Abstract
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.
Congenital synechia of the gums is a rare malformation. The true incidence is unknown. The fusion may be partial or complete and may be associated with other facial malformations, such as facial hemiatrophy or temporomandibular ankylosis and cleft palate, cleft lip, microglossia, micrognathia, or limb anomalies . Complete fusion may interfere with feeding, leading to severe nutritional problems. Major difficulties may also arise during intubation for surgery. Such adhesions can be fibrous or bony and may develop between the upper and lower alveolar ridges or between the tongue and margins of the palate or maxilla . The authors report a newborn, who is the youngest case reported, with partial but broad and continuous synechia of the gums, and discuss its management during the first days of life.
Case report
This female newborn was born at 39 weeks’ gestational age by vaginal delivery to a 27-year-old, gravida 2, para 2 mother. The APGAR scores at 1 and 5 min were 8 and 10, respectively, and no resuscitation was needed. She weighed 2670 g and was 43 cm tall. The prenatal inquiry revealed solely a history of polyhydramnios during gestation, and there was no history of a similar baby in the previous gestation. There was no consanguinity between parents, and there was no history of ingestion of drugs or alcohol or of trauma during the gestation period.
The physical inspection revealed normal head circumference at 33 cm and normal eyes and ears in shape, with a flattened nasal saddle. The maxillary bony structure looked hypoplastic. The upper and lower parts of the lips were free and mobile. There was a broad synechia between the upper and lower gums from the right side of the mouth to nearly half of the left side of the mouth, leaving an approximately 1.5 cm aperture, which permitted passage of an orogastric feeding tube ( Fig. 1 ). Through this aperture, movement of the tongue could be seen with difficultly whilst the baby was crying. Some oral secretions were discharged through the non-synechial aperture in the oral cavity but did not cause any difficulty with respiratory effort. The rest of the baby’s body was normal on examination.
The baby was admitted to the neonatal intensive care unit for follow-up and to avert possible complications of vomiting and aspiration of gastric fluid, which might have caused respiratory difficulty or suffocation. The complete blood count, C-reactive protein, and biochemical assessment were all in the normal range.
An orogastric feeding tube was advanced through the oral cavity aperture to investigate whether there was an accompanying malformation of the oesophageal passage to the stomach; the passage was continuous. It was decided not to insert a permanent orogastric tube to decompress the gastric fluid because it might trigger the gagging reflex and vomiting. To prevent vomiting and aspiration, the baby’s head was kept angled upward by 45°. Intermittent oropharyngeal aspiration was done gently when oropharyngeal secretions were seen around the mouth. The baby was not fed orally because the mouth opening was too narrow to discharge the gastric contents, which might predispose to aspiration and would not allow prompt intubation in an emergency. Total parenteral nutrition was used for feeding to ensure sufficient nutrition. In addition to an open crib, other arrangements to enable emergency care in the case of airway obstruction or respiratory distress included having equipment for an airway, tracheostomy, resuscitation, and mechanical ventilation immediately available.
The antero-posterior and lateral X-rays of the head and jaws showed no bony fusion or accompanying bony anomalies ( Fig. 1 ). Transfontanel and abdominal ultrasonography showed no accompanying anomalies. An apical muscular ventricular septal defect, small ductal shunt, and small patent foreman ovale were detected on echocardiography, but these did not cause any haemodynamic disruption. A genetic consultation determined that the karyotype was normal.
On postnatal day 5, preoperative nasopharyngeal intubation was performed, and the synechial parts of the gums were detached successfully. The baby was fed orally on day 3 and discharged on day 7 after surgery. On follow-up, the baby was assessed as normal, and she weighed 6.4 kg at 4 months ( Fig. 2 ).
Discussion
Congenital maxillomandibular fusion comprises a rare group of anomalies varying in severity from simple mucosal adhesions (synechia) to extensive bony fusion (syngnathia) . The exact aetiology is unknown, although several hypotheses have been proposed. It is hypothesised that during in the seventh week of embryological life, with development of the alveolar ridge and when the tongue and palatal shelves are in close contact, palatal closure depends on downward and forward contraction of the tongue. When the tongue protrudes from the mouth as a result of medial movements of the oral cavity walls, it prevents the alveolar ridges from fusing. Genetic, teratogenic, or mechanical insults during this critical period may lead to prolonged close contact between oral structures, leading to abnormal fusion . The maternal history did not reveal such insults in this case.
Congenital alveolar synechia is rarely seen in isolation. It is generally observed together with various syndromes, such as Van der Woude syndrome and cleft palate lateral alveolar synechiae syndrome, and is often concomitant with other anomalies in the maxillofacial or other regions of the body . Only nine cases of isolated gum synechia have been reported ( Table 1 ). This case is the tenth newborn reported with isolated gum synechia. In a review, G artlan et al. reported six cases with isolated gingival adhesions prior to 1993 . H aydar et al. reported a case in which the synechia consisted of two mucosal bands extending from the hard palate to the anterior portion of the lingual sulcus . Two additional cases were reported that only sought medical care at 2 and 10 months of life, respectively . The present case was diagnosed earlier. The literature notes a predilection for females, which should be investigated further.
| Authors, year | Intraoral synechia | Age, gender | Micrognathia | Microglossia | Limb malformations |
|---|---|---|---|---|---|
| S zelely , 1941 | Fibrous gingival adhesions | Unknown | |||
| H ochstetter , 1948 | Fibrous gingival adhesions | Unknown | |||
| H oggins , 1969 | Fibrous gingival adhesions | Unknown | X | X | X |
| M iskinyar , 1979 | Fibrous gingival adhesions | Unknown | X | ||
| R andall , 1984 | Fibrous gingival adhesions | Unknown | |||
| G oodacre , 1990 | Fibrous gingival adhesions laterally | Unknown | X | ||
| H aydar , 2003 | Fibrous gingival adhesions with two mucosal bands | 8 days old, baby girl | |||
| T anrikulu , 2005 | Isolated congenital gum synechia | 10 months old, baby girl | |||
| M ortazavi , 2007 | Isolated congenital gum synechia | 2 months old, baby girl | |||
| Present case, 2010 | Isolated congenital gum synechia | 1st day, girl | X |
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