Umbilical Appendix Masquerading as a Patent Omphalomesenteric Duct in a Neonate.

The umbilicus is the site of a number of well-recognized and unusual abnormalities. Well-known neonatal umbilical abnormalities include umbilical hernias, granulomas/polyps, and congenital remnants of development. In this article, we describe a rare case of an appendix draining through the umbilicus of a neonate. In the literature, there are only 15 cases with possible umbilical appendix. We describe this rare case along with a review of the literature and discuss the underlying pathophysiology.

Diagnóstico diferencial desafiador de Lesão na Cicatriz Umbilical: relato de caso / Challenging differential diagnosis of Umbilical Scar Lesion: case report

Introdução: Nevo epidérmico corresponde a um conjunto de lesões benignas caracterizadas pela hiperplasia da camada basal da epiderme, sendo comum na infância e raramente observado na idade adulta. Objetivo: Relatar um caso de nevo epidérmico verrucoso de apresentação atípica, bem como o curso da sua investigação diagnóstica. Relato do caso: Paciente M.E.M.P, mulher, fototipo III, 69 anos, procurou o Ambulatório de Dermatologia do Hospital do Servidor Público Municipal de São Paulo com queixa de nódulo pruriginoso de surgimento há 1 ano na cicatriz umbilical, sem história de trauma local. Discussão: O diagnóstico diferencial das lesões da cicatriz umbilical é desafiador, sendo o umbigo sede tanto de processos benignos quanto malignos. Conclusão: É infrequente a observação de nevos epidérmicos verrucosos nessa localização, ressaltamos a importância da imuno-histoquimica e da dermatoscopia para a elucidação diagnóstica, uma vez que os principais diagnósticos diferenciais considerados compartilham achados histológicos comum. Palavras-chave: Nevo epidérmico. Dermatoscopia. Umbigo.

Presentation of an open entry technique for laparoscopy through a congenital defect in the umbilical fascia.

OBJECTIVE: We present and describe a modification of the Hasson open entry technique to gain access to the abdominal cavity for laparoscopy in which a congenital defect in the umbilical fascia is identified for entry into the peritoneum and insertion of the primary port. METHODS: A single centre, prospective, observational, pilot study has been conducted with no change in clinical practice. Data regarding the success of the technique, time to laparoscope insertion, complications and patient risk factors were collected and presented. RESULTS: The team enrolled 114 patients that had the St Helier technique attempted for entry in the abdominal cavity. Entry was achieved for all patients. The technique had 82.5 % success rate while this reduced to 65 % in patients with previous laparoscopies. The mean time to insertion of the laparoscope was 220 s, and there was no significant difference in success with variation in BMI. We recorded no minor or significant intra-operative complications. The superficial wound infection rate was 2.6 % with no other postoperative complications identified at 6-week follow-up. CONCLUSIONS: The presented technique is a safe and successful method of laparoscopic entry with a presumed shorter time until laparoscope insertion than other techniques used. The absence of complications could be attributed to the avoidance of sharp dissection of the umbilical fascia. The less invasive nature could reduce risks of hematoma, infection or hernia that are associated with the standard entry techniques used. Formal studies of long-term outcomes are required, as well as evaluating use in emergency and contaminated cases.

[Omphalomesenteric fistulas; epidemiological diagnostic and therapeutic features: study of four cases in the Department of Pediatric Surgery at the Aristide Le Dantec University Hospital in Dakar]. / Fistules omphalo-mésenteriques; aspects épidémio logiques, diagnostiques et thérapeutiques: à propos de quatre observations au Service de Chirurgie Pédiatrique du CHU Aristide Le Dantec de Dakar.

This study aims to determine the epidemiological, therapeutic and diagnostic features of omphalomesenteric fistulas (OMF). We conducted a study of four cases over a period of 10 years, from January 2004 to December 2013. The parameters studied were: frequency, age, sex, clinical and radiological signs, therapeutic and evolutionary features. Frequency was 0.4 cases per year. Patients were aged 11 days, 40 days, 45 days and 3 years respectively (three girls and one boy). Clinical examination showed intestinal fluid discharge from the belly button and belly button bud catheterisable in all the cases. The bud was prolapsed in the patient aged 45 days. Fistulography performed in two cases helped to confirm the diagnosis by showing a communication between the fistula and the small intestine. The assessment of malformations revealed congenital cyanogen heart disease with interventricular communication in the newborn aged 45 days, anorectal cloacal malformation associated with urachus fistula in the newborn aged 11 days. All patients underwent surgery. Semicircular periumbilical incision was performed in the absence of associated abdominopelvic malformations. A communication between the fistula and the ileum was found in the majority of cases. Bowel resection with termino-terminal anastomosis was performed in three cases. Cuneiform resection was performed in one case and was completed by complete resection of the urachal fistulous tract and bladder suture, with colostomy in newborn with urachal fistula and anorectal cloacal malformation. The postoperative course was marked by non-febrile seizures in the first child with good evolution and by superficial parietal suppuration followed by death due to cardiac decompensation in the third case. Omphalomesenteric fistula is rare. Diagnosis is based on clinical examination complemented by the fistulography. Surgical treatment using semicircular periumbilical incision gives good results. However, the assessment of malformations is necessary.

A simple technique for neoumbilical reconstruction for congenital hernia associated proboscis deformity.

INTRODUCTION: Umbilical reconstruction in pediatric patients who have developed a large proboscoid redundancy can be challenging after standard umbilical repair. We present a simple and unique surgical technique that results in a cosmetically appealing reconstruction. OPERATIVE TECHNIQUE: The operation is initiated with circumferential redundant skin excision and isolation of the hernia sac. Primary fascial repair is performed. Reconstruction utilizes the cut dermal/epidermal edge by approximating it to the exposed fascia just below the skin edge outside of the fascial repair in a purse string fashion for the creation of a neoumbilicus. CONCLUSION: This simple technique is unique from any currently published methods and results in a cosmetically pleasing reconstruction without evidence of any incision. LEVEL OF EVIDENCE: Level V: Expert Opinion.

Congenital prepubic sinus-An aborted dorsal urethral duplication or a cloacal remnant?

BACKGROUND: Congenital Prepubic Sinus (CPS) is an uncommon urogenital anomaly characterized by a blind tract between the skin over the pubis to anterior of the urinary bladder, Urethra or umbilicus. We report four such cases to emphasize varied clinical presentation and embryological conundrum. METHODS: Following Ethical Review Committee (ERC) approval, medical records of pediatric patients (<16 years) presenting with CPS (identified through operating room records and Hospital Information Management System (HIMS) between 1994 and 2018 were reviewed for demographics, clinical presentation, investigations including histopathology, management and outcome. RESULTS: Four cases of CPS, 3 females and 1 male, age range 9 months to 13 years were managed over 25-years. Clinical presentation includes a discharging sinus and recurrent episodes of cellulitis and abscess formation in pubic area and labia majora. Urological investigations were mostly normal. Insertion of lacrimal probe or plastic sheath of intravenous cannula through the sinus opening was useful to determine the course of sinus and aid its excision. Histology of excised sinus highlights the possible embryological origin. CONCLUSIONS: CPS is a rare anomaly with varied clinical presentation. It seems CPS is an aborted urethral duplication (Stephen Type 3) or a Cloacal remnant. Complete excision of the tract in the reported cases was curative. TYPE OF STUDY: Case series. LEVEL OF EVIDENCE: IV.

Laparoscopic Evaluation of Umbilical Disorders in Calves.

OBJECTIVE: To describe a laparoscopic technique for evaluating umbilical disorders in calves, including feasibility, visualization of umbilical structures, and related complications. STUDY DESIGN: Prospective clinical study. ANIMALS: Male calves (15 Holstein, 2 Montbeliard) with umbilical disorders (n=17). METHODS: Calves <2 months old with obvious umbilical disease were assessed by clinical examination and ultrasonography of the umbilical structures. Laparoscopic evaluation was performed in dorsal recumbency under subarachnoid lumbosacral anesthesia and sedation. An open insertion technique with short 60 mm cannulas was used after creating 2 portals 10 cm cranial to the umbilicus (one 5 cm left of midline for the laparoscope and one 5 cm right of midline as an instrument portal). After laparoscopy, abnormal tissues were resected by laparotomy during the same anesthetic period. RESULTS: Laparoscopic evaluation of umbilical structures was performed quickly (mean surgery time 7.1 ± 2.5 minutes). Umbilical structures could be completely visualized in all calves without intraoperative complications. In addition to abnormalities previously detected on ultrasound, laparoscopy enabled detection of adhesions 7 calves that were not suspected on ultrasound, as well as focal enlargements of the umbilical arteries and urachus close to the bladder in 5 calves. Laparoscopy failed to detect abnormalities observed with ultrasound or laparotomy in 4 calves, including small hernias and omphalitis. CONCLUSION: Laparoscopic evaluation of umbilical structures was performed safely and quickly in young calves and allowed complete evaluation of intra-abdominal umbilical structures and may, therefore, be a useful adjunct to physical examination and ultrasound to fully assess the abdomen in calves.

Abnormal appearance of the umbilicus: an indicator of urachal anomalies.

The urachus is an intra-abdominal fibrous remnant of the allantois. The non-involution of the allantois can result in urachal anomalies. The abnormal appearance of the umbilicus may be a sign of such anomalies. We have observed 3 cases of term neonates with atypical appearance of the umbilical stump, all of which manifested urachal anomalies, as documented by ultrasound scan. These appearances are rarely described in the literature, and seem to regress at around 2 months. Therefore, it is important that healthcare professionals should be aware of the possible implications of atypical umbilical stumps, evaluate each case accordingly and, if an urachal anomaly is diagnosed, refer the patient to a paediatric surgery centre, as such malformations carry an underlying risk of infection or malignancy.

[Nonsurgical and surgical treatment of an urachal fistula in an alpaca cria. A case report]. / Konservative und chirurgische Therapie der Urachusfistel bei einem Alpaka-Cria. Ein Fallbericht.

Urachal fistula is a neonatal condition. There are two reported forms: a congenital and an acquired form. We describe the case of a 6-hour-old female alpaca cria that was presented with a damp umbilicus and a meconium impaction. Conservative treatment of the urachal fistula with local and systemic medication was unsuccessful after 6 days; therefore, a resection of the umbilicus under general anaesthesia was performed. Reconvalescence was uneventful.

Umbilical and periumbilical dermatoses.

The umbilicus may be the site of congenital and acquired malformations and may harbor clinical clues to the diagnosis of potentially fatal inherited disorders, primary skin conditions, and a variety of infectious diseases. Both benign and malignant tumors may involve the umbilicus, and some are unique to this site. Finally, cutaneous signs localized to this anatomic location may be found in diverse systemic diseases.

Patología del ombligo / Pathology navel

El cordón umbilical, tan vital en la vida intrauterina, no tiene una función fisiológica en el ser humano después del nacimiento. Entre los primeros 10-20 días de vida del recién nacido requiere de cuidados que el pediatra y personal de salud que realiza los controles debe indicar a la madre para asegurar que el proceso de desecación y eliminación del cordón se complete de forma apropiada. Sin embargo, aun cuando la madre haya seguido correctamente las indicaciones, existen posibilidades de que la evolución clínica no sea la normal y la cicatrización se retarde o complique. Las posibilidades que el clínico debe sospechar son bastante variadas dentro de un espectro que va desde patologías leves hasta aquellas más complejas que incluyen alteraciones del desarrollo intrauterino con resultado de malformaciones congénitas. El propósito de este artículo es revisar la literatura publicada al respecto y actualizar aspectos clínicos importantes que el pediatra o médico que atiende niños debe conocer para orientar correctamente el diagnóstico y oportunidad terapéutica.

The umbilical cord, is a vital structure in utero, but it has no one physiological function in humans after birth. Among the first 10-20 days old newborn baby requires care pediatrician and health staff should learn to the mother how she must be care to make the drying process and complete removal of the cord can be appropriate. However, even if the mother has correctly followed the instructions, there are chances that the clinical course than the normal healing is delayed or complicated. The chances that the clinician should suspect are quite varied within a spectrum from mild to the most complex pathologies including disturbances of intrauterine development resulting in congenital malformations. The purpose of this article is to review the published literature and update important clinical aspects pediatrician or physician who treats children should learn to correctly orient the diagnosis and therapeutic opportunity.

Prolapsed urachal sinus with pyourachus in an infant.

We report an infant who presented with a discharging single-lumen, tubular, and mucosa-lined umbilical mass and a large central abdominal mass, inferior to the umbilicus. At operation, the mass was discovered to be a prolapsed, open, and inflamed urachal sinus leading to a pyourachus. Primary total excision from dome of the bladder to the umbilicus led to smooth recovery with excellent results.

Vitello-intestinal duct injury after transabdominal preperitoneal inguinal hernia repair.

A 71-year-old patient underwent a transabdominal preperitoneal right inguinal hernia repair and presented to the emergency department 48 hours postoperatively with nonspecific abdominal and chest pain and was diagnosed an abdominal abscess at the umbilicus by computed tomography. Laparotomy showed a vitello-intestinal duct injury caused by the umbilical trocar at his previous laparoscopic surgery. We will discuss this rare complication and how to avoid it in future practice.

Coexistence of multiple omphalomesenteric duct anomalies.

The omphalomesenteric duct is an embryonic structure which connects the yolk sac to the midgut. The omphalomesenteric duct attenuates between the 5th and 9th week of gestation. Failure of the omphalomesenteric duct involution, either partial or complete, results in various omphalomesenteric duct remnants including Meckel's diverticulum, patent vitelline duct, fibrous band, sinus tract, umbilical polyp and cyst. Omphalomesenteric duct remnants are present in 2% of the population but related diseases have seldom been reported in adults. The simultaneous presence of sinus tract, omphalomesenteric cyst, fibrous ligament and Meckel's diverticulum has, according to authors' knowledge, never been reported. We present a case of a 23 years old male with persisting umbilical discharge for 2 years in whom there was coexistence of the above mentioned anomalies of the omphalomesenteric duct.

Evolution of congenital malformations of the umbilical-portal-hepatic venous system.

OBJECTIVE: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. MATERIALS AND METHODS: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. RESULTS: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. CONCLUSION: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.

How to reconstruct a natural and deep umbilicus: three methods of umbilicoplasty for five types of umbilical deformities.

UNLABELLED: Conventional methods of umbilicoplasty using V-Y advancement flap often result in unnatural wide or shallow umbilical depressions facing upward or downward. Moreover, although the umbilical deformities have many variations, no report has described the selection of an umbilicoplasty method for types of umbilical deformity. To resolve these problems, we devised 3 methods of umbilicoplasty. In this report, we classified all kinds of umbilical deformities into 5 types, and studied the most suitable method for each type of umbilical deformity. METHOD: The umbilical deformities are classified into Type 0: the defect of umbilicus; Type I, the low-grade protrusion; Type II, the high-grade protrusion with wide base; Type III, the high-grade protrusion with narrow base; and Type IV, the protrusion in depression. The most suitable method among our 3 methods was adapted to each type. Method 1 with a S-shaped skin incision was adapted to Type 0 and I, Method 2 with fan-style flaps was adapted to Type II, and Method 3 with dividing the umbilical protrusion was adapted to Type III and IV. RESULTS: Sixty-three patients (10 cases of Type 0, 31 cases of Type I, 10 cases of Type II, 5 cases of Type III, and 7 cases of Type IV) underwent umbilicoplasty using the suitable method, and all were well corrected. CONCLUSIONS: Using the best choice among our 3 methods, it is easy to create a natural, vertically long and deep umbilical depression without conspicuous scars in all types of umbilical deformities.

MASP1 mutations in patients with facial, umbilical, coccygeal, and auditory findings of Carnevale, Malpuech, OSA, and Michels syndromes.

Distinctive facial features consisting of hypertelorism, telecanthus, blepharophimosis, blepharoptosis, epicanthus inversus, periumbilical defects, and skeletal anomalies are seen in autosomal-recessive Carnevale, Malpuech, Michels, and oculo-skeletal-abdominal (OSA) syndromes. The gene or genes responsible for these syndromes were heretofore unknown. We report on three individuals from two consanguineous Turkish families with findings characteristic of these syndromes, including facial dysmorphism, periumbilical depression, mixed hearing loss, radioulnar synostosis, and coccygeal appendage. Homozygosity mapping yielded an autozygous region on chromosome 3q27 in both families. In one family, whole exome sequencing revealed a missense mutation, MASP1 c.2059G>A (p.G687R), that cosegregated with the phenotype. In the second family, Sanger sequencing of MASP1 revealed a nonsense mutation, MASP1 c.870G>A (p.W290X), that also cosegregated with the phenotype. Neither mutation was found in 192 Turkish controls or 1200 controls of various other ancestries. MASP1 encodes mannan-binding lectin serine protease 1. The two mutations occur in a MASP1 isoform that has been reported to process IGFBP-5, thereby playing a critical role in insulin growth factor availability during craniofacial and muscle development. These results implicate mutations of MASP1 as the cause of a human malformation syndrome and demonstrate the involvement of MASP1 in facial, umbilical, and ear development during the embryonic period.