Amniotic band syndrome (ABS) is a rare birth defect caused by strands of the amniotic sac that separaqte and entangle digits, limbs, or other parts of the fetus. This condition can cause a variety of problems depending on where strands are located and how tightly they are wrapped. It can cause a variety of congenital anomalies which includes disruption, deformation, and malformations of organs which were intended to develop normally. (13) Different theories have attempted to explain the etiology of amniotic band sequence; however, none has individually been able to support each and every defect observed, so it has been considered to be a multifactorial condition (2). Feto-maternal factors like prematurity, maternal illnesses, low birth weight and maternal drug exposure are predisposing factors for this syndrome (14), although the risk factors are poorly known till now. The approach to ABS is affected by identifying the pre and post natal anomalies. The amniotic bands which are isolated are released by fetoscopy and if it is associated with other anomalies, performing a post-natal surgery repair is recommended. It is also helpful to provide genetic counseling (2). Clinical presentation can classify into four major categories. Constrictive rings/limb defects/neural or spine defects/craniofacial defects. The distribution of amniotic bands is also different, for example extremity involvement can account for more than 70% of cases, but any other anatomic region of the fetus can be involved (umbilical cord, abdomen, head, chest, limb-body-wall-complex) (3). The amniotic bands may be seen confined to the skin or soft tissue and may extend deep into the tissue. If constriction results in amputation in-utero, then amputated part is usually resorbed and not visible after birth.
There are no standard guidelines of management of pregnancy complicated with fetal ABS.
Follow-up, ultrasound scans and intervention are individually tailored and depend on the severity of ABS complications (15).
The in-utero intervention of amniotic band lysis has the potential to slow down the progression of the effect of constriction and restoring normal flow to the downstream organ. There is a hypothesis that fetal limb recovery is more likely after fetal intervention than postnatal recovery because of the plasticity of tissue healing during fetal life. Although fetoscopic intervention may restore blood flow and save the limb, plastic surgery may still be necessary after birth. However, the efficacy of this intervention is unknown, as there are no set criteria for the selection of candidates and a lack of clinical studies (16).
Prognosis depends on the extension and severity of the defects.
Parents should receive counseling from the nursing and clinical interprofessional team.
Osteomyelitis (OM) is a severe neonatal infection which usually has a predisposing factor and necrosis of soft tissue and limb surgeries could be a trigger for late infections. The most significant pathogens are those involved in neonatal sepsis with Staphylococcus aureus, found in 70–90% of culture positive cases. Long lasting sequels could be observed if it is not appropriately treated and even with best treatments, shortening of the affected limbs could be observed. Osteomyelitis may become responsible for permanent sequelae in 6–50% like joint disabilities, change in bone growth due to the damage of the cartilaginous growth plate, limb length discrepancies, arthritis, pathologic fractures, and rarely complete destruction of joints. MRI has become the gold standard to evaluate musculoskeletal infection and the positive rate of MRI in detecting osteomyelitis was 100% (21). It has the capability of assessing the osseous, articular and muscular structures simultaneously and does not require ionizing radiation (18). Increased marrow intensity with surrounding inflammation are the most suggestive signs of OM. Radiography is usually the first radiological investigation in a neonate with suspected OM, although it is reported that only 20% of the radiographs are abnormal at 10–14 days (21).
There were several differential diagnoses in our case presentation such as congenital malformation, protein c and protein s deficiency, DIC due to sever septicemia, amniotic band syndrome, diabetes of mother. According to the normal range of coagulation proteins, the diagnosis of protein c and s deficiency was omitted. We ruled out the septicemia due to the neonate’s sign and symptoms and the lab data. Based on the mother’s history and the lab tests during pregnancy the diagnosis of GDM was well defined. On the other hand, we surely attributed the diagnosis to the amniotic band syndrome since the amniotic band was whirled toward the humerus meanwhile the delivery.