Cleft palate lateral synechia syndrome in two patients and literature review.

Cleft palate lateral synechia (CPLS) syndrome is an extremely rare congenital malformation syndrome of unknown origin, characterized by the association of cleft palate and one or more intraoral lateral synechiae (OMIM # 119550). Fewer than 20 cases have been described to date. The clinical and histological findings and results of genetic investigations for two additional cases of CPLS are presented herein, in order to better delineate this syndrome, within the context of the relevant literature. The first case presented with a U-shaped cleft palate, bilateral synechiae, and Pierre Robin sequence, requiring early sectioning of the synechiae because of severe feeding problems. The second case presented with a V-shaped cleft palate and a single synechia, running from the left border of the cleft to the floor of the mouth, and was without feeding difficulties. In both cases, histopathological examination of the synechiae revealed an aspect of mucous membranes macroscopically, while staining of sections indicated lymphocyte infiltrates and parakeratosis with stratified squamous epithelium, associated with vessel and connective tissue abnormalities. Sequencing of candidate genes did not identify a genetic cause. Accurate clinical descriptions, histopathological diagnosis, and genetic investigations of patients with synechiae are lacking in the literature. Better characterization of future cases of CPLS will give new insights into its developmental causes.

A Peculiar Case of Small Bowel Stricture in a Coronavirus Disease 2019 Patient with Congenital Adhesion Band and Superior Mesenteric Vein Thrombosis.

BACKGROUND: There is increasing evidence supporting coronavirus disease 2019 (COVID-19)-related coagulopathy. In the available literature, only 2 cases of superior mesenteric vein thrombosis have been described. METHODS: We present a peculiar case of high-grade small bowel obstruction in a patient with COVID-19 infection. RESULTS: Exploratory laparotomy revealed a congenital adhesion band with associated focal bowel ischemia contributed by superior mesenteric vein thrombosis and positive lupus anticoagulant. CONCLUSIONS: It is important to consider the rare differential of mesenteric vein thrombosis and its related sequelae of mesenteric ischemia in a patient with COVID-19 who presents with abdominal pain.

Intestinal obstruction due to congenital bands in adults who have never had abdominal surgery Two case reports and a review of the literature.

Congenital abdominal bands are a very rare entity and are often silent for a person's entire life. In a very few circumstances, however, they can manifest with clinical symptoms. Diagnosis of these bands is very challenging as the patient has never submitted for abdominal surgery and imaging, laboratory tests and clinical examination can be silent or unspecific. Two patients were admitted to and operated on in our hospital between June 2017 and July 2018. All patients had a bowel obstruction at the time of presentation, presenting no emission of faeces or gas. Conservative therapy was tried in one case, and surgical intervention was required. At surgery, both patients were found to have congenital bands causing the occlusion. The postoperative courses were uneventful. Small bowel obstruction (SBO) by congenital bands remains a challenging disease for surgeons. Diagnosis of SBO has to be suspected even if radiology does not indicate any relevant features. Laparoscopy should be the preferred approach, but if not feasible, then open surgery can resolve the case. KEY WORDS: Adhesions, Congenital bands, Intestinal occlusion, Laparoscopy.

A veil in the oral cavity: report of two cases of oral synechiae.

An oral synechia is an adhesion between the maxilla and the mandible; these adhesions can be found at various locations in the oral cavity. The presence of oral synechiae associated with cleft palate represents a rare congenital deformity. Oral synechiae with cleft palate can have serious implications for airway management and feeding in babies, and hence requires early intervention to reduce morbidity. Two cases of congenital oral synechiae accompanied by cleft palate, in which the synechiae veiled the oral cavity restricting a detailed intraoral examination, are reported here.

Peritoneal encapsulation; a rare cause of bowel obstruction.

Peritoneal encapsulation is a rare congenital cause of bowel obstruction in children. We present the case of a 12-year-old male with severe dehydration and recurrent episodes of vomiting. This pathology should be considered in cases of bowel obstruction with a virgin abdomen.

Congenital alveolar synechiae with cleft palate.

Congential maxillomandibular fusion or jaw adhesions is a rare entity. It is often associated with other congenital anomalies like cleft palate and is characterized by restricted mouth opening. This unusual presentation of congenital alveolar synechiae is highlighted in a 15 days old baby boy. Under inhalational anaesthesia his fibrocartilagenous adhesions were excised and endotracheal tube was then placed. Good recovery without any residual scars or adhesions and a 25 mm mouth opening was achieved.

Familial adhesive arachnoiditis associated with syringomyelia.

Adhesive arachnoiditis is a rare condition, often complicated by syringomyelia. This pathologic entity is usually associated with prior spinal surgery, spinal inflammation or infection, and hemorrhage. The usual symptoms of arachnoiditis are pain, paresthesia, and weakness of the low extremities due to the nerve entrapment. A few cases have had no obvious etiology. Previous studies have reported one family with multiple cases of adhesive arachnoiditis. We report a second family of Belgian origin with multiple cases of arachnoiditis and secondary syringomyelia in the affected individuals.

Management of serious isolated gingival synechia in a newborn: case report and review of the literature.

Congenital synechia of the gums is a rare malformation. The fusion may be partial or complete and may be associated with other facial dysmorphologic malformations. The synechia can be fibrous or bony and may develop between the upper and lower alveolar ridges. The authors report a newborn with partial, but broad and continuous, synechia of the gums and discuss its management during the first days of life.

Oral synechia with epithelial cyst in neonate with cleft palate: a case report.

Cleft palate with oral synechia is a rare congenital deformity that is represented in the literature by only a handful of cases. Midline synechia is less common than lateral. Failure to recognize and appropriately treat this condition has serious implications for neonatal airway management and feeding. We present a case of cleft palate with midline subglossopalatal synechia that was transferred from an outside institution after a prolonged period of nonsurgical management in which the patient ultimately required intubation for respiratory distress. Release of the synechia was performed without complications. We review the current literature and discuss diagnosis and surgical management of this rare condition.

Congenital lateral cleft palate and lateral palatal synechiae.

We report a sporadic case of congenital lateral cleft palate and lateral palatal synechiae and suggest that its mechanism is embryonal. This is the first published case that we know of with cleft palate-lateral synechiae syndrome including the lateral cleft palate.

Congenital fusion of the maxilla and mandible: brief case report.

Congenital fusion of the mandible and maxilla is a rare anomaly usually seen in association with various syndromes. Reports of isolated cases of bony fusion of the jaws are sparse. Only 10 reported cases were found in the literature search. Maxillomandibular fusion restricts mouth opening, causing feeding problems and difficulties in swallowing, respiration, growth, and development, and thus must be treated early. We report a case of congenital fusion of the mandible and maxilla in a 1-year-old boy and describe the clinical features of this anomaly to add to the existing literature on the subject. This is our second encounter of such a case.

Congenital alveolar synechiae - a case report.

Congenital alveolar synechiae is rarely seen as an isolated disease. It is generally observed together with various syndromes such as Van der Woude and cleft palate lateral alveolar synechiae syndrome, and is concomitant with other anomalies in the maxillofacial or other regions of the body. Prior to this case report , eight cases of isolated congenital alveolar synechiae have been reported. This paper reports a case of isolated congenital alveolar synechiae in a 10-month-old baby girl. The report concentrates on the clinical features of isolated congenital alveolar synechiae, the likely aetiological causes and the treatment.

Cleft palate and congenital synechiae syndrome: a case report.

OBJECTIVE: A 1-day-old baby girl with a cleft of the secondary palate and a soft tissue band connecting the upper and lower jaws and preventing mouth opening was referred to the cleft lip and palate team by her pediatrician. This case represents a further example of an interesting but rare anomaly known as congenital alveolar synechia syndrome that requires early management to allow normal feeding and oral development.

Congenital gum synechiae as an isolated anomaly: a case report.

Congenital adhesions between different parts of the oral cavity rarely occur. These adhesions usually present difficulty in feeding and even respiration soon after birth. In the case presented here the mild form of gum synechia without associated congenital anomaly was treated by excision of the anterior and posterior alveolar bands. Potential jaw and temporomandibular joint development was not compromised and functional problems were eliminated with such a noninvasive procedure.

Cleft-palate lateral synechia syndrome: insight into the phenotypic spectrum of Fryns syndrome?

BACKGROUND: In 1972, Fuhrmann et al. (Humangenetik 1972;14:196-203) described a novel syndrome consisting of cleft palate (CP) and lateral synechiae (LS) between the palate and the floor of the mouth. This constellation of malformations, since denoted as cleft-palate lateral synechiae syndrome (CPLS), is a rare syndrome; only five cases have been reported since the original description. Because of the paucity of recognized cases, little is known regarding the phenotypic spectrum of this presumably autosomal dominant condition. CASES: We report two unrelated patients who presented with remarkably similar phenotypic features, including multiple intraoral synechiae (filiforme intraalveolar bands), cleft palate, micrognathia, and redundant lower lip tissue. Their phenotypic findings indicate a diagnosis of CPLS; however, case 3 (the monozygotic twin of case 2) had classic phenotypic features of Fryns syndrome. CONCLUSIONS: This report presents two new cases of CPLS, and suggests that the CPLS phenotype may represent the mild end of the Fryns syndrome phenotypic spectrum. Supplementary material for this article can be found on the Birth Defects Research (Part A) website (http://www.interscience.wiley.com/ jpages/1542-0752/suppmat/67/fig5.xls).

Popliteal pterygium syndrome with syngnathia.

We report a case of perioperative management of a neonate with popliteal pterygium syndrome complicated by interalveolar syngnathia. Syngnathia were excised in the operating room without a major anaesthetic. We discuss our management of this case, as well as other possible strategies to secure the airway in neonates with syngnathia. We also reviewed the literature regarding airway management in presence of syngnathia in similar situations.

Syngnathia and Van der Woude syndrome: a case report and literature review.

OBJECTIVE: Syngnathia is a rare anomaly involving soft tissue or bony adhesions between the maxilla and mandible. We review the literature and present a case of syngnathia associated with Van der Woude syndrome. Syngnathia can have very different etiologies, but this has rarely been reported in Van der Woude syndrome. Treatment of this condition is rarely discussed in the literature because of a paucity of case reports. RESULTS: Oral adhesion (syngnathia) in our patient was caused by a mandibular to maxillary fibrous band. Surgical treatment was successful. Clinical implications and review of literature for the treatment of this rare association of syngnathia and Van der Woude syndrome are discussed.