THE
TREATMENT
OF
COMMUNICATING HYDROCEPHALUS
h~[. G. P ~ E a ~ , M.D. MIL~VAUKEE, WIS. results of t r e a t m e n t of hydrocephalus in children have been T HsoE unsatisfactory t h a t most p a r e n t s are advised against interference. Buoy, 1 in a recent contribution on the subject, concluded: " I t is to be regretted that such extreme pessimism is w a r r a n t e d in connection with the surgical t r e a t m e n t of hydroeepha]~us, yet such is the case. A t the present time even the best-intentioned surgery can hardly serve other t h a n to deny to some few cases the possibility of a spontaneous recovers by subjecting them to an operation and, a surgical death." Scarff 2 has recently revived interest in the surgical t r e a t m e n t of hydrocephalus b y r e p o r t i n g on a comparatively large series Of cases treated by destruction of the choroid plexus. H e has improved upon the techniques of D a n d y ~ and of P u t n a m 4 and stimulates a new optimism. Searff did forty-eight operations on t w e n t y children and reported a mortality of 15 per cent. The results are reported as satisfactory in ten children, or in 50 per cent of the cases, and as. unsatisfactory in 35 per cent. About 20 per cent of the original group developed with a normal mentality. I t is m y purpose in this report to ~ireet attention to: a simple and safe surgical t r e a t m e n t of hydrocephalus proposed by Hiller in 19332 My series of cases is small, but it has been followed a sufficiently long time to permit of an evaluation of the procedure. Hiller suggested the insertion of a waxed silk seton into the lumbar spinal canal with one end buried in the adjacent subcutaneous tissue. To facilitate the introduction of the seton Hiller described a simple modification of a standard spinal puncture needle. The hub of a 17~ gauge spinal needle is heated a n d slid down the shaft of t h e needle just f a r enough to leave the proximal end of the shaft level with the top of the hub. The top of the styler is bored to accommodate ttie portion of the shaft which then extends out. A No. 9 tonsil wire twice the length of the needle is added. A length of No. 1 well-waxed silk completes the equipment. I have modified the needle b y widening the proximal end of the shaft into a funnel shape. I have also m a r k e d the wire to indicate when the tip is level with the tip of the needle. The tip of the wire has also been made slightly bulbous and the distal end roughened with a cross to hold the M~ot better. W i t h the equipment sterilized the patient is p r e p a r e d in the usual manner. The spinal needle is inserted with the child u n d e r local or general anesthesia. The excess fluid is allowed to drain slowly. The knotted end of the waxed silk t h r e a d is then introduced into the lumen 690
PETE,]~]YIAI~ :
CO.IVLI~[UlXTICATINGHYDRO.CF~PI~IALIJS
691
of the needle and is started with the stylet. The wire is then introduced and the knot is pushed through the needle into the spinal subarachnoid space. With the proximal end of the thread free, the needle is withdrawn holding the wire in place. Next, the wire is pulled out leaving the knot and the seton in p l a c e . The proximal end of the seton is now cut off at the skin level, and the skin is moved over the cut end of the thread. The patient is kept in bed with his head elevated for twentyfour hours. PATiEnTS TI~EATED I n 1~35 I became interested in Hiller's suggestion and treated my first patient (Case 1). The results were satisfactorT, and four more patients have now been similarly treated. A brief r~sum6 of these cases is appended. There has been no mortality, no secondary infection, and the results have been unifol~nly good. Two patients have had one seton each, one patient has had two, and two patients have had seven setons. The two patients who have required multiple setons developed recurrences of hydrocephalus following falls on the head. Apparently the injuries were sufficient to disturb the delicate balance or equilibrium provided by the seton drainage. It is quite possible that the setons became encased and had ceased to function. CO1VilVIE~T
In any report of treatment of hydrocephalus it must be fully realized that some patients make spontaneous recoveries. I am quite aware of this fact and have seen cases of arrested development in hydrocephalus. However, the complete control o f recurrences in two of the patients reported with the insertion of new" setons demonstrates the value and effectiveness of the technique described. One patient has been kept under observation for seven years, one six years, one four years, one two years, and one six months. ~M1 of the patients" were thoroughly studied before operation. This study included spinal and ventricle punctures. The, injection Of dye into the ventricles and a study of the dye absorption and excretion into the urine were done. SUM1KARu
A simple technique is described for the treatment of communicating hydrocephalus in children. The procedure consists in the introduction of a waxed silk seton into the eerebrospina] subarachnoid space with the proximal end of the seton buried under the skin. The method is simple and free of objections. Additional setons may be introduced whenever indicated. A simple needle is described to facilitate the introduction of the seton. In a series of five cases there has been no mortality and no secondary infection. CASE REPORTS CASE 1 . - - F . J. (M~0.tt. 36761), a n 8-month-old i n f a n t , was a d m i t t e d to Milwaukee C h i l d r e n ' s H o s p i t a l 1~o~. 4~ 1935. H e h a d b e e n well u n t i l two weeks before
692
THE JOURlqAL OF PEDIATRICS
when he awakened, held his head, and screamed. One week later he became blind. This was the m o t h e r ' s first pregnancy and the i n f a n t was delivered by forceps a f t e r a long difficult labor. There was resulting skull fracture and lacerations of the scalp. Examination.--Multiple healed scars on the forehead and scalp. Head large, circumference, 47.5 cm.; chest, 45 cm.; anterior fontanel, 3 by 4 cm., tense. The pupils were dilated a n d did not react to light, and there was apparently no vision. The sclerae showed well above the pupils. Both optic discs were pale and edematous. The spinal and ventricular punctures revealed clear fluid under increased pressure. Pheno]sulphonphthalein injected into the lateral ventricles was observed in the lumbar fluid within fifteen minutes. Daily spinal punctures were done until l~ovember 20 when the p a t i e n t ' s vision had completely returued. On Nov. 22, 1935, a seton was inserted into the lumbar space and the patient was discharged Nov. 26, 1935. He was seen Dec. 6, 1935, when his head circumference was 49 cm. and his vision good. He was readmitted April 7, ]936, because of restlessness and sleeplessness. " His head measured 50 era., and Macewen's sign was positive. There was some strabismus. The spinal fluid was under increased pressure. I t was drained off and a second seton was inserted. On Oct. ]1, 1936, the symptoms had recurred and the patient was readmitted. The head circumference was 54 cm. The spinal fluid was under increased pressure; 75 c.c. were drained off and a third seton was inserted. On Sept. 10, 1937, he had a cold and otitis media on the fight side. The membrana tympani was incised Sept. 13, 1937; he had a bulging fontanel. Head circumference, 57 era.; chest, 53 cm.; spinal fluid under increased pressure. The fluid was drained off and a seton inserted. On l~ay 17, 1938, he fell and struck his forehead on concrete walk. Examination revealed a large hematoma on the forehead. Spinal fluid was under increased pressure and ground glass in appearance containing many red blood cells. X-ray showed a fracture of the right frontal bone extending up 4 cm. from the orbit. He made a complete recovery. On _Aug. 21, 1938, he fell and struck his forehead. /-Ie had a severe frontal headache, vomiting, and fever, On Aug. 28, 1938, the head circumference was 58.5 era., and Maeewen~s sign was positive. Spinal fluid was under increased pressure. The fluid was drained eft and a seton inserted. On Sept. 18, 1938, he fell and struck his head. He then had severe frontal headaches, nausea, and vomiting. On Sept. 20, 1938, the head circumference was 59 cm. The spinal fluid was under increased pressure. I t was drained off and a seton was inserted. On Feb. ]9, 1940, the child had had an occasional attack of vomiting and had developed an unsteady gait. Examination revealed a precocious mental development and adiadokokinesis. The head circumference was 61.5 cm. The scalp veins were not prominent. Spinal fluid was under increased pressure, and 76 c.c. were removed. On Feb. 23, 1940, a seton was inserted. On Aug. 5, 194], the child weighed 65 pounds and measured 45 inches in height. His gait was slightly unsteady and he had some adiadokokinesis. His blood pressure was 112/80. His mental development had been precocious. Head circumference, 63.5 em. ; chest, 65 cm. ; abdomen, 65 cm. Aug. ]8, 1942--child had been well. His weight was 69 pounds; height, 47 inches; head circumference, 63 cm.; chest, 69 cm.; abdomen, 67 cm. The gai~ was unsteady, but the child had not fallen. The mentality was excellent. The head circumference had not increased for one year; previously it had increased only ].5 cm. in two years. CASE 2 - - J . ]3. (M.C.H. 42534), an 8-month-old infant, was admitted to the Milwaukee Children's I-Iospital Aug. 23, 1938. She had apparently had meningitis one month before and had three attacks of ~'arching of the b a c k ' ' following. The mother also noted a marked increase in the size of the head in the month preceding admission. Examination revealed a large head, circumference of 51 cm., with large, dilated scalp veins. The selerae were visible above the pupils. The spinal
P~TE~IVfAI~ :
COlYII~UNICATII~G ttYDROC~tF..ALUS
693
fluid was under increased pressure but otherwise normal. Phenolsulphonphthalein was injected into the right ventricle. There was no dye recovered in the lumbar spinal fluid in one hour. Only 10 per cent of the dye was recovered in the urine in nine hours. On Aug. 26, 1938, no dye was recovered in the eisternal fluid. On Sept. 1, 1938, a lumbar spinal puncture was done. The fluid was clear but under increased pressure. Pressure applied to the anterior fontanel was transmitted to the lumbar needle. On Sept.. 2, 1938, the spinal fluid was drained off through a lumbar puncture and a seten was inserted. On Oct. 3, 1938, the child had a stiff back. Examination revealed an increased area of sclerae visible above the pupils. T h e a n t e r i o r 2ontanel was bulging. Macewen's sign was positive. The head circumference was 51.2 cm. On Oct. 26~ 1938, another seton was inserted. On l%ov. 28, 1938, the child was progressing normally. The head circumference was 53 cm. ; chest, 45 cm. ; abdomen~ 45 cm. There was no increased tension over the anterior fontanel. On Jan. 23, 1939, the head circumference was 53.5 cm.; chest, 45 era.; abdomen, 45 era. I~ormal development; no symptoms, l~arch 2, 1939--For the past few days the child had been listless and whining. The head circumference was 54.3 cm.; there was increased tension over the anterior fontanel. The right side of the head was larger than the left. A subdural tap in the right angle of the anterior fontanel revealed a ground glass fluid under no increased pressure; 28 c.c. were removed. Spinal puncture revealed a clear fluid under no increased pressure; 15 c.c. were removed. Pressure on the anterior fontanel was transmitted to the lumbar needle. On Mar. 3, 1939, there was a reaccumuiation of the subdural fluid, and 60 c,c. were removed. On March 9, 1939, 40 c.c. of clear spinal fluid were drained off and a seton inserted. Head circumference was 53.2 cm. On Dec. 25, 1939, the child pulled a chair over on her head. Since then she had been fussy and held her head. On Dee. 29, 1939, the head circumference w a s 58 era. Spinal puncture revealed a clear fluid under greatly increased pressure; 35 c.c. were drained and a seton was inserted. On l~arch 25, 1942, the child was well and making a normal development until past few weeks when she would scream and hold the back of her neck. She complained of some stiffness of her legs. The head circumference was 61 cm., and there was a positive Macewen's sign. On 1VIarch 27~ 1942, the spinal fluid was clear, under increased pressure. I t was drained off and a seton was inserted. On Aug. 13, 1942, the child was well and had made a normal development. The head circumference was 60.5 cm.; chest, 59 cm.; and abdomen, 60 cm. There was no Macewea sign and no other sign of increased intracranial pressure. On March 23, 1943, the head circumfereKce was 62 cm. CASE 3.--5. H. (1VLC.H. 38821), a 4-month-old infant, was admitted to i~ilwaukee Children's Hospital Nov. 9, 1936. She had had a large head since birth; it had increased four inches in circumference in four months. On examination the head circumference was 65 cm. The anterior fontanel was large and bulging. Right ventricle puncture revealed a clear fluid under slightly increased pressure. The fluid was normal. Phenolsulphonphthalein injected appeared in the lumbar canal in eight minutes, and traces of the dye were recovered in the urine in twelve hours. Pressure on the anterior fontanel was transmitted to the lumbar needle. On I~ov. 1!3, 1936, the spinal fluid was drained off and a seton was inserted. The head circumference was then 55 cm. By Dec. 29, 1936, the head had increased 1.5 cm. in circumference. A second seten was inserted. This i n f a n t was taken home (Michigan) and was said to be doing well for five months. At that time she developed ~ flu," then pneumonia, and died. CASE 4.--5. H. (M.C.H. 47121), a 10-day-old infant, was admitted to the Milwaukee Children's Hospital Aug. 3, ]940. When the baby was 4 days old he had twitching of the upper extremities and a s p a s t i c convulsion. There was no bulging of the anterior fontanel. The spinal fluid was clear and normal. A subdural tap
694
THE
JOURI~AIJ
OF PEDIATRICS
yielded no fluid. Two days later nyst~gmus and projectile vomiting developed. Three days later there was increased tension over the anterior fontanel. The head was large, circumference 40 cm., and the sclerae showed pronfinently above the pupils. Chest circumference was 34.5 cm.; abdomen, 84.5 era. Phenolsulphonphthalein injected into the right ventricle was recovered in the lumbar canal in five minutes; 30 per cent of the dye was recovered in the urine in twelve hours. The infant developed gradually increasing tension over the anterior fontanel but was discharged Aug. 11, 1940. He was readmitted Aug. 19, 1940. The head circumference was 42 cm., and there was considerable tension over the anterio~ fontanel. The spinal fluid was clear and flowed freely: I t was drained. The i n f a n t was readmitted April 25, 1942, because of increasing size of the head. The infant had made a normal development. The sclerae showed prominently above the pupils; the head was large~ circumference 53.75 cm. T h e chest circumference w a s 43.25 cm. The s p i n a l fluid was clear and normal, but under increased pressure. Pressure on the anterior fontanel was transmitted to the lumbar needle. Thirty cubic centimeters of fluid were drained off, and a seton was inserted. From this time to July 10, !942 (twenty-seven months), the child made a normal development a~d his head circumference increased 4.25 era. while his chest increased 3 cm. and his abdomen decreased 1 cm. The anterior fontanel decreased from 5 by 7.5 cm. to 4 by 5 cm. The eyes looked normal. The child walked well. C.~SE 5.--T. H. (M.C.tt. 51243), an 1]-month-old infant, was admitted to Milwaukee Children's Hospital Aug. ]3, 1942. The birth history was normal. He had been slow in his development and had always had a large head. He could not sit or stand. The head was large, circmnference 49.5 cm., and the scalp veins were prominent. The a~terior fontanel was tense. The chest circumference was 41 cm.; abdomen, 36 era. On Aug. 17, 1942, spinal puncture revealed clear fluid which was normal and under no increased pressure. On Aug. 18, 1942, ventricuiar puncture revealed clear fluid under no increased pressure. Phenolsulphonphthalein was injected and only a trace noted in the lumbar fluid in twenty minutes; 40 per cent of the dye was recovered in the u r i n e in twelve hours. On Aug. 20, 1942~ lumbar puncture was done; 20 c.c. of fluid were drained off and a seton inserted. On March 24, 1943, the infant was examined and had no evident irncreased intracranial pressure. Skull circumference was 48.8 cm. -REFEREN CES ]. ]3ucy, Paul: Practice of Pediatrics, Hagerstown, Md., 1942, W. F. Prior Co., vol. 4, chap. 3, p. 27. 2. Scarff, J. E.: Am. J. Dis. Child. 63: 297, 1942. 3. Dandy, W. E.: finn. Surg. 68: 569, 1918. Dandy, W . E . : Ann. Surg. 108: 194, 1938. 4. Putnam, T . J . : Am. J. Dis. Child. 55: 990, ]938. Putnam, T . J . : New Eng. J. ivied. 210: 1373, 1934. 5. Hiller, ~. I.: Am. J. Surg. 20: 119, 1933.