DOI: 10.21276/sjm.

2017

Saudi Journal of Medicine ISSN 2518-3389 (Print)

Scholars Middle East Publishers ISSN 2518-3397 (Online)
Dubai, United Arab Emirates
Website: http://scholarsmepub.com/

Case Report

Congenital Constriction Band Syndrome: A Case Report and Literature Review

Ozinko MO1, Otei OO2, Ekpo RG2, Ebri OI2
1
Burns and Plastic Surgery Unit, Department of Surgery, University of Calabar Teaching Hospital, Calabar, Nigeria
2
Department of Anaesthesia, University of Calabar Teaching Hospital, Calabar, Nigeria

*Corresponding Author:
Ozinko MO
Email: [email protected]

Abstract: Congenital constriction band syndrome is a congenital disorder caused by entrapment of parts in fibrous band
while in utero. A review of the operating register revealed only two cases in three years. We are presenting a case report
of a patient and a literature review pointing out the rarity, aetiology, mechanisms, diagnosis and different treatment
options. The condition has good prognosis.
Keywords: Congenital, band syndrome,

INTRODUCTION
Congenital constriction band syndrome or CASE REPORT
amniotic syndrome band is a congenital disorder caused A 2-year-old child presented with unilateral
by entrapment of fetal parts in fibrous band while in congenital constriction rings at the distal third of the
utero [1]. It is a rare congenital hand anomaly. However legs since birth. Mother attended antenatal clinic at a
the incidence is on the increase in literature. It has Government General Hospital. She had high grade fever
different names which suggest that the real cause of the in the first trimester of pregnancy for which she was
disease is not yet known. However there have been treated for malaria with sulfadoxine- pyrimethamine.
speculations that it could be due to entanglement by No history of bleeding, intake of herbal preparation nor
fragments of ruptured amniotic membrane in utero or it drugs not prescribed by a clinician. No family history of
could be due to vascular disruption to certain parts of hand and foot deformity. Both parents were public
the body. After birth, the diagnosis is clinical but in servants. Further evaluation of child did not reveal any
utero, it is made by using 3D or 4D Doppler scan or other congenital abnormality. Multiple z-plasty was
MRI. Presentation could be varied, usually the limb used for the surgery. The outcome was satisfactory as
deformities are accompanied with other body shown below. Follow up period was one year.
anomalies. Treatment is both conservative and surgical
with good prognosis.

Fig-1: Congenital Constriction Band Syndrome Before Surgery

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Ozinko MO,et al.; Saudi J. Med.; Vol-2, Iss-4(Jun-Jul, 2017):93-97

Fig-2: Congenital Constriction Band Syndrome During Surgery

Fig-3: Congenital Constriction Band Syndrome After Surgery

DISCUSSION trauma which could lead to blunt trauma to the placenta

It was once believed to have an incidence of has been shown to cause amniotic band syndrome in
1:100,000, recent literature support the incidence today some cases .Thirdly, it has been reported that intense
as 1:1200- 1:1500 births [2, 3]. No distinct sex uterine contractions caused by a drug known as
predilection has been determined. Prenatal risk factors misoprostol (a prostaglandin E analogue) has resulted in
associated with amniotic band syndrome include amniotic band syndrome. It has been used to induce
prematurity (<37weeks ), low birth weight(<2500g), abortion. If the pregnancy continues after 6 to 8 weeks
maternal illness in pregnancy or placental haemorrhage of gestation the infant may have the amniotic band
in trauma, attempted abortion in the first trimester are syndrome. Finally, genetic factors may predispose
highly associated findings [2]. Family history seldom infants to the development of amniotic band syndrome
reveals any direct inheritance pattern, since the .The disorder is not expressed unless it is triggered or
syndrome occurs in no particular association with activated under certain circumstances such as particular
known genetic or chromosomal disorders. Karotypes environmental factors (multifactorial inheritance).
are virtually always normal and the syndrome is almost Researchers have discussed the possibility that genetic
always sporadic in nature. The incidence of factors influence the development of amniotic band
malformation seen in the hand is two times as common syndrome in certain cases [4-6].
as accompanied foot deformities [3].
The mechanisms of congenital constriction
Aetiologically, several factors have been band are complex and controversial. Several different
suggested to be the cause of amniotic band syndrome. theories have been proposed to explain the complex
Firstly, damage to the amniotic sac has been implicated mechanisms that underlie amniotic band syndrome. The
as a cause of amniotic band syndrome under the two theories are the extrinsic and intrinsic ones. The
extrinsic theory. The exact cause of the amniotic tear is extrinsic or amniotic band theory for the development
not known .Secondly, environmental factors have been of amniotic band syndrome is that strands of tissue
implicated. Some researchers speculated that abdominal separate from the inner layer (amnion) of the amniotic

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Ozinko MO,et al.; Saudi J. Med.; Vol-2, Iss-4(Jun-Jul, 2017):93-97
sac. The amniotic sac is composed of two main layers – subcutaneous tissue. [16] Another pattern associated
the outer layer ( chorion) and the inner layer called the with amniotic constriction band is referred to as limb-
amnion .According to this theory, amniotic band body- wall complex [17, 18]. The infants have
syndrome occurs when the inner layer (amnion )of the abdominal wall defects with defects of the arms and
amniotic sac ruptures , thereby exposing the fetus to legs and other abnormalities. The infant may present
strands of fibrous tissue that may float freely in the with encephalocele, omphalocele, through a fissure in
amniotic fluid and still remain partially attached to the the abdominal wall (abdominoschisis) or the chest wall
amniotic sac. The bands of tissue can wrap and constrict (thoracoschisis) and a variety of defects affecting the
the fingers, toes, arms, legs, and other parts of the arms and legs [19]. Additional abnormalities can occur
developing fetus. The symptoms that occur due to in infants with limb –body –wall complex [20]. A third
amniotic bands depend on specific part of the body pattern associated with amniotic band syndrome
affected by these strands of tissue and how tightly they involves craniofacial abnormalities such as incomplete
have wrapped around the body part. The second theory closure of the roof of the mouth (cleft palate), cleft lip,
is the intrinsic or the vascular disruption theory which facial cleft, microphthalmia, cloanal atresia and
was proposed because some researchers noted that malformations affecting the size and shape of the skull
while the above theory explains some cases of amniotic [21]. In severe cases the neural tube defects could lead
band syndrome it is insufficient to explain all cases. The to anencephaly. In some cases, serious complications of
external theory fails to explain why there is intact the arms and legs called terminal transverse limb
amniotic sac in some infants with amniotic band deficiencies. In such cases affected infants may be
syndrome. Why there are a high number of missing a portion or all of a limb, ranging from one
malformations affecting internal organs in some cases finger or toe to an entire arm or leg. In 1961, Paterson
and why some infants have defects of the body not used a classification that is still widely used today [22,
affected by amniotic constriction bands. The intrinsic 23]. The classification is as follows: (A) Simple ring
theory attributes the development of amniotic band constriction; (B) Ring constriction accompanied by
syndrome to impaired blood flow to specific parts of the deformity of the distal part with or without
developing fetus (vascular disruption). The exact cause lymphedema;(C) Ring constrictions accompanied by
of impaired blood supply is not known but it is thought fusion of distal parts ranging from mild to severe
to be vascular injury leading to haemorrhage with acrosyndactyly, and (D)Uterine amputation.
eventual tissue loss in the affected part of the body. The
intrinsic theory attributes the presence of constriction Diagnosis of amniotic band syndrome after
bands as a secondary effect of the impaired blood flow child birth is clinical. However, the diagnosis in
and subsequent damage to the fetus. In a 1987 article in intrauterine life is often difficult. 3D ultrasonography
the journal of Tetralogy, Webster et al demonstrated and Magnetic Resonance Imaging can be used for more
this theory of vascular disruption with an intact amnion detailed and accurate diagnosis of bands and the
in animal models [6]. resulting damage or danger to the fetus. Although the
earliest may be difficult to detect by ultrasound and are
The signs and symptoms of amniotic band more often diagnosed by the effects they have on fetal
syndrome vary greatly from one infant to another [7]. anatomy. The visualization of amniotic bands attaching
Some infants develop severe and even life-threatening to fetus with restriction of motion, constrictions rings on
complications. No two infants can develop the same the extremities and irregular amputation of digits with
number of or types of abnormalities. [8] Amniotic band syndactyly, on prenatal ultrasonography is diagnostic.
syndrome develops at any point during the first 20 The latest ultrasound techniques of 3D and 4D
weeks of pregnancy [9]. Generally, the most severe ultrasound contribute to more sensitive prenatal
complications occur when amniotic band syndrome diagnosis of the condition. In complicated cases, fetal
develops early in the first trimester [10, 11]. Several MRI may be helpful.
different patterns have been identified with amniotic
band syndrome. The three most common patterns are Surgery is the mainstay of treatment. The goal
amniotic band syndrome characterized by one or more of surgery is to release the constriction rings and
limbs being affected; limb-body-wall complex; and acrosyndactyly so that the child may have an improved
amniotic band syndrome characterized by abnormalities appearance and obtain basic hand functions such as grip
of the head and face (craniofacial abnormalities), and pinch. Acute exacerbation of the distal edema or
certain birth defects of the brain and spinal cord and nerve palsy is an indication for emergent excision for
serious malformation of the arms and legs [12-14]. the ring to relieve the tension [7, 22]. Distal edema can
Most infants with amniotic band syndrome have some rapidly progress in the first week after birth. In these
form of deformity of the arms and legs or fingers and cases, expeditious release can resolve this condition.
toes. One or more limbs may be affected. Upper limbs Correction of constriction ring can be performed at
may be affected more than the lower limbs [15]. Some 3months of age if necessary [9]. In cases of
infants may have bands of tissues that encircle or acrosyndactyly surgical treatment is performed between
gradually constrict certain fetal areas. These bands can 6 and 12 months of age [9, 23].
cause superficial constrictions without damage to the

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Regarding anaesthetic management, the child What is the expected outcome? The resection
should be evaluated properly for any hidden systemic of constriction ring and the correction of acrosyndactyly
anomaly during pre-anaesthetic checkup. Anaesthetist can improve both appearance and function. In the case
should always be prepared for difficult airway in such of severe deformity with distal finger amputations and
cases. In addition to deformities in amniotic band acrosyndactyly, the limited functional benefit with
syndrome, Paediatric airway is challenging because of reconstruction should be weighed against the risk of
unique anatomy (smaller size vocal cord between 9-24, scar formation [29]. There is satisfactory prognosis.
with an anterior angulation, a large and floppy
epiglottis, a large occiput) and physiology (frequent In conclusion, congenital constriction band
upper airway obstruction under general anaesthesia, syndrome is a rare condition in our facility. However,
higher metabolism and faster desaturation during period literature search has revealed that the condition is on the
of apnea). Difficult airway cart with different sizes of increase. Successful results can be achieved with
endotracheal tubes, laryngeal tubes, laryngeal mask excision of the fibrotic band and the correction of the
airway, paediatric fiberscope, etc were kept ready subcutaneous deficiency under the constriction. Infants
although they were not used. Adequate covering of with congenital constriction ring deformities should be
extremities with warm blankets, elevation of room referred for surgery early, particularly if there is
temperature and warm infusion fluids were measures vascular compromise.
taken to prevent hypothermia. Extubation should be
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