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Anomalies of the first branchial cleft
Anomalies of the First Branchial Cleft By A. J.
Dougall
THE L O W E R P A R T of the face and anterior part of the neck are derived 1 from the branchial arches and clefts of an early embryonic stage. Failure of complete fusion of the second branchial cleft leads to the relatively c o m m o n group of anomalies usually referred to simply as branchial cyst, sinus, or fistula. More rarely the first branchial cleft may also fail to close completely producing a similar group of conditions. The upper end of the first branchial fistula extends from and communicates with the lateral end of the external auditory canal. It runs downwards and medially in the subcutaneous tissues to emerge in the area in front of and below the angle of the mandible. Its relation to the facial nerve is discussed below. The sinus has the same course but its upper end stops short of the external auditory canal. In both, the upper end is usually surrounded by cartilage which is continuous with that of the lateral end of the auditory canal, l The cyst is usually situated at the lower end of a sinus or fistula before it has ever discharged. Lincoln 2 carried out a full review of the embryology, collected 31 cases from the literature, and added one more. Isolated case reports have appeared since then 3,4 and three more are presented here. CASE HISTORIES Case 1. A girl of 3 yr presented with a chronic right s u b m a n d i b u l a r sinus and a history o f a right submandibular abscess which had been incised at the age of a few weeks and again at 9 mo. The sinus dried up after s o m e delay and the tract was then excised t h r o u g h two incisions. It. extended from a point below and in front of the angle of the jaw, upwards, to end blindly deep to the skin of the anterior wall of the external auditory canal. T h e upper part lay deep to the facial nerve. On microscopy of the specimen it was seen that the sinus was lined with keratinized stratified s q u a m o u s epithelium. Hair follicles, sebaceous glands, a n d sweat glands were present. In places this lining was interrupted by areas of granulation tissue containing giant cells a n d there was a piece of cartilage at the upper end. The w o u n d healed satisfactorily. She failed to attend for follow-up. Case 2. A female infant was first seen at the age o f 16 m o with an infected sinus in the left s u b m a n d i b u l a r area. A m o n t h later she developed a discharge from the left ear. No opening in the external auditory canal was seen, but she was found to have a perforated eardrum. F o u r m o n t h s later when both neck and ear were dry, a tract was excised t h r o u g h an incision r o u n d the m o u t h of the sinus in the neck. T h e upper end was close to the auditory canal b u t did not appear to c o m municate with it. Microscopic examination of the specimen indicated that the lining was mainly c o m p o s e d of granulation tissue, but in places it consisted o f skin. F u r t h e r infection occurred a n d the upper end was explored t h r o u g h an incision behind the ear 14 m o later. N o further r e m n a n t of a branchial sinus was found, but since that operation there has been no recurrence. Case 3. A girl of 6 m o presented with chronic discharge from a site in the left s u b m a n d i b u l a r area where a swelling h a d been incised 3 m o previously. A sinogram was carried out (Fig. 1) which
From the Royal Hospital for Sick Children and Stobhill General Hospitals, Glasgow, Scotland. A . J . Dougall, F.R.C.S., E d . Senior Paediatric Surgical Registrar, Stobhill General Hospital,
Glasgow, Scotland. Address for reprint requests." A. J. Dougall, F.R.C.S., Stobhill General Hospital, Glasgow, G21 3 U W Scotland. 9 1974 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 9, N o 2 (April), 1974
203
204
A.J. DOUGALL
Fig. 1.
Sinogram in Case 3.
showed that the upper end opened into the anterior wall of the external auditory canal just inside its lateral end. The whole sinus was exposed t h r o u g h a linear incision with ellipses at top and bottom, and removed intact. Its upper end lay deep to the facial nerve. The w o u n d became infected but healed with a good long-term result. The tract was found on microscopy to be lined with skin, except in one area where it was replaced by granulation tissue. DISCUSSION
Anomalies of the first branchial cleft have been divided into two groups. 4 In Type I it is suggested that cell rests are buried during closure of the cleft. These develop into a cyst, situated in, or very close to the parotid gland which presents in early or middle adult life as a painful swelling. The cyst is intimately related to the facial nerve and is lined with simple s q u a m o u s epithelium. A b o u t 20 of these have been recorded. These will not be considered further here. The Type II anomaly is the variety with which this paper is concerned. It is generally accepted that it is the result of partial failure of fusion of the cleft. ~ It presents as a discharging sinus in the upper neck in infancy or childhood and is lined with true skin. A b o u t 35 of these have been recorded prior to this publication. The chief points of interest in the m a n a g e m e n t of a child with a first branchial arch a n o m a l y are: (1) the diagnosis; (2) its complete removal; (3) its relation to the facial nerve. (1) S u b m a n d i b u l a r abscesses are c o m m o n in childhood, s and it is not practicable to investigate all of these as potential first-arch sinuses. If there is a recurrence at a nonmidline site, or the discharge becomes chronic, the diagnosis should be considered. Even on the initial presentation the points in which it m a y differ from an abscess are: (1) a previous history of discharge from that site without evidence of inflammation; (2) the presence of a clear or relatively nonpurulent discharge; (3) the absence of a lot of deep induration; and (4) the possible coexistence of ear infection or discharge. If any of these features are present or if an abscess recurs at the same site it is advisable to try and confirm or refute the presence of a tract running up from the neck lesion, by passing a probe. It m a y be necessary to give a general anesthetic for this purpose. A sino-
FIRST BRANCHIAL CLEFT
205
g r a m is w o r t h a t t e m p t i n g a l t h o u g h the n u m b e r w h i c h a r e fully o u t l i n e d b y this m e t h o d is said to be small. 2 (2) T h e sinus or fistula has to be r e m o v e d c o m p l e t e l y if r e c u r r e n c e is to be a v o i d e d . This r e q u i r e s d i s s e c t i o n r i g h t u p to t h e e x t e r n a l a u d i t o r y c a n a l alt h o u g h in a b o u t t w o t h i r d s o f the p a t i e n t s no c o m m u n i c a t i o n w i t h t h a t c a n a l will be d e m o n s t r a b l e . A s m o s t p a t i e n t s will p r e s e n t w i t h an i n f e c t e d sinus, it m a y be n e c e s s a r y to t r e a t the lesion c o n s e r v a t i v e l y with a n t i b i o t i c s i n i t i a l l y . A r a d i c a l excision s h o u l d be d e f e r r e d u n t i l t h e a c u t e i n f l a m m a t o r y e p i s o d e h a s settled. O p t i m u m e x p o s u r e is o b t a i n e d t h r o u g h an e l l i p t i c a l i n c i s i o n a r o u n d the o p e n i n g in t h e neck e x t e n d e d u p w a r d as far as t h e e a r lobe. T h e t r a c t c a n t h e n be d i s s e c t e d up in full view a n d t h e facial n e r v e identified if it is s i t u a t e d close to t h e t r a c t . 6 (3) T h e u p p e r p a r t o f the t r a c t m a y lie s u p e r f i c i a l to the facial n e r v e o r d e e p to it as in C a s e s 1 a n d 3. S o m e t i m e s t h e e x a c t p o s i t i o n o f the n e r v e h a s n o t b e e n e s t a b l i s h e d , b u t in 22 p a t i e n t s in w h i c h it has been, t h e t r a c t h a s been s u p e r ficial to the n e r v e in 12, a n d deep to t h e n e r v e in 10. 2,7 S p l i t t i n g o f t h e m a i n nerve t r u n k to encircle t h e fistula has also been r e c o r d e d . 8 T h a t c h i l d is also t h e o n l y r e c o r d e d i n s t a n c e o f a fistula with t w o l o w e r o p e n i n g s so t h a t it h a d t h e s h a p e o f an i n v e r t e d " T . "
SUMMARY T h r e e cases o f first b r a n c h i a l a r c h a n o m a l i e s in y o u n g c h i l d r e n a r e p r e s e n t e d . T h e t w o different t y p e s o c c u r r i n g , r e s p e c t i v e l y , in c h i l d r e n a n d a d u l t s a r e described, and some points concerning the diagnosis and treatment of the f o r m e r are d i s c u s s e d .
ACKNOWLEDGMENTS I am grateful to Mr. J. C. Grant and Mr. J. C. Mustard6 for permission to publish these cases, and to Dr. E. M. Sweet for the sinogram. REFERENCES
1. Bill AH: Branchiogenic cysts and sinuses, in Mustard WT, Ravitch MM et al (eds): Paediatric Surgery, vol I. Chicago, Year Book Medical Publishers, 1969 2. Lincoln JCR: Cervico auricular fistulae. A review of published cases with a report. Arch Dis Child, 40:218, 1965 3. Whitson TC: Anomaly of the first branchial cleft. Case report. Plast Reconstr Surg 41:493, 1968 4. Arnot RS: Defects of the first branchial cleft. S Afr Med J 9:93, 1971
5. Scobie WG: Acute suppurative adenitis in children, a review of 964 cases. Scot Med J 14:352, 1969 6. Lindsay WK: Anomalies of the first branchial cleft, in Mustard6, JC (Ed.) Plastic Surgery in Infancy and Childhood, Edinburgh, Livingstone, 1971 7. Bill AH, Vadheim JL: Cysts, sinuses and fistulas of the neck arising from the first and second branchial clefts. Ann Surg 142:904, 1955 8. Cryrnble B, Braithwaite F: Anomalies of the first branchial cleft. Br J Surg 51:420, 1964
Dougall
THE L O W E R P A R T of the face and anterior part of the neck are derived 1 from the branchial arches and clefts of an early embryonic stage. Failure of complete fusion of the second branchial cleft leads to the relatively c o m m o n group of anomalies usually referred to simply as branchial cyst, sinus, or fistula. More rarely the first branchial cleft may also fail to close completely producing a similar group of conditions. The upper end of the first branchial fistula extends from and communicates with the lateral end of the external auditory canal. It runs downwards and medially in the subcutaneous tissues to emerge in the area in front of and below the angle of the mandible. Its relation to the facial nerve is discussed below. The sinus has the same course but its upper end stops short of the external auditory canal. In both, the upper end is usually surrounded by cartilage which is continuous with that of the lateral end of the auditory canal, l The cyst is usually situated at the lower end of a sinus or fistula before it has ever discharged. Lincoln 2 carried out a full review of the embryology, collected 31 cases from the literature, and added one more. Isolated case reports have appeared since then 3,4 and three more are presented here. CASE HISTORIES Case 1. A girl of 3 yr presented with a chronic right s u b m a n d i b u l a r sinus and a history o f a right submandibular abscess which had been incised at the age of a few weeks and again at 9 mo. The sinus dried up after s o m e delay and the tract was then excised t h r o u g h two incisions. It. extended from a point below and in front of the angle of the jaw, upwards, to end blindly deep to the skin of the anterior wall of the external auditory canal. T h e upper part lay deep to the facial nerve. On microscopy of the specimen it was seen that the sinus was lined with keratinized stratified s q u a m o u s epithelium. Hair follicles, sebaceous glands, a n d sweat glands were present. In places this lining was interrupted by areas of granulation tissue containing giant cells a n d there was a piece of cartilage at the upper end. The w o u n d healed satisfactorily. She failed to attend for follow-up. Case 2. A female infant was first seen at the age o f 16 m o with an infected sinus in the left s u b m a n d i b u l a r area. A m o n t h later she developed a discharge from the left ear. No opening in the external auditory canal was seen, but she was found to have a perforated eardrum. F o u r m o n t h s later when both neck and ear were dry, a tract was excised t h r o u g h an incision r o u n d the m o u t h of the sinus in the neck. T h e upper end was close to the auditory canal b u t did not appear to c o m municate with it. Microscopic examination of the specimen indicated that the lining was mainly c o m p o s e d of granulation tissue, but in places it consisted o f skin. F u r t h e r infection occurred a n d the upper end was explored t h r o u g h an incision behind the ear 14 m o later. N o further r e m n a n t of a branchial sinus was found, but since that operation there has been no recurrence. Case 3. A girl of 6 m o presented with chronic discharge from a site in the left s u b m a n d i b u l a r area where a swelling h a d been incised 3 m o previously. A sinogram was carried out (Fig. 1) which
From the Royal Hospital for Sick Children and Stobhill General Hospitals, Glasgow, Scotland. A . J . Dougall, F.R.C.S., E d . Senior Paediatric Surgical Registrar, Stobhill General Hospital,
Glasgow, Scotland. Address for reprint requests." A. J. Dougall, F.R.C.S., Stobhill General Hospital, Glasgow, G21 3 U W Scotland. 9 1974 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 9, N o 2 (April), 1974
203
204
A.J. DOUGALL
Fig. 1.
Sinogram in Case 3.
showed that the upper end opened into the anterior wall of the external auditory canal just inside its lateral end. The whole sinus was exposed t h r o u g h a linear incision with ellipses at top and bottom, and removed intact. Its upper end lay deep to the facial nerve. The w o u n d became infected but healed with a good long-term result. The tract was found on microscopy to be lined with skin, except in one area where it was replaced by granulation tissue. DISCUSSION
Anomalies of the first branchial cleft have been divided into two groups. 4 In Type I it is suggested that cell rests are buried during closure of the cleft. These develop into a cyst, situated in, or very close to the parotid gland which presents in early or middle adult life as a painful swelling. The cyst is intimately related to the facial nerve and is lined with simple s q u a m o u s epithelium. A b o u t 20 of these have been recorded. These will not be considered further here. The Type II anomaly is the variety with which this paper is concerned. It is generally accepted that it is the result of partial failure of fusion of the cleft. ~ It presents as a discharging sinus in the upper neck in infancy or childhood and is lined with true skin. A b o u t 35 of these have been recorded prior to this publication. The chief points of interest in the m a n a g e m e n t of a child with a first branchial arch a n o m a l y are: (1) the diagnosis; (2) its complete removal; (3) its relation to the facial nerve. (1) S u b m a n d i b u l a r abscesses are c o m m o n in childhood, s and it is not practicable to investigate all of these as potential first-arch sinuses. If there is a recurrence at a nonmidline site, or the discharge becomes chronic, the diagnosis should be considered. Even on the initial presentation the points in which it m a y differ from an abscess are: (1) a previous history of discharge from that site without evidence of inflammation; (2) the presence of a clear or relatively nonpurulent discharge; (3) the absence of a lot of deep induration; and (4) the possible coexistence of ear infection or discharge. If any of these features are present or if an abscess recurs at the same site it is advisable to try and confirm or refute the presence of a tract running up from the neck lesion, by passing a probe. It m a y be necessary to give a general anesthetic for this purpose. A sino-
FIRST BRANCHIAL CLEFT
205
g r a m is w o r t h a t t e m p t i n g a l t h o u g h the n u m b e r w h i c h a r e fully o u t l i n e d b y this m e t h o d is said to be small. 2 (2) T h e sinus or fistula has to be r e m o v e d c o m p l e t e l y if r e c u r r e n c e is to be a v o i d e d . This r e q u i r e s d i s s e c t i o n r i g h t u p to t h e e x t e r n a l a u d i t o r y c a n a l alt h o u g h in a b o u t t w o t h i r d s o f the p a t i e n t s no c o m m u n i c a t i o n w i t h t h a t c a n a l will be d e m o n s t r a b l e . A s m o s t p a t i e n t s will p r e s e n t w i t h an i n f e c t e d sinus, it m a y be n e c e s s a r y to t r e a t the lesion c o n s e r v a t i v e l y with a n t i b i o t i c s i n i t i a l l y . A r a d i c a l excision s h o u l d be d e f e r r e d u n t i l t h e a c u t e i n f l a m m a t o r y e p i s o d e h a s settled. O p t i m u m e x p o s u r e is o b t a i n e d t h r o u g h an e l l i p t i c a l i n c i s i o n a r o u n d the o p e n i n g in t h e neck e x t e n d e d u p w a r d as far as t h e e a r lobe. T h e t r a c t c a n t h e n be d i s s e c t e d up in full view a n d t h e facial n e r v e identified if it is s i t u a t e d close to t h e t r a c t . 6 (3) T h e u p p e r p a r t o f the t r a c t m a y lie s u p e r f i c i a l to the facial n e r v e o r d e e p to it as in C a s e s 1 a n d 3. S o m e t i m e s t h e e x a c t p o s i t i o n o f the n e r v e h a s n o t b e e n e s t a b l i s h e d , b u t in 22 p a t i e n t s in w h i c h it has been, t h e t r a c t h a s been s u p e r ficial to the n e r v e in 12, a n d deep to t h e n e r v e in 10. 2,7 S p l i t t i n g o f t h e m a i n nerve t r u n k to encircle t h e fistula has also been r e c o r d e d . 8 T h a t c h i l d is also t h e o n l y r e c o r d e d i n s t a n c e o f a fistula with t w o l o w e r o p e n i n g s so t h a t it h a d t h e s h a p e o f an i n v e r t e d " T . "
SUMMARY T h r e e cases o f first b r a n c h i a l a r c h a n o m a l i e s in y o u n g c h i l d r e n a r e p r e s e n t e d . T h e t w o different t y p e s o c c u r r i n g , r e s p e c t i v e l y , in c h i l d r e n a n d a d u l t s a r e described, and some points concerning the diagnosis and treatment of the f o r m e r are d i s c u s s e d .
ACKNOWLEDGMENTS I am grateful to Mr. J. C. Grant and Mr. J. C. Mustard6 for permission to publish these cases, and to Dr. E. M. Sweet for the sinogram. REFERENCES
1. Bill AH: Branchiogenic cysts and sinuses, in Mustard WT, Ravitch MM et al (eds): Paediatric Surgery, vol I. Chicago, Year Book Medical Publishers, 1969 2. Lincoln JCR: Cervico auricular fistulae. A review of published cases with a report. Arch Dis Child, 40:218, 1965 3. Whitson TC: Anomaly of the first branchial cleft. Case report. Plast Reconstr Surg 41:493, 1968 4. Arnot RS: Defects of the first branchial cleft. S Afr Med J 9:93, 1971
5. Scobie WG: Acute suppurative adenitis in children, a review of 964 cases. Scot Med J 14:352, 1969 6. Lindsay WK: Anomalies of the first branchial cleft, in Mustard6, JC (Ed.) Plastic Surgery in Infancy and Childhood, Edinburgh, Livingstone, 1971 7. Bill AH, Vadheim JL: Cysts, sinuses and fistulas of the neck arising from the first and second branchial clefts. Ann Surg 142:904, 1955 8. Cryrnble B, Braithwaite F: Anomalies of the first branchial cleft. Br J Surg 51:420, 1964