively obtained because the conventional contrast studies have insufficient capability. In this article, we reported the complete removal of congenital prepubic fistula based on preoperative magnetic resonance imaging findings, especially T2-weighted imaging. Magnetic resonance imaging clearly displayed not only the tract of the prepubic fistula originating from a subcutaneous cyst but also the tract extending and ending near the top of the urinary bladder., (Copyright 2010 Elsevier Inc. All rights reserved.)

The course of a third branchial fistula is derived from its embryological origin, in accordance with the branchial apparatus theory. Treatment of this condition requires complete removal of the tract in order to avoid recurrence; however, this can pose a risk to the surrounding structures. We report the case of a complete third branchial fistula as well as a literature review on its theoretical course and management.

The authors propose an update on cervicofacial congenital cysts and fistulas' symptomatology. Embryological data, epidemiology and clinical manifestations are described. A reminder of the therapeutic principles is proposed as well as the evolution of these congenital pathologies, which may or may not involve the branchial system. branchial.

Objective: After excision of a preauricular sinus, a head bandage compressive dressing is usually used to reduce dead space and to decrease the risk of recurrence. However, such use of a head bandage may cause various problems. We assessed a new method of compressive dressing, using suture transfixion of silicone sheets to the former sinus tract, following preauricular sinus excision., Methods: This retrospective study reviewed the medical records of patients undergoing preauricular sinus excision in a tertiary referral centre over a five-year period. After excision of the preauricular sinus, patients underwent suture transfixion of silicone sheets. Post-operative outcomes were analysed., Results: The new dressing method was performed on 50 ears of 37 patients. The post-operative incidence of recurrence and haematoma formation was 4 and 2 per cent, respectively. Other problems possibly caused by head bandaging, such as headache, facial flushing, and nausea and/or vomiting, were not observed., Conclusion: Compressive dressing by suture transfixion of silicone sheets is safe and effective following preauricular sinus excision.

Objective: We present an extremely rare case of a 44-year-old woman with right gustatory otorrhoea and otalgia., Case Report: The patient had been initially treated for otitis externa after Pseudomonas aeruginosa was grown from a microbiological swab. The otorrhoea fluid was collected and tested positive for amylase. Sialography and computed tomography imaging of the temporal bone confirmed a sialo-aural fistula from the right parotid gland to the bony external acoustic meatus. The defect was consistent with a patent foramen of Huschke. The fistula was identified surgically via a superficial parotidectomy approach, after contrast injection of Bonney's blue dye into the parotid duct, and then ligated and divided. The patient had immediate and sustained resolution of her otorrhoea., Conclusions: Sialo-aural fistulae are extremely rare, and usually arise as a complication of surgery or as an acquired disease process. To date, only four cases have been reported. This case demonstrates the use of sensitive investigation involving sialography and computed tomography, as well as successful surgical management, with complete resolution of symptoms.

Summary: Congenital anterior urethrocutaneous fistulas are infrequent. We report a case of a congenital anterior urethrocutaneous fistula associated with a stenosis of the bulbar urethra in the context of a high anorectal malformation. We describe the surgical technique for the reconstruction of the urethra.

Unlabelled: We report the imaging findings in a rare case of an accessory parotid gland fistula., Material and Methods: An eight-year-old boy was presented with complaints of serous discharge from his left cheek since birth. As part of the radiological investigation, magnetic resonance imaging, computed tomography sialography with fistulography, and digital sialography with fistulography were performed., Results: Magnetic resonance imaging demonstrated the exact location of an accessory parotid gland but failed to demonstrate the accessory duct. The presence of an accessory gland was well delineated on computed tomography fistulography and computed tomography sialography. Fistulography revealed a small accessory parotid duct and gland. No communication between the ductal systems of both glands was demonstrated., Conclusions: In such cases, pre-operative imaging (with sialography, magnetic resonance sialography and computed tomography sialography with fistulography) is helpful for exact delineation of the ductal anatomy. To the best of our knowledge, only four previous cases of congenital accessory parotid gland fistula have been reported in the English literature.

Background: Van der Woude syndrome (VWS; OMIM 119300) is an autosomal dominant developmental malformation presenting with bilateral lower lip pits related to cleft lip, cleft palate, and other malformations in about half the patients. These congenital lip pits appear clinically as a defect in the vermilion border of the lip. They are commonly related to fistulae of minor salivary glands with or without excretion. Usually the cause of cosmetic defects, VWS is preferably treated through simple excision. This report aims to describe a case of VWS involving a patient who presented with isolated congenital bilateral lower lip pits related to fistulae of the minor salivary glands, which were treated successfully through simple excision., Case Report: A 5-year-old girl with a diagnosis of congenital bilateral lip pits associated with fistulae of minor salivary glands was treated successfully through simple excision. The diagnosis was confirmed by clinical examination, sialography, and scintigraphy. A good aesthetic result was observed after 2 years of follow-up evaluation., Conclusion: Physicians must be aware of VWS because, although underreported and frequently not diagnosed, it is the most common cleft syndrome.

Midline congenital abnormalities of the skin may be related to subjacent visceral developmental defects. We report on three unrelated children presenting a small midline pit on the bridge of the nose from which a small tuft of hair was emerging. This presentation is the hallmark of a nasal dermoid sinus cyst. In one of the patients the sinus tract was connected to the anterior cerebral fossa and required neurosurgical management. Although midline cutaneous abnormalities may look trivial, dermatologists should keep a high index of suspicion for possible association with more severe underlying defect. The diagnosis of nasal dermoid sinus cyst requires appropriate imaging studies to determine the extension of the sinus tract thus allowing proper management.