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Late presentation of congenital muscular torticollis in siblings
Journal of Pediatric Surgery Case Reports 20 (2017) 45e47
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Late presentation of congenital muscular torticollis in siblings Sam Aruputha John, MS, MCh (ped surgery, India) *, Ahamed Faiz ali, MS, MRCS, FAMS (PEDSURG), Rasha kassem, Ali H. Ashkanani, MB, BCH, B.A.O(R.C.S.I) German Board (Facharzt) pedsurgery, Ola Abdelfattah Taher, MSC (ped surgery), Hassan Ahmed Adly, MS (PEDSURG) Department of Pediatric Surgery, Ibn Sina Hospital, Kuwait
a r t i c l e i n f o
a b s t r a c t
Article history: Received 2 February 2017 Received in revised form 10 March 2017 Accepted 14 March 2017 Available online 22 March 2017
Congenital muscular torticollis (CMT) is the third most common congenital musculoskeletal anomaly seen in children. It is a condition in which the affected sternocleidomastoid muscle becomes a fibrosed cord with associated shortening of the muscle. This leads to an ipsilateral side tilt of the neck and contralateral rotation of the face and chin to the other side. Apart from surgery various other treatment modalities are used to treat this condition. We present two siblings of Sudanese nationality with manifestation of Congenital Muscular Torticollis, a male child and female child at the age of 12 and 10 years respectively. Both had CMT of the right sternomastoid muscle. Both were delivered normally. They did not undergo any form of physiotherapy or surgical procedure since birth. Because of this neglected circumstances, the facial asymmetry and deformity were severe. Both the siblings were operated in our hospital with satisfactory result on follow up. Management for CMT starts during the early infancy and physiotherapy is the main treatment for this condition. But some of these CMT children may need surgical intervention to prevent further deterioration of cranial deformity and facial asymmetry. If these children are neglected and present later in life, they will have severe facial deformity with its associated sequelae. These patients are usually managed by other specialities worldwide. But awareness of these conditions is required among paediatric surgeons, as some of these children may be referred to them for treatment. In our case report we have also described various other modalities of treatment for this condition. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: CMT Neglected
1. Case study Two children, a brother and sister from Sudan presented to us at the age of 12 and 10 years respectively with CMT. Both of them had ipsilateral neck tilt on the right side with contra lateral rotation of face and chin to the left side. There was no history of consanguinity or relevant family history. Both the siblings were delivered normally. Also there was no past history of trauma or any other musculoskeletal involvement. There was no previous history of any treatment given. The parents sought medical advice now because the rotational deformity had worsened and hampered the patient's day to today activities. Both the children were moderately built and well nourished. On clinical examination the right
* Corresponding author. E-mail address: [email protected] (S.A. John).
sternocleidomastoid muscle felt like a prominent cord. There was no mass felt along the SCM axis, except for shortening of the muscle. Oral examination showed deviation of uvula to the contra lateral side. There were no other lymph nodes felt on either side of the neck. There was no orthopaedic or neurosurgical involvement. Ultrasound showed moderate fibrosis on the right side of neck along the course of Sternocleidomastoid muscle. Distal unipolar division of both heads of Rt sternocleidomastoid was done under GA. Pt was placed in supine position with a roll placed under the shoulder and a head ring to extend the neck. Head was slightly turned to the left to make RT SCM prominent. After preparing the whole of neck from upper region of mandible bilaterally to both shoulders and the infra clavicular region inferiorly, draping was done. Two sterile large pads are initially places posteriorly at the junction of shoulder and neck. Then drapes were placed along mandible border superiorly, at the clavicular area
http://dx.doi.org/10.1016/j.epsc.2017.03.007 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
46
S.A. John et al. / Journal of Pediatric Surgery Case Reports 20 (2017) 45e47
inferiorly and bilaterally over the posterolateral aspect of the neck. Facilitation of head rotation to ascertain complete release was possible by this draping. A transverse 3 cm incision was made 2 cm above the clavicle over both heads of the Sternocleidomastoid muscle. The medial sternal head which was fibrosed was divided initially using cautery along with division of surrounding fibrous tissues. The fleshy lateral clavicular head was then isolated and divided. Carotid sheath was untouched but some fibrous taught strands associated with the deep cervical fascia investing carotid sheath were also divided medially. Post-operative course was uneventful. Both the children were followed up at the Outpatient after 3 weeks. Both had a good range of neck movements with very mild torticollis. Physiotherapy was started after 24 h post surgery. Free active range of motion exercises was given to attain lateral flexion to the opposite side and rotation to the same side. This was accompanied by passive stretching and neck strengthening exercises under analgesic cover. A normal Soft collar according the age was applied for support and comfort and continued for 6 weeks. Both the children were counseled about postural correction and awareness like, visual feedback with mirror (Self correction in the front of the mirror), about sleeping position (lying on the side and using hard pillows under the operated side with the body straight to maintain the length of the muscles for 10 min). These children were also taught how to do home exercises properly using mirror feedback. They will undergo continue supervised physiotherapy for 6 months until complete and satisfactory recovery is gained. Regular follow up will be then done for 2 years.
physiotherapy with passive stretching exercises and active neck exercise with massage. More than 90% of children with CMT improve with this non-operative treatment by 1 year of age [6]. Surgical intervention is required in cases of contracture of the SCM persisting beyond 1 year of age or if cranio-facial anomaly develops. Canale et al. reported that full recovery of facial asymmetry after the age of 4 difficult to achieve. In Children older than 7yrs, surgical release with appropriate orthosis and structured physiotherapy regime has shown satisfactory results [7]. Treatment with Botulinum type A in infant with refractory CMT has also been used [8]. Older patients require operative interventions more frequently than younger patients. Surgery involves segmental resection or
2. Discussion Torticollis is defined as a twisting, jerking or malrotation of the neck. Most common cause of torticollis are congenital muscular torticollis (CMT) and acquired torticollis [1].CMT is the third most common congenital musculoskeletal anomaly. Unilateral CMT is most common and bilateral presentation are very rare. It has an unknown aetiology and is seen in neonatal period. CMT is a condition in which the SCM is shortened on the affected side, leading to ipsilateral tilt of the neck and contra lateral rotation of the face and chin. This is due to fibrosis of the affected SCM or the trapezius muscle [2].Macdonald divided patients of CMT into two groups, one with the sternocleidomastoid tumor and the other with fibrosis of the sternocleidomastoid without apparent tumor. The presence of other associated skeleton-muscular anomaly in patients with CMT varies from 5 to 10%in the literature. The most commonly associated anomaly is congenital hip dysplasia [3].Acquired torticollis can occur due to inflammation or trauma sustained primarily by SCM in later years. Infection process such as retropharyngeal abscess and space occupying lesions such as hematomas or neoplasms can also give rise to acquired torticollis [4]. CMT initially presents as a hard mass on the SCM of the affected side and accompanied by the above described posture. With time the hard mass subsides and the affected muscle becomes like a tight cord or band [5].In the literature, fetal malposition and birth trauma have been claimed to be responsible for the development of CMT. According to the classic theory, the birth trauma causes stretching and hematoma of the SCM muscle, which is then followed by fibrosis and contraction of the muscle. However the presence of CMT in the infants who were delivered by caesarean or suction made this theory questionable. Apart from the mode of delivery, vascular accident, family history, congenital or acquired central nervous lesions, cervical hemivertebra are other possible causes of torticollis in infants. Muscle atrophy and interstitial fibrosis were regarded as the basic pathological features of CMT. The initial treatment for the patient with CMT should consist of
Pic 1. Lateral rotation of neck. (pre operative).
Pic 2. (postoperative). Mimimal torticollis noted which is improving with physiotherapy.
S.A. John et al. / Journal of Pediatric Surgery Case Reports 20 (2017) 45e47
47
Pic 4. (POST OPERATIVE) No obvious torticollis.
Pic 3. (Pre operative) Prominent SCM noticed. The RT shoulder is raised to compensate for the oblique interpupillary line to make it to a horizontal line.
division of the involved muscle. Microcurrent therapy is given for the children under 3 months of age. Microcurrent therapy sends minute electrical signals into the tissue to restore the normal frequencies in cells [9]. Stealth surgery refers to the endoscopic subcutaneous procedure performed through transaxillary approach. Absence of scar on the neck region is the advantage of this procedure [10]. The efficiency of surgical treatment of neglected CMT after skeletal maturity in an adult remains controversial. Cranio-facial asymmetry is irreversible in adult and the risk of complications after surgery is greater than in children. Moreover in adults complete contracture release through SCM release alone is difficult because of the long standing contractures of adjacent tissues. In older children with late presentation, cervical spine and occipito-facial bones are affected due to long standing deformity. They will definitely need a structured physiotherapy after the
surgery. It may take a while for these children to have complete correction of torticollis. Hence long term post operative follow up is needed in these patients (see Pics 1e4). References [1] Cheng JC, et al. Infantile torticollis a review of 624 cases. J Pediatr Orthop 1994;14:802e8. [2] Robin NH. Congenital muscular torticollis. Pediatr Rev 1996;17:374e5. [3] Pharise C, et al. Neck complain in pediatric emergency department. Pediatr Emerg Care 2009;25:823e6. [4] MacDonald. Sternocleidomastoid tumor and muscular torticollis. J Bone Jt Surg 1969;51:432e43. [5] Demirbilek S, et al. Congenital muscular torticollis and sternomastoid tumor results of non-operative treatment. J Pediatr Surg 1999;34:549e51. [6] Tom, et al. Torticollis in children.Otolaryngol. Head Neck Surg 1991:1051e5. [7] Lepetsos P, et al. Surgical management of congenital torticollis in children older than 7 yrs with an average 10 yrs follow up. J Pediatr Orthop B May 19, 2016. [8] Sin CN, et al. Treatment with botulinum type A in infants with refractory CMT 10 yrs. J Pmrj 2016;07:023. [9] Won K, et al. Efficiency of micro current in CMT. Clin Rehabil 2014 oct;28(10): 983e91. [10] Raut, et al. Sheath surgery for congenital muscular torticollis. Asian J Endos Surg May 2016;9(2):163e6.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Late presentation of congenital muscular torticollis in siblings Sam Aruputha John, MS, MCh (ped surgery, India) *, Ahamed Faiz ali, MS, MRCS, FAMS (PEDSURG), Rasha kassem, Ali H. Ashkanani, MB, BCH, B.A.O(R.C.S.I) German Board (Facharzt) pedsurgery, Ola Abdelfattah Taher, MSC (ped surgery), Hassan Ahmed Adly, MS (PEDSURG) Department of Pediatric Surgery, Ibn Sina Hospital, Kuwait
a r t i c l e i n f o
a b s t r a c t
Article history: Received 2 February 2017 Received in revised form 10 March 2017 Accepted 14 March 2017 Available online 22 March 2017
Congenital muscular torticollis (CMT) is the third most common congenital musculoskeletal anomaly seen in children. It is a condition in which the affected sternocleidomastoid muscle becomes a fibrosed cord with associated shortening of the muscle. This leads to an ipsilateral side tilt of the neck and contralateral rotation of the face and chin to the other side. Apart from surgery various other treatment modalities are used to treat this condition. We present two siblings of Sudanese nationality with manifestation of Congenital Muscular Torticollis, a male child and female child at the age of 12 and 10 years respectively. Both had CMT of the right sternomastoid muscle. Both were delivered normally. They did not undergo any form of physiotherapy or surgical procedure since birth. Because of this neglected circumstances, the facial asymmetry and deformity were severe. Both the siblings were operated in our hospital with satisfactory result on follow up. Management for CMT starts during the early infancy and physiotherapy is the main treatment for this condition. But some of these CMT children may need surgical intervention to prevent further deterioration of cranial deformity and facial asymmetry. If these children are neglected and present later in life, they will have severe facial deformity with its associated sequelae. These patients are usually managed by other specialities worldwide. But awareness of these conditions is required among paediatric surgeons, as some of these children may be referred to them for treatment. In our case report we have also described various other modalities of treatment for this condition. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords: CMT Neglected
1. Case study Two children, a brother and sister from Sudan presented to us at the age of 12 and 10 years respectively with CMT. Both of them had ipsilateral neck tilt on the right side with contra lateral rotation of face and chin to the left side. There was no history of consanguinity or relevant family history. Both the siblings were delivered normally. Also there was no past history of trauma or any other musculoskeletal involvement. There was no previous history of any treatment given. The parents sought medical advice now because the rotational deformity had worsened and hampered the patient's day to today activities. Both the children were moderately built and well nourished. On clinical examination the right
* Corresponding author. E-mail address: [email protected] (S.A. John).
sternocleidomastoid muscle felt like a prominent cord. There was no mass felt along the SCM axis, except for shortening of the muscle. Oral examination showed deviation of uvula to the contra lateral side. There were no other lymph nodes felt on either side of the neck. There was no orthopaedic or neurosurgical involvement. Ultrasound showed moderate fibrosis on the right side of neck along the course of Sternocleidomastoid muscle. Distal unipolar division of both heads of Rt sternocleidomastoid was done under GA. Pt was placed in supine position with a roll placed under the shoulder and a head ring to extend the neck. Head was slightly turned to the left to make RT SCM prominent. After preparing the whole of neck from upper region of mandible bilaterally to both shoulders and the infra clavicular region inferiorly, draping was done. Two sterile large pads are initially places posteriorly at the junction of shoulder and neck. Then drapes were placed along mandible border superiorly, at the clavicular area
http://dx.doi.org/10.1016/j.epsc.2017.03.007 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
46
S.A. John et al. / Journal of Pediatric Surgery Case Reports 20 (2017) 45e47
inferiorly and bilaterally over the posterolateral aspect of the neck. Facilitation of head rotation to ascertain complete release was possible by this draping. A transverse 3 cm incision was made 2 cm above the clavicle over both heads of the Sternocleidomastoid muscle. The medial sternal head which was fibrosed was divided initially using cautery along with division of surrounding fibrous tissues. The fleshy lateral clavicular head was then isolated and divided. Carotid sheath was untouched but some fibrous taught strands associated with the deep cervical fascia investing carotid sheath were also divided medially. Post-operative course was uneventful. Both the children were followed up at the Outpatient after 3 weeks. Both had a good range of neck movements with very mild torticollis. Physiotherapy was started after 24 h post surgery. Free active range of motion exercises was given to attain lateral flexion to the opposite side and rotation to the same side. This was accompanied by passive stretching and neck strengthening exercises under analgesic cover. A normal Soft collar according the age was applied for support and comfort and continued for 6 weeks. Both the children were counseled about postural correction and awareness like, visual feedback with mirror (Self correction in the front of the mirror), about sleeping position (lying on the side and using hard pillows under the operated side with the body straight to maintain the length of the muscles for 10 min). These children were also taught how to do home exercises properly using mirror feedback. They will undergo continue supervised physiotherapy for 6 months until complete and satisfactory recovery is gained. Regular follow up will be then done for 2 years.
physiotherapy with passive stretching exercises and active neck exercise with massage. More than 90% of children with CMT improve with this non-operative treatment by 1 year of age [6]. Surgical intervention is required in cases of contracture of the SCM persisting beyond 1 year of age or if cranio-facial anomaly develops. Canale et al. reported that full recovery of facial asymmetry after the age of 4 difficult to achieve. In Children older than 7yrs, surgical release with appropriate orthosis and structured physiotherapy regime has shown satisfactory results [7]. Treatment with Botulinum type A in infant with refractory CMT has also been used [8]. Older patients require operative interventions more frequently than younger patients. Surgery involves segmental resection or
2. Discussion Torticollis is defined as a twisting, jerking or malrotation of the neck. Most common cause of torticollis are congenital muscular torticollis (CMT) and acquired torticollis [1].CMT is the third most common congenital musculoskeletal anomaly. Unilateral CMT is most common and bilateral presentation are very rare. It has an unknown aetiology and is seen in neonatal period. CMT is a condition in which the SCM is shortened on the affected side, leading to ipsilateral tilt of the neck and contra lateral rotation of the face and chin. This is due to fibrosis of the affected SCM or the trapezius muscle [2].Macdonald divided patients of CMT into two groups, one with the sternocleidomastoid tumor and the other with fibrosis of the sternocleidomastoid without apparent tumor. The presence of other associated skeleton-muscular anomaly in patients with CMT varies from 5 to 10%in the literature. The most commonly associated anomaly is congenital hip dysplasia [3].Acquired torticollis can occur due to inflammation or trauma sustained primarily by SCM in later years. Infection process such as retropharyngeal abscess and space occupying lesions such as hematomas or neoplasms can also give rise to acquired torticollis [4]. CMT initially presents as a hard mass on the SCM of the affected side and accompanied by the above described posture. With time the hard mass subsides and the affected muscle becomes like a tight cord or band [5].In the literature, fetal malposition and birth trauma have been claimed to be responsible for the development of CMT. According to the classic theory, the birth trauma causes stretching and hematoma of the SCM muscle, which is then followed by fibrosis and contraction of the muscle. However the presence of CMT in the infants who were delivered by caesarean or suction made this theory questionable. Apart from the mode of delivery, vascular accident, family history, congenital or acquired central nervous lesions, cervical hemivertebra are other possible causes of torticollis in infants. Muscle atrophy and interstitial fibrosis were regarded as the basic pathological features of CMT. The initial treatment for the patient with CMT should consist of
Pic 1. Lateral rotation of neck. (pre operative).
Pic 2. (postoperative). Mimimal torticollis noted which is improving with physiotherapy.
S.A. John et al. / Journal of Pediatric Surgery Case Reports 20 (2017) 45e47
47
Pic 4. (POST OPERATIVE) No obvious torticollis.
Pic 3. (Pre operative) Prominent SCM noticed. The RT shoulder is raised to compensate for the oblique interpupillary line to make it to a horizontal line.
division of the involved muscle. Microcurrent therapy is given for the children under 3 months of age. Microcurrent therapy sends minute electrical signals into the tissue to restore the normal frequencies in cells [9]. Stealth surgery refers to the endoscopic subcutaneous procedure performed through transaxillary approach. Absence of scar on the neck region is the advantage of this procedure [10]. The efficiency of surgical treatment of neglected CMT after skeletal maturity in an adult remains controversial. Cranio-facial asymmetry is irreversible in adult and the risk of complications after surgery is greater than in children. Moreover in adults complete contracture release through SCM release alone is difficult because of the long standing contractures of adjacent tissues. In older children with late presentation, cervical spine and occipito-facial bones are affected due to long standing deformity. They will definitely need a structured physiotherapy after the
surgery. It may take a while for these children to have complete correction of torticollis. Hence long term post operative follow up is needed in these patients (see Pics 1e4). References [1] Cheng JC, et al. Infantile torticollis a review of 624 cases. J Pediatr Orthop 1994;14:802e8. [2] Robin NH. Congenital muscular torticollis. Pediatr Rev 1996;17:374e5. [3] Pharise C, et al. Neck complain in pediatric emergency department. Pediatr Emerg Care 2009;25:823e6. [4] MacDonald. Sternocleidomastoid tumor and muscular torticollis. J Bone Jt Surg 1969;51:432e43. [5] Demirbilek S, et al. Congenital muscular torticollis and sternomastoid tumor results of non-operative treatment. J Pediatr Surg 1999;34:549e51. [6] Tom, et al. Torticollis in children.Otolaryngol. Head Neck Surg 1991:1051e5. [7] Lepetsos P, et al. Surgical management of congenital torticollis in children older than 7 yrs with an average 10 yrs follow up. J Pediatr Orthop B May 19, 2016. [8] Sin CN, et al. Treatment with botulinum type A in infants with refractory CMT 10 yrs. J Pmrj 2016;07:023. [9] Won K, et al. Efficiency of micro current in CMT. Clin Rehabil 2014 oct;28(10): 983e91. [10] Raut, et al. Sheath surgery for congenital muscular torticollis. Asian J Endos Surg May 2016;9(2):163e6.